Chapter 10 Seborrheic Dermatitis, Psoriasis, Recalcitrant Palmoplantar Eruptions, Pustular Dermatitis, And Erythroderma

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Seborrheic Dermatitis, Psoriasis, RecalcitrantPalmoplantar Eruptions, Pustular Dermatitis,and Erythroderma10

Seborrheic dermatitis

Clinical features

Seborrheic dermatitis is common, occurring in 25% of thepopulation. It is a chronic, supercial, inammatory diseasewith a predilection for the scalp, eyebrows, eyelids, nasolabialcreases, lips, ears (Fig. 10-1), sternal area, axillae, submam-mary folds, umbilicus, groins, and gluteal crease. The diseaseis characterized by scaling on an erythematous base. The scaleoften has a yellow, greasy appearance. Itching may be severe.

Dandruff (pityriasis sicca) represents a mild form of seborrheicdermatitis. An oily type, pityriasis steatoides, is accompaniedby erythema and an accumulation of thick crusts.

Other types of seborrheic dermatitis on the scalp includearcuate, polycyclic, or petaloid patches, and psoriasiform, exu-dative, or crusted plaques. The disease frequently spreadsbeyond the hairy scalp to the forehead, ears, postauricularregions, and neck. On these areas the patches have convexborders and are reddish-yellow or yellowish. In dark-skinnedindividuals, arcuate and petaloid lesions commonly involvethe hairline. In extreme cases the entire scalp is covered by agreasy, dirty crust with an offensive odor. In infants, yellowor brown scaling lesions on the scalp, with accumulated adher-ent epithelial debris, are called cradle cap.

Erythema and scaling are often seen in the eyebrows. The

lids may show yellowish-white, ne scales and faint erythema.The edges of the lids may be erythematous and granular (mar-ginal blepharitis), and the conjunctivae may be injected. If theglabella is involved, ssures in the wrinkles at the inner endof the eyebrow may accompany the ne scaling. In the naso-labial creases and on the alae nasi, there may be yellowish orreddish-yellow scaling macules, sometimes with ssures. Inmen, folliculitis of the beard area is common.

In the ears, seborrheic dermatitis may be mistaken for aninfectious otitis externa. There is scaling in the aural canals,around the auditory meatus, usually with marked pruritus.The postauricular region and skin under the lobe may beinvolved. In these areas the skin often becomes red, ssured,and swollen. In the axillae the eruption begins in the apices,bilaterally, and later progresses to neighboring skin. This

pattern resembles that of allergic contact dermatitis to deodor-ant, but differs from that of clothing dermatitis (which involvesthe periphery of the axillae but spares the vault). The involve-ment may vary from simple erythema and scaling to morepronounced petaloid patches with ssures. The inframam-mary folds and the umbilicus may be involved. The presternalarea is a favored site on the trunk.

Seborrheic dermatitis is common in the groin and glutealcrease, where its appearance may closely simulate tinea crurisor candidiasis. In these areas, the appearance often overlapswith that of inverse psoriasis. In fact, many of these patientshave an overlap of the two conditions (sebopsoriasis or sebor-

rhiasis) in the groin, as well as the scalp. The lesions mabecome generalized and progress to a generalized exfoerythroderma (erythroderma desquamativum), especiainfants. A minority of these infants will have evidenimmunosuppression. In adults, generalized eruptions maccompanied by adenopathy and may simulate mycosigoides or psoriatic erythroderma.

Seborrheic dermatitis may be associated with severalnal diseases. Parkinsons disease is often accompanisevere refractory seborrheic dermatitis involving the scaface, with waxy, profuse scaling. A unilateral injury innervation of the face, or a stroke, may lead to unilateral

ized seborrheic dermatitis. Patients with acquired immdeciency syndrome (AIDS) have an increased incideseborrheic dermatitis. An increased incidence has alsonoted in patients who are seropositive for human immdeciency virus (HIV), but have not developed other siclinical disease. Diabetes mellitus, especially in obese pesprue; malabsorption disorders; epilepsy; neuroleptic such as haloperidol; and reactions to arsenic and gold haproduced seborrheic dermatitis-like eruptions.

Etiology and pathogenesis

The etiology of this common disorder is complex, but mrelated to the presence of the lipophilic yeast Pityrosovale, which produces bioactive indoles. The density ofhas been correlated with the severity of the disease, and rtion of the yeast occurs with response to therapy. P. ovaalso be abundant on the scalps of patients who have no csigns of the disease, and the yeast may only be pathogepredisposed individuals.

Patients with seborrheic dermatitis may show upreguof interferon (IFN)-gamma, expressed interleukin expressed IL-1, and IL-4. Expression of cytotoxicity-actiligands and recruitment of natural killer (NK) cells havbeen noted.

Histology

The epidermis demonstrates regular acanthosis with

thinning of the suprapapillary plates. Varying degrspongiosis and lymphocyte exocytosis are noted. A charistic nding is the presence of a focal scale crust adjacthe follicular ostia.

Differential diagnosis

Some cases of seborrheic dermatitis bear a close clinical rblance to psoriasis, and the two conditions may ovPsoriasis tends to have more pronounced erythema and hsilvery scales that peel in layers. Removal of scales in psomay disclose bleeding points (Auspitz sign). This s
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Fig. 10-1Seborrheic dermatitis.

common but lacks great specicity. Severe itching favors seb-orrheic dermatitis. Characteristic psoriasis elsewhere (nailpitting, balanitis) may resolve the question. Impetigo of thescalp, especially when associated with pediculosis, may causedifculty in differentiation. Scalp impetigo can be an indolentcrusted dermatosis associated with failure to thrive. Langerhanscell histiocytosis may also resemble seborrheic dermatitis, buttypically demonstrates yellowbrown perifollicular papulesand groin ssuring. Crusted scabies of the scalp can also beconfused with seborrheic dermatitis, and Trichophyton ton-suransoften produces a subtle seborrheic scale. In subtle casesof tinea, a moist gauze pad rubbed vigorously on the scalp willtypically dislodge short, broken KOH-positive hairs. This canbe the fastest way to make the diagnosis.

Treatment

Agents suitable for use on glabrous skin include corticosteroidcreams, gels, sprays and foam. Corticosteroids tend to producea rapid effect, but on the face even mid-potency corticosteroidscan produce steroid rosacea. For this reason, antifungal agentsand topical calcineurin inhibitors are often preferred.Ketoconazole, ciclopirox, tacrolimus, zinc pyrithione, andpimecrolimus preparations are all effective alone and in com-bination. The antifungals are now available in a wide rangeof vehicles to include foams, gels, and liquids. Bifonazoleshampoo has been shown to be effective in treating infants andsmall children. Topical calcineurin inhibitors may be associ-

ated with a burning sensation, especially on moist skin, andmay produce ushing if patients consume alcohol. Patientsgenerally tolerate these agents better after initial treatmentwith a corticosteroid. An open, randomized, prospective, com-parative study of topical pimecrolimus 1% cream vs topicalketoconazole 2% cream found the two to be equally effective,but side effects were somewhat more common with pime-crolimus. Preliminary studies suggest oral itraconazole andoral terbinane may show some efcacy. Oral uconazoleshowed marginal benet. Study results with topical metroni-dazole have been mixed.

When secondary bacterial infection is present, a topical ororal antibiotic may be required. In patients infected with HIV,

lithium succinate ointment (Efalith) has been used for facdisease. Lithium gluconate 8% ointment has also been showto compare favorably with ketoconazole 2% emulsion healthy adults and was more effective in terms of control scaling and symptoms. Sodium sulfacetamide products, wor without sulfur, are effective in some refractory patients.

For scalp disease, selenium sulde, ketoconazole, tar, zpyrithione, uocinolone, and resorcin shampoos are effectivIn many patients, these agents may be used 23 times a wewith a regular shampoo used in between as requireAntifungal foams and gels, as well as corticosteroid solutiofoams, gels, and sprays, are often preferred by Caucasipatients, while ointment or oil preparations are preferred some black patients.

Itching of the external ear canal usually responds to a topicorticosteroid, calcineurin inhibitors, or antifungals such ketoconazole or ciclopirox. Some patients require the use oclass 1 corticosteroid on weekends to control refractory prutus. Cortisporin otic suspension can bring about prompt cleing, but contact dermatitis to neomycin may complicate tuse of some Cortisporin products. Desonide otic lotion (0.0desonide and 2% acetic acid) is also effective and may be bettolerated than Domeboro otic solution.

Sodium sulfacetamide drops or ointment may be effectifor seborrheic blepharitis. Oral tetracyclines can also be efftive, and have been shown to decrease the density of micr

organisms in the affected follicles. Steroid preparations asuitable for short-term use, but may induce glaucoma acataracts. Daily gentle cleansing with a cotton-tipped applictor and baby shampoo in water can reduce symptoms. severe cases, oral antibiotics or oral antifungals may be cobined with topical agents.

BerkT, et al:Seborrheic dermatitis. P.T. 2010 Jun; 35(6):348352.CmertA, et al:Efficacy of oral fluconazole in the treatment ofseborrheic dermatitis: a placebo-controlled study. Am J Clin Dermat2007; 8(4):235238.DawsonTLJr:Malassezia globosa andrestricta: breakthroughunderstanding of the etiology and treatment of dandruff andseborrheic dermatitis through whole-genome analysis. J InvestigDermatol Symp Proc 2007; 12(2):1519.ElewskiB, et al:Efficacy and safety of a new once-daily topicalketoconazole 2% gel in the treatment of seborrheic dermatitis: a pha

III trial. J Drugs Dermatol 2006; 5(7):646650.FiroozA, et al:Pimecrolimus cream, 1%, vs hydrocortisone acetatecream, 1%, in the treatment of facial seborrheic dermatitis: arandomized, investigator-blind, clinical trial. Arch Dermatol 2006;142(8):10661067.Food and Drug Administration, HHS:Dandruff, seborrheic dermatitis, anpsoriasis drug products containing coal tar and menthol for over-thecounter human use; amendment to the monograph. Final rule. FedRegist 2007; 72(43):98499852.GaitanisG, et al:AhR ligands, malassezin, and indolo[3,2-b]carbazolare selectively produced by Malassezia furfur strains isolated fromseborrheic dermatitis. J Invest Dermatol 2008; 128(7):16201625.GeeBC:Seborrhoeic dermatitis. Clin Evid 2004; 12:2344.HighWA, et al:Pilot trial of 1% pimecrolimus cream in the treatment seborrheic dermatitis in African American adults with associatedhypopigmentation. J Am Acad Dermatol 2006; 54(6):10831088.

JacksonWB:Blepharitis: current strategies for diagnosis andmanagement. Can J Ophthalmol 2008; 43(2):170179.KircikL:The evolving role of therapeutic shampoos for targetingsymptoms of inflammatory scalp disorders. J Drugs Dermatol 2010;9(1):4148.KocE, et al:An open, randomized, prospective, comparative study otopical pimecrolimus 1% cream and topical ketoconazole 2% cream

the treatment of seborrheic dermatitis. J Dermatolog Treat 2008; 1:1OzcanH, et al:Is metronidazole 0.75% gel effective in the treatment oseborrhoeic dermatitis? A double-blind, placebo controlled study. EuJ Dermatol 2007; 17(4):313316.SeckinD, et al:Metronidazole 0.75% gel vs. ketoconazole 2% cream the treatment of facial seborrheic dermatitis: a randomized, double-blind study. J Eur Acad Dermatol Venereol 2007; 21(3):345350.



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Fig. 10-2 Psoriasis.

Fig. 10-3 Pustular psoriasis of the hand.

Fig. 10-4 Nail pitting and distal onycholysis in psoriasis.

ShemerA, et al:Treatment of moderate to severe facial seborrheicdermatitis with itraconazole: an open non-comparative study. Isr MedAssoc J 2008; 10(6):417418.SiadatAH, et al:The efficacy of 1% metronidazole gel in facialseborrheic dermatitis: a double blind study. Indian J Dermatol VenereolLeprol 2006; 72(4):266269.TajimaM, et al:Molecular analysis of Malasseziamicroflora inseborrheic dermatitis patients: comparison with other diseases andhealthy subjects. J Invest Dermatol 2008; 128(2):345351.VenaGA, et al:Oral terbinafine in the treatment of multi-site seborrheicdermatitis: a multicenter, double-blind placebo-controlled study. Int JImmunopathol Pharmacol 2005; 18(4):745753.WaldroupW, et al:Medicated shampoos for the treatment of seborrheic

dermatitis. J Drugs Dermatol 2008; 7(7):699703.WarshawEM, et al:Results of a randomized, double-blind, vehicle-controlled efficacy trial of pimecrolimus cream 1% for the treatment ofmoderate to severe facial seborrheic dermatitis. J Am Acad Dermatol2007; 57(2):257264.

Psoriasis

Clinical features

Psoriasis is a common, chronic, and recurrent inammatorydisease of the skin characterized by circumscribed, erythema-tous, dry, scaling plaques of various sizes. The lesions areusually covered by silvery white lamellar scales. The lesionshave a predilection for the scalp, nails, extensor surfaces of thelimbs, umbilical region, and sacrum. The eruption is usuallysymmetrical. It usually develops slowly but may be exanthema-tous, with the sudden onset of numerous guttate (droplike)lesions (Fig. 10-2). Subjective symptoms, such as itching orburning, may be present and may cause extreme discomfort.

The early lesions are small erythematous macules, whichfrom the beginning are covered with dry, silvery scales. Thelesions increase in size by peripheral extension and coales-cence. The scales are micaceous, meaning that they peel inlayers. They are looser toward the periphery and adherentcentrally. When removed, bleeding points appear (Auspitzsign). Although plaques typically predominate, lesions may beannular or polycyclic. Old patches may be thick and coveredwith tough lamellar scales like the outside of an oyster shell

(psoriasis ostracea). Various other descriptive terms have inthe past been applied to the diverse appearances of the lesions:psoriasis guttata, in which the lesions are the size of waterdrops; psoriasis follicularis, in which tiny, scaly lesions arelocated at the orices of hair follicles; psoriasis gurata, pso-riasis annulata, and psoriasis gyrata, in which curved linearpatterns are produced by central involution; psoriasis discoi-

dea, in which central involution does not occur andpatches persist; and psoriasis rupioides, in which clesions occur, resembling syphilitic rupia. The term cplaque psoriasis is often applied to stable lesions of theand extremities. Inverse psoriasis predominates in intenous areas. Pustular variants of psoriasis may be chrothe palms and soles (Fig. 10-3), or may be eruptive and apanied by severe toxicity and hypocalcemia.

Involved nails (Fig. 10-4) can demonstrate distal onlysis, random pitting (the result of parakeratosis the proximal matrix), oil spots (yellow areas of subuparakeratosis from the distal matrix), or salmon p(nailbed psoriasis). Thick subungual hyperkeratosisresemble onychomycosis.

Types

Seborrheic-like psoriasisSome cases of psoriasis overlap with seborrheic dermSeborrheic lesions may predominate on the face, und



4. oligoarthritis with swelling and tenosynovitis of one ofew hand joints (70%)

5. ankylosing spondylitis alone or with peripheral arthr(5%).

Most radiographic ndings resemble those in rheumatoarthritis, but certain ndings are highly suggestive of psoriasThese include erosion of terminal phalangeal tufts (acrostelysis), tapering or whittling of phalanges or metacarpwith cupping of proximal ends of phalanges (pencil in a cdeformity), bony ankylosis, osteolysis of metatarsals, prediltion for distal interphalangeal and proximal interphalang

joints, relative sparing of metacarpal phalangeal and metatsal phalangeal joints, paravertebral ossication, asymmetrisacroiliitis, and rarity of bamboo spine when the spineinvolved. Nearly half the patients with psoriatic arthritis hatype human leukocyte antigen (HLA)-B27.

Rest, splinting, passive motion, and nonsteroidal aninammatory drugs (NSAIDs) may provide symptomarelief but do not prevent deformity. Methotrexate, cyclosporitacrolimus, and biologic agents are disease-modifying druthat prevent deformity.

Guttate psoriasisIn this distinctive form of psoriasis typical lesions are the siof water drops, 25 mm in diameter. Lesions typically occas an abrupt eruption following some acute infection, such

a streptococcal pharyngitis. Guttate psoriasis occurs mostlypatients under age 30. This type of psoriasis usually responrapidly to broad-band ultraviolet (UV) B at erythemogedoses. Suberythemogenic doses often have little impact on tlesions. This is one of the few forms of psoriasis where broaband UVB may have an advantage over narrow-band UVMinimal erythemogenic dose (MED) testing is recommendto allow for appropriately aggressive treatment. Recurrent esodes may be related to pharyngeal carriage of the responsibstreptococcus by the patient or a close contact. A course osemisynthetic penicillin (such as dicloxacillin, 250 mg fotimes a day for 10 days) with rifampin (600 mg/day for adult) may be required to clear chronic streptococcarriage.

Generalized pustular psoriasis (von Zumbusch)Typical patients have had plaque psoriasis and often psoriaarthritis. The onset is sudden, with formation of lakes of pperiungually, on the palms, and at the edge of psoriaplaques. Erythema occurs in the exures before the generized eruption appears. This is followed by a generalized ethema and more pustules (Fig. 10-7). Pruritus and intenFig. 10-5 Penile psoriasis with erythema and silver scale.

Fig. 10-6Psoriatic arthritis.

breasts, and in the scalp, exures, and axillae. Lesions in theseareas are moist and erythematous, with yellow, greasy, softscales, rather than dry and micaceous scales. Terms such assebopsoriasis and seborrhiasis may be used to describe thecondition of such patients.

Inverse psoriasisThis form selectively and often exclusively involves folds,recesses, and exor surfaces such as the ears, axillae, groins,inframammary folds, navel, intergluteal crease, penis (Fig.10-5), lips, and web spaces. Other areas, such as the scalp andnails, may be involved.

Napkin psoriasisNapkin psoriasis, or psoriasis in the diaper area, is character-istically seen in infants between 2 and 8 months of age. Lesionsappear as brightly erythematous, sharply demarcated patchesof skin involving much of the diaper area. The lesions typicallyclear with topical therapy, but psoriasis may reappear inadulthood.

Psoriatic arthritisFive clinical patterns of arthritis occur:

1. asymmetrical distal interphalangeal joint involvementwith nail damage (16%)

2. arthritis mutilans with osteolysis of phalanges and meta-

carpals (5%) (Fig. 10-6)3. symmetrical polyarthritis-like rheumatoid arthritis, with

claw hands (15%)

Fig. 10-7Pustular psoriasis.



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burning are often present. Mucous membrane lesions arecommon. The lips may be red and scaly, and supercial ulcera-tions of the tongue and mouth occur. Geographic or ssuredtongue frequently occurs (Fig. 10-8).

The patient is frequently ill with fever, erythroderma, hypo-calcemia, and cachexia. A number of cases of acute respiratorydistress syndrome associated with pustular and erythroder-

mic psoriasis have been reported. Other systemic complica-tions include pneumonia, congestive heart failure, andhepatitis.

Episodes are often provoked by withdrawal of systemic cor-ticosteroids. The authors have also observed generalized pus-tular psoriasis as the presenting sign of Cushings disease.Other implicated drugs include iodides, coal tar, terbinane,minocycline, hydroxychloroquine, acetazolamide, and sali-cylates. There is usually a strong familial history of psoriasis.Generalized pustular psoriasis may occur in infants and chil-dren with no implicated drug. It may also occur as an episodicevent punctuating the course of localized acral pustularpsoriasis.

Acitretin is the drug of choice in this severe disease. Theresponse is generally rapid. Isotretinoin is also effective.

Cyclosporine, methotrexate, and biologicals are alternatives.Sometimes dapsone is effective in doses of 50100 mg/day.

Acrodermatitis continua of HallopeauTypical patients develop acral erythematous plaques studdedwith pustules. The nailbeds are heavily involved, and the n-gernails oat away on lakes of pus, resulting in anonychia.Hyperkeratosis often ensues, and the ngertips becomeincreasingly painful, tapering to long keratotic points.Occasionally, patients may develop generalized pustular ares(Fig. 10-9). Acrodermatitis continua is discussed in more detailbelow.

Impetigo herpetiformisThis term has been applied to generalized pustular psoriasis

of pregnancy. Flexural erythema, studded with pustules, oftenoccurs initially, followed by a generalized pustular are andincreasing toxicity. As the patients are pregnant, systemicretinoids are not appropriate. Many patients only respond todelivery, and early delivery should be strongly considered assoon as it is safe for the infant. Alternatively, patients mayrespond to prednisone at a dose of 1 mg/kg/day. The cortico-steroid can also contribute to neonatal lung maturity.

Keratoderma blennorrhagicum (Reiter syndrome)Keratoderma blennorrhagicum resembles psoriasis both histo-logically and clinically, except for its tendency for thicker kera-

Fig. 10-8 Geographic tongue in pustular psoriasis.

Fig. 10-9 Generalized pustular flare in a patient with acrodermcontinua.

Fig. 10-10Erythrodermic psoriasis.

totic lesions. Patients are often positive for HLA-B2develop reactive arthritis and skin disease after a bout othritis or enteritis.

Erythrodermic psoriasis

Patients with psoriasis may develop a generalized erderma (Fig. 10-10). Erythrodermic psoriasis is covergreater detail under exfoliative dermatitis.

Course

The course of psoriasis is unpredictable. It usually begthe scalp or elbows, and may remain localized in the orregion for years. Chronic disease may also be almost enlimited to the ngernails. Involvement over the sacrumeasily be confused with candidiasis or tinea. Onset mabe sudden and widespread.



Two of the chief features of psoriasis are its tendency torecur and its persistence. The isomorphic response (Koebnerphenomenon) is the appearance of typical lesions of psoriasisat sites of even trivial injury (Fig. 10-11). Lesions may occur atsites of scratches, incisions, and burns. Lesions may rstappear after a viral exanthema or following pityriasis rosea.The isomorphic response may occur if psoriatic lesions areseverely burned during phototherapy. With a reduction inlight dosage, the erythema and burning resolve, and theplaques begin to clear. Woronoffs ring is concentric blanchingof the erythematous skin at or near the periphery of a healingpsoriatic plaque. It is often the rst sign that the patientspsoriasis is responding to phototherapy.

The palms and soles are sometimes exclusively affected,showing discrete erythematous dry scaling patches, circum-scribed verrucous thickenings, or pustules on an erythematousbase. The patches usually begin in the mid-portions of thepalms or on the soles, and gradually expand. Psoriasis of thepalms and soles is typically chronic and extremely resistant totreatment.

Many studies report an association between hepatitis C andpsoriasis, and hepatitis C has also been implicated in psoriaticarthritis. If treatment of psoriasis is to include a potentiallyhepatotoxic drug, such as methotrexate, a hepatitis C serologyshould be obtained. It should also be noted that interferontreatment of the hepatitis can further exacerbate or inducepsoriasis. Anti-tumor necrosis factor (TNF)- therapy showspromise in the treatment of psoriasis, even in the setting of

chronic hepatitis C infection.

Inheritance

In a large study of psoriasis in monozygotic twins, heritabilitywas high and environmental inuence low. Patients with pso-riasis often have relatives with the disease, and the incidencetypically increases in successive generations. Multifactorialinheritance is likely. Analysis of population-specic HLA hap-lotypes has provided evidence that susceptibility to psoriasisis linked to the class I and II major histocompatibility complex(MHC) on human chromosome 6. A number of genetic loci are

Fig. 10-11 Koebner phenomenon in psoriasis.

linked to psoriasis, including PSORS1on chromosome 6 awithin the MHC, and PSORS2on chromosome 17q. It has abeen shown that there are two subsets that differ in ageonset and in the frequency of HLA associations. Early onsetype I psoriasis and is associated mostly with Cw6, B57, aDR7. Late onset is type II and this predominantly featurCw2. PSORS9has also been conrmed as a susceptibility locfor psoriasis.

A variety of other HLA associations have been reportedis believed that any individual who has B13 or B17 carrieve-fold risk of developing psoriasis. In pustular psoriaHLA-B27 may be seen, whereas B13 and B17 are increasedguttate and erythrodermic psoriasis. In palmoplantar pustusis, there is an association with HLA-B8, Bw35, Cw7, and DRHLA typing is a research tool for population-based studibut is of limited value in assessing an individual patient.

Epidemiology

Psoriasis occurs with equal frequency in both sexes. Betwe1 and 2% of the US population has psoriasis. It occurs lfrequently in the tropics. It is less common in North Americand West African black persons. Native Americans and natFijians rarely have psoriasis. The onset of psoriasis is at a meage of 27 years, but the range is wide, from the neonatal perito the seventies. Severe emotional stress tends to aggrav

psoriasis in almost half of those studied.In pregnancy there is a distinct tendency for improveme

or even temporary disappearance of lesions in the majorof women studied. After childbirth there is a tendenfor exacerbation of lesions. Paradoxically, pregnancy is athe milieu for impetigo herpetiformis, and psoriasis mbehave differently from one pregnancy to another in the sampatient.

A high prevalence of celiac disease has been noted in patienwith psoriasis. Lymphoma also has an increased incidencethese patients, and psoriasis has been linked to the metabosyndrome and a higher risk of cardiovascular disease.

Pathogenesis

Psoriasis is a hyperproliferative disorder, but the proliferatiis driven by a complex cascade of inammatory mediatoPsoriasis appears to represent a mixed T-helper (Th)1 aTh17 inammatory disease. Th17 cells appear to be more proimal in the inammatory cascade. T cells and cytokines plpivotal roles in the pathophysiology of psoriasis. Overexpressiof type 1 cytokines, such as IL-2, IL-6, IL-8, IL-12, IFN-aTNF-, has been demonstrated, and overexpression of ILleads to the accumulation of neutrophils. The main signal Th1 development is IL-12, which promotes intracellular IFNproduction. In animal models, shifting from Th1 to Tresponses improves psoriasis. IL-4 is capable of inducing Tresponses and improving psoriasis. Reduced expression of tanti-inammatory cytokines IL-1RA and IL-10 has been foun

and polymorphisms for IL-10 genes correlate with psoriasIL-10 is a type 2 cytokine with major inuences on immunregulation, inhibiting type 1 proinammatory cytokine pduction. Patients on established traditional therapies shorising levels of IL-10 mRNA expression, suggesting that IL-may have antipsoriatic capacity.

The response to biologic agents has demonstrated that CDlymphocytes, CD-11a and TNF-are important in the pathgenesis of psoriasis. IL-15 triggers inammatory cell recrument, angiogenesis, and production of inammatory cytokinincluding IFN-, TNF-, and IL-17, all of which are upreglated in psoriatic lesions. The interplay is complex, but IL-



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Psoriasis,


10appears to be proinammatory, while IL-22 may serve toretard keratinocyte differentiation. IL-23 stimulates survival,as well as proliferation of Th17 cells. Circulating NK cells arereduced in psoriasis.

Specic targets for therapy include TNF-, leukocytefunction-associated antigen-1 (LFA-1)/intercellular adhesionmolecule-1 (ICAM-1) binding, and LFA-3/CD2 binding. AnIL-15 monoclonal antibody has been shown to be effective ina mouse model of psoriasis.

StreptococciStreptococci play a role in some patients. Patients with psoria-sis report sore throat more often than controls. Beta-hemolyticstreptococci of Lanceeld groups A, C, and G can cause exac-erbation of chronic plaque psoriasis. Th1 cells recognize cell-wall extract isolated from group A streptococci. HLA variationhas a signicant effect on the immune response to group Astreptococci.

StressVarious studies have shown a positive correlation betweenstress and severity of disease. In almost half of patients studied,stress appears to play a signicant role.

Drug-induced psoriasisPsoriasis may be induced by -blockers, lithium, antimalarials,

terbinane, calcium channel blockers, captopril, glyburide,granulocyte colony-stimulating factor, interleukins, interfer-ons, and lipid-lowering drugs. Systemic steroids may causerebound or pustular ares. Antimalarials are associated witherythrodermic ares, but patients traveling to malaria-endemicregions should take appropriate prophylaxis. Often, drugssuch as doxycycline or meoquine are appropriate for thegeographic area, but when a quinine derivative offers the bestprotection, it is generally better to take the prophylactic dosesof a quinine derivative than to risk disease and full-dosetreatment.

Pathology

Histologically, all psoriasis is pustular. The microscopic pus-tules include spongiform intraepidermal pustules, and Munromicroabscesses within the stratum corneum. In early guttatelesions, focal parakeratosis is noted within the stratumcorneum. The parakeratotic focus typically has an outlineresembling a childs rendition of a seagull. Neutrophils aregenerally noted immediately above the focus of parakeratosis,but in some sections, the neutrophils will not be visible as aresult of sampling error. In plaque psoriasis, neutrophilic fociare so numerous that they are rarely missed. Neutrophilicmicroabscesses are generally present at multiple levels in thestratum corneum, usually on top of small foci of parakeratosis.These foci generally alternate with areas of orthokeratoticstratum corneum, suggesting that the underlying spongiformpustules arise in a rhythmic fashion. The granular layer is

absent focally, corresponding to areas producing foci of para-keratosis. In well-developed plaques, there is regular epider-mal acanthosis with long, bulbous rete ridges, thinning overthe dermal papillae, and dilated capillaries within the dermalpapillae. The last two ndings correlate with the Auspitz sign.The stratum corneum may be entirely parakeratotic but stillshows multiple small neutrophilic microabscesses at varyinglevels. Spongiosis is typically scant, except in the area imme-diately surrounding collections of neutrophils.

In pustular psoriasis, geographic tongue, and Reiter syn-drome, intraepidermal spongiform pustules tend to be muchlarger. Grossly pustular lesions often have little associated

acanthosis. In Reiter syndrome, the stratum corneum ismassively thickened, with prominent foci of neutrophils parakeratosis, alternating with orthokeratosis.

Acral lesions often demonstrate nondiagnostic featurtologically. Spongiosis is typically prominent in these land often leads to a differential diagnosis of psoriachronic psoriasiform spongiotic dermatitis. Foci of neutroften contain serum and may be interpreted as impetigcrusting.

On direct immunouorescence testing, the stratum cordemonstrates intense uorescence with all antibodies, coment, and brin. This uorescence may be partially indeent of the uorescent label, as it has been noted in hematand eosin (H&E)-stained sections and frozen unstainetions. The same phenomenon of stratum corneum autocence has been noted in some cases of candidiasidemonstrate a psoriasiform histology.

Psoriasis can generally be distinguished from dermatthe paucity of edema, the relative absence of spongiostortuosity of the capillary loops, and the presence otrophils above foci of parakeratosis. Neutrophils in the stcorneum are commonly seen in tinea, impetigo, candiand syphilis, but rarely are found atop parakeratosis alting with orthokeratosis in a rhythmic fashion. In psoriassyphilis the rete are typically long and slender, a vainterface dermatitis is commonly present, dermal blood v

appear to have no lumen because of endothelial swellinplasma cells are present in the dermal inltrate. Abouthird of biopsies of syphilis lack plasma cells, but the reing characteristics still suggest the correct diagPsoriasiform lesions of mycosis fungoides exhibit epidtropism of large lymphocytes with little spongiosis. Thephocytes are typically larger, darker, and more angulatethe lymphocytes in the dermis. There is associated papdermal brosis, and the supercial perivascular inltrasymmetrically distributed around the postcapillary vefavoring the epidermal side (bare underbelly sign).

Clinical differential diagnosis

Psoriasis must be differentiated from dermatomyositis,

erythematosus, seborrheic dermatitis, pityriasis rosea, planus, eczema, and psoriasiform syphilid. The distribupsoriasis is on the extensor surfaces, especially of the eand knees, and on the scalp; dermatomyositis sharedistribution, whereas lupus erythematosus generallyinvolvement of the extensor surfaces. Patients with dermyositis may exhibit a heliotrope sign, atrophy, poikilodand nailfold changes. Advanced lesions of discoid luputhematosus often demonstrate follicular hyperkeratosis (tack sign). Seborrheic dermatitis has a predilection foeyebrows, nasolabial angle, ears, sternal region, and eThe scales in psoriasis are dry, white, and shiny, whereasin seborrheic dermatitis are greasy and yellowish. On reof the scales in psoriasis there is an oozing of blood frocapillaries (Auspitz sign), whereas this does not occur i

orrheic dermatitis.In pityriasis rosea the eruption is located on the upper

trunk, and thighs, and the duration is a matter of wLesions are typically oval and follow skin tension Individual lesions show a crinkling of the epidermis anlarette scaling. A herald patch is frequently noted. Lplanus chiey affects the exor surfaces of the wristankles. Often the violaceous color is pronounced. In dskinned individuals, the lesions have a tendency tonounced hyperpigmentation. The nails are not pittedpsoriasis, but longitudinally ridged, rough, and thickPterygium formation is characteristic of lichen planus.


http:///reader/full/chapter-10-seborrheic-dermatitis-psoriasis-recalcitrant-palmopla

Hand eczema may resemble psoriasis. In general, psoriaticlesions tend to be more sharply marginated, but at times thelesions are indistinguishable. Psoriasiform syphilid has inl-trated copper-colored papules, often arranged in a guratepattern. Serologic tests for syphilis are generally positive, butprozone reactions may occur, and the serum may have to bediluted in order to obtain a positive test. Generalized lymph-adenopathy and mucous patches may be present.

Treatment

Topical therapy is generally suitable for limited plaques.Localized treatments, such as the excimer laser or other formsof intense pulsed light, may be suitable for limited plaques.Phototherapy remains highly cost-effective for widespreadpsoriasis. Cyclosporine has a rapid onset of action, but is gen-erally not suitable for sustained therapy. Methotrexate remainsthe systemic agent against which others are compared. Biologicagents can produce dramatic responses at dramatic expense.Rotating therapeutic agents that have varying toxicities haveconceptual appeal, and combination therapy may reduce tox-icity and reduce the incidence of neutralizing antibodies toagents such as iniximab. Attention should be paid to comor-bidities including metabolic syndrome, cardiac risk, and jointmanifestations.

Topical treatmentCorticosteroidsTopical application of corticosteroids in creams, ointments,lotions, foams, and sprays is the most frequently prescribedtherapy for psoriasis. Class I steroids are suitable for 2-weekcourses of therapy on most body areas. Therapy can be con-tinued with pulse applications on weekends to reduce theincidence of local adverse effects. On the scalp, corticosteroidsin propylene glycol, gel, foam, and spray bases are preferredby most white patients. Black patients may nd them drying,and may prefer oil and ointment preparations. Low to mid-strength creams are preferred in the intertriginous areas andon the face. To augment effectiveness of topical corticosteroidsin areas with thick keratotic scale, the area should be hydrated

prior to application, and covered with an occlusive dressingof a polyethylene lm (Saran wrap) or a sauna suit.Unfortunately, there is typically a rapid recurrence of diseasewhen topical corticosteroid therapy is discontinued. Sideeffects include epidermal atrophy, steroid acne, miliaria, andpyoderma.

Intralesional injections of triamcinolone are helpful forrefractory plaques. Triamcinolone acetonide (Kenalog) sus-pension, 10 mg/mL, may be diluted with sterile saline to makea concentration of 2.55 mg/mL. Good results are also obtainedin the treatment of psoriatic nails by injecting triamcinoloneinto the region of the matrix and the lateral nailfold. A digitalblock can be performed prior to injection to provide anesthe-sia. Injections are given once a month until the desired effectis achieved.

TarsCrude coal tar, and tar extracts such as liquor carbonisdetergens, can be compounded into agents for topical use.Tar bath oils and shampoos are readily available. Oil of cade(pine tar) or birch tar in concentrations of 510% may also beincorporated into ointments. The odor of all tars may beoffensive.

AnthralinAnthralin is effective, but is irritating and stains skin, clothing,and bedding. To avoid these drawbacks, short-contact anthra-

lin treatment (SCAT) can be helpful, with anthralin washed after 1530 min. In warmer climates, SCAT is often done odoors to keep the mess out of the house. Anthralin exertsdirect effect on keratinocytes and leukocytes by suppressineutrophil superoxide generation and inhibiting monocyderived IL-6, IL-8, and TNF-.

TazaroteneTazarotene is a nonisomerizable retinoic acid receptor-speciretinoid. It appears to treat psoriasis by modulating keratincyte differentiation and hyperproliferation, as well as by su

pressing inammation. Combining its use with a topicorticosteroid and weekend pulse therapy can decreairritation.

CalcipotrieneVitamin D3affects keratinocyte differentiation partly throuits regulation of epidermal responsiveness to calciuTreatment with the vitamin D analog calcipotriene (Dovonein ointment, cream, or solution form has been shown to very effective in the treatment of plaque-type and scalp priasis. Combination therapy with calcipotriene and higpotency steroids may provide greater response rates, fewside effects, and steroid-sparing. Calcipotriene is unstablethe presence of many other topical agents and degrades in tpresence of UV light. Monitoring of serum calcium levels

adults is not required. Calcipotriene plus betamethasodipropionate (Taclonex) is more effective than either agealone.

Macrolactams (calcineurin inhibitors)Topical macrolactams such as tacrolimus and pimecrolimare especially helpful for thin lesions in areas prone to atropor steroid acne. The burning commonly associated with theagents can be problematic, but may be avoided by prior trement with a corticosteroid, and by application to dry skrather than after bathing.

Salicylic acidSalicylic acid is used as a keratolytic agent in shampo

creams, and gels. It can promote the absorption of other topiagents. Widespread application may lead to salicylate toxicmanifesting with tinnitus, acute confusion, and refractohypoglycemia, especially in patients with diabetes and thowith compromised renal function.

Ultraviolet lightPhototherapy is a cost-effective and underutilized modalfor psoriasis. In most instances sunlight improves psoriasHowever, severe burning of the skin may cause the Koebnphenomenon and an exacerbation. Articial UVB lightproduced by uorescent bulbs in broad-band or narrow-baspectrums. Maximal effect is usually achieved at MEDAlthough suberythemogenic doses can be effective, tresponse is slower than with erythemogenic regimens. W

treatment, a tanning response occurs, and the dose must increased to maintain efcacy. Maintenance UVB photothapy after clearing contributes to the duration of remission ais justied for many patients.

Using a monochromator, it has been shown that wavlengths of 254, 280, and 290 nm are ineffective; at 296, 300, 3and 313 nm there is clearing. Narrow-band UVB (peak emsion around 311 nm) has been shown to be more effectivetreating psoriasis than broad-band UVB. Erythemogenic doare not required in order to achieve a response. The responrates are better than 70% and close to those achievable wPUVA therapy.



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10Goeckerman techniqueThe Goeckerman technique remains an effective and cost-effective method of treatment. In its modern form, a 25% tarpreparation is applied to the skin, and a tar bath is taken atleast once a day. The excess tar is removed with mineral orvegetable oil, and UV light is given. In psoriasis daycarecenters, patients clear in an average of 18 days, and 75%remain free of disease for extended periods. The addition of atopical corticosteroid to the Goeckerman regimen shortens thetime required for remission. Phototoxic reactions (tar smarts)may occur as a result of UVA generated by the predominantly

UVB bulbs.

Ingram techniqueThe Ingram technique consists of a daily coal tar bath in asolution such as 120 mL liquor carbonis detergens to 80 L ofwarm water. This is followed by daily exposure to UV lightfor increasing periods. An anthralin paste is then applied toeach psoriatic plaque. Talcum powder is sprinkled over thelesions and stockinette dressings are applied. Modern versionsof the technique employ SCAT.

PUVA therapyHigh-intensity longwave UV radiation (UVA) given 2 h afteringestion of 8-methoxypsoralen (Oxsoralen-Ultra), twice aweek, is highly effective, even in severe psoriasis. Most patientsclear in 2025 treatments, but maintenance treatment is needed.

Although PUVA therapy is highly effective, in patients withless than 50% of the skin surface affected, UVB may be as good.Polyethylene sheet bath PUVA is another therapeutic alterna-tive to oral psoralen-UVA. The patient is immersed in a psor-alen solution contained in plastic sheeting that conforms to thepatients body.

Oral psoralen can produce cataracts, and protective eyewearmust be used. PUVA therapy is a risk factor for skin cancer,including squamous cell carcinoma and melanoma. Arsenicexposure is a more signicant cofactor than prior exposure tomethotrexate, UVB, or concomitant use of topical tar. Mentreated without genital protection are at an increased risk ofdeveloping squamous cell carcinomas of the penis and

scrotum. Although the risk of cancer is dose-related, there isno denitive threshold dose of cumulative PUVA exposureabove which carcinogenicity can be predicted.

Surgical treatmentIn patients with pharyngeal colonization by streptococci, anexcellent response has been reported after tonsillectomy. Moreeffective antibiotic regimens, such as a 10-day course ofdicloxacillin combined with rifampin (600 mg/day for anadult), have largely replaced tonsillectomy.

HyperthermiaLocal hyperthermia can clear psoriatic plaques, but relapse isusually rapid. Microwave hyperthermia may produce signi-cant complications, such as pain over bony prominences andtissue destruction.

Occlusive treatmentOcclusion with surgical tape or dressings can be effective asmonotherapy or when combined with topical drugs.

Systemic treatment

CorticosteroidsThe hazards of the injudicious use of systemic corticosteroidsmust be emphasized. There is great risk of rebound orinduction of pustular psoriasis when therapy is stopped.

Corticosteroid use is generally restricted to unique cistances, such as impetigo herpetiformis when expeddelivery is not possible.

MethotrexateThis folic acid antagonist remains the standard against other systemic treatments are measured. Methotrexategreater afnity for dihydrofolic acid reductase than haacid. The indications for the use of methotrexate includriatic erythroderma, psoriatic arthritis, acute pustular psis (von Zumbusch type), or widespread body sinvolvement. Localized pustular psoriasis or palmoppsoriasis that impairs normal function and employmenalso require systemic treatment.

It is important to make sure that the patient has no hof liver or kidney disease. Methotrexate can be toxic liver and decreased renal clearance can enhance toxicity.important factors to consider are alcohol abuse, cryptocirrhosis, severe illness, debility, pregnancy, leukothrombocytopenia, active infectious disease, immunciency, anemia, colitis, and ability to comply with direcHepatic enzymes, bilirubin, serum albumin, creatinineline phosphatase, complete blood count (CBC), platelet hepatitis serology (B and C), HIV antibody, and urinshould all be evaluated before starting treatment. Patienthypoalbuminemia have a higher risk of developing p

nary complications.The need for liver biopsy remains controversial. Bionot without risks and is not commonly performed setting of methotrexate therapy for rheumatic dHowever, patients with psoriasis have a greater risk odisease than other patient populations. In most patientno risk factors for liver disease, the rst liver biopsy ismonly obtained at approximately 1.01.5 g of cumumethotrexate and repeated every subsequent 1.52.0 g utotal of 4.0 g is reached. The frequency then changes to1.01.5 g cumulative intervals. These recommendatiolikely to change as more data are evaluated. Weekly counts and monthly liver enzyme assessment are rmended at the onset of therapy or when the dosage is chaMonitoring of aminoterminal procollagen III peptide an

abolic panels that predict the risk of brosis (NASH Fibrmay reduce the need for liver biopsy.

Numerous treatment schedules have evolved. The aurecommend either three divided oral doses (12 h apart) wweekly single doses orally, or single weekly subcutainjections. The weekly dose varies from 5 mg to more50 mg, with most patients requiring 1530 mg a week. Osingle dose exceeds 25 mg, oral absorption is unprediand subcutaneous injections are recommended. Middoses can result in severe toxicity and must be avoOral or cutaneous ulceration may be a sign that the phas taken a mid-week dose. Oral folic acid has been repto decrease side effects, especially nausea, and do14 mg/day are used. Oral folic acid is not adequathe treatment of overdosage and leukovorin must be u

such cases.

CyclosporineThe therapeutic benet of cyclosporine in psoriatic dmay be related to downmodulation of proinammepidermal cytokines. The microemulsion formulation Nhas greater bioavailability and is now standard. Do25 mg/kg/day generally produce rapid clearing of psoUnfortunately, the lesions recur rapidly as well, and tranto another form of therapy is required. Treatment duratiup to 6 months are associated with a low incidence ofcomplications, but blood pressure and serum creatinine



be monitored and doses adjusted accordingly. Usually, thedose is reduced if the baseline creatinine increases byone-third.

DietThe anti-inammatory effects of sh oils rich in n-3 polyun-saturated fatty acids have been demonstrated in rheumatoidarthritis, inammatory bowel disease, psoriasis, and asthma.n-3 and n-6 polyunsaturated fatty acids affect a variety ofcytokines, including IL-1, IL-6, and TNF. Herbal remedieshave also been used with variable effects. Many of theseproducts are unpalatable, and their efcacy does not comparefavorably to pharmacologic agents.

Oral antimicrobial therapyThe association of streptococcal pharyngitis with guttate pso-riasis is well established. Staphylococcus aureusand streptococcisecrete exotoxins that act as superantigens, producing massiveT-cell activation. Oral antibiotic therapy for patients with pso-riasis infected with these organisms is imperative. The efcacyof antimicrobial agents in other subsets of psoriasis is unclear.Oral bile acid supplementation has been shown to improvepsoriasis, presumably by affecting the microora and endotox-ins in the gut. Oral ketoconazole, itraconazole, and other anti-biotics have shown efcacy in a limited number of patientswith psoriasis.

RetinoidsOral treatment with the aromatic retinoid ethylester, etreti-nate, was effective in many patients with psoriasis, especiallyin pustular disease. Because of its long half-life, the drug hasbeen replaced by acitretin. Alcohol ingestion can convertacitretin to etretinate and is strongly discouraged. 13-Cis-retinoic acid can also produce good results in some patientswith pustular psoriasis. All of these drugs are potent tera-togens and elevations in triglycerides may complicate therapy.Combinations of retinoic acids with photochemotherapy canbe effective in chronic plaque psoriasis, resulting in loweredcumulative doses of light.

Dapsone

Dapsone use is limited largely to palmoplantar pustulosis orother variants of pustular psoriasis. Even in this setting, it is asecond- or third-line agent with limited efcacy.

Biologic agentsA number of biologic agents are available that can producedramatic responses in some patients with psoriasis; all areexpensive. Three agents block TNF-. Iniximab is a chimericmonoclonal antibody to TNF-and requires intravenous infu-sion; etanercept is a fusion protein of human TNF type IIreceptor and the Fc region of IgG1; and adalimumab is arecombinant, fully human IgG1 monoclonal antibody to TNF-. Alefacept is a fusion protein of the external domain ofLFA-3 and the Fc region of IgG1; it blocks T-cell activation andtriggers apoptosis of pathogenic T cells. Efalizumab, a human-

ized monoclonal antibody to the CD11a portion of LFA-1, hasbeen withdrawn from the market. Ustekinumab, a humanmonoclonal antibody against IL-12 and 23, is the rst of a newclass of agents that appear highly effective. They block theinammatory pathway at a more proximal point than TNFagents. Neutralizing antibodies may decrease the effectivenessof many of the biologic agents.

Percentage of patients clearing with each drugPublished data allow for various comparisons between bio-logic agents, but as trials are designed by the manufacturerto demonstrate the efcacy of the agent, the endpoints of some

trials differ. In controlled trials of iniximab, the percentaof patients reaching at least 75% improvement from baseliin the psoriasis area and severity index (PASI 75) at weekis about 70% with iniximab 3 mg/kg and 90% with 5 mkg, as compared to 6% with placebo. About 35% of patienreceiving etanercept, 25 mg subcutaneously twice a weachieve PASI 75 at 12 weeks and 45% at 24 weeks. With t50 mg induction dose administered twice a week, about 46of patients achieve PASI 75 at 12 weeks and 50% at 24 weeAbout 14% of patients receiving 12 weekly intramuscular intravenous injections of alefacept will achieve PASI 75, aabout 38% PASI 50. After two 12-injection courses, about 26of patients reach PASI 75 and 55% PASI 50. The onset of actiis somewhat slower than with other agents, but ultimate cleing can be excellent. The data available suggest that abo53% of patients taking 40 mg of adalimumab every othweek achieve PASI 75 by week 12, and about 80% of thotaking 40 mg a week achieve PASI 75. An analysis of 24 radomized controlled trials including 9384 patients suggestthat iniximab was superior to the other agents studied, athat adalimumab was superior to etanercept, 50 mg twweekly, and cyclosporine. Ustekinumab was included in tstudy.

A phase III, parallel, double-blind, placebo-controlled stuof ustekinumab for moderate to severe psoriasis (45 mg 90 mg at weeks 0 and 4, and then every 12 weeks) show

that 67.1% of those who received 45 mg and 66.4% receivi90 mg achieved PASI 75 at week 12. In a second multicentphase III, double-blind, placebo-controlled trial of ustekinmab in patients with moderate to severe psoriasis, 66.7%patients receiving 45 mg and 75.7% receiving 90 mg achievPASI 75.

Rapidity of clearing and relapseThe effects of iniximab are rapid and similar to those achievwith cyclosporine. In contrast to cyclosporine, clinical improvment after three intravenous infusions of iniximab maintained for as long as 6 months in approximately hthe patients. Adalimumab is also rapid in onset, with mapatients demonstrating a response within the rst week treatment. About 15% of patients treated with alefacept w

maintain benets for more than 6 months.

RisksTNF agents may induce ares of psoriasis through upregution of plasmacytoid dendritic cells. This may be a class effeThe biologic agents all suppress the normal immune responIniximab has been associated with reactivation of tubercusis, demyelinating disease, and serious systemic opportunisinfection. It may also lose its effect because of neutraliziantibodies. Methotrexate or azathioprine may be needed concomitant therapy to reduce the incidence of neutraliziantibodies and infusion reactions. Even though adalimumis a fully human antibody, it may also induce an antiboresponse. Serious infections have been reported in patiewith rheumatoid arthritis treated with this agent. Etanerce

has been associated with infection, onset, or exacerbationmultiple sclerosis, vasculitis, and lupus erythematosus-lmanifestations. All these manifestations are rare, and may nbe statistically increased from the general population. A sin12-week course of alefacept does not appear to impair primaor secondary antibody responses to a neoantigen or memoresponses to a recall antigen, but roughly 10% of patients hato interrupt therapy because CD4 counts fall below 250/mmand CD4 counts must be monitored with this agent. Manythe reported complications, such as lymphoma, demyelinatidisease, and infection, are not unique to any one bioloagent.



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10The National Psoriasis Foundation has endorsed a recom-

mendation that all patients be screened for latent tuberculosisinfection prior to any immunologic therapy. They recommenddelaying immunologic therapy until prophylaxis for latenttuberculosis infection is completed, although they note thatpatients with severe disease may be treated after 12 monthsof prophylaxis. IFN- assays have greater specicity thantuberculin skin tests and are being used along with imagingstudies to conrm tuberculosis in patients with positive skintests.

Combination therapyIn more severe forms of psoriasis a combination of treatmentmodalities may be employed. In treating patients with metho-trexate, for example, concomitant topical agents may be usedto minimize the dose. Methotrexate has been combined withiniximab to reduce the incidence of neutralizing antibodies,and has been used with acitretin in managing patients withsevere, generalized pustular psoriasis. The use of PUVA andretinoids is called Re-PUVA and has been studied extensively.Acitretin has been combined with biologic agents to treatrefractory psoriasis. Combination systemic therapy has thepotential to reduce overall toxicity if the toxicities of eachagent are different. However, new regimens should be usedwith caution because the potential for cumulative toxicity ordrug interaction exists.

Alternative therapiesAlternative therapies for psoriasis include mycophenolatemofetil, sulfasalazine, paclitaxel, azathioprine, fumaricacid esters, climatotherapy, and Grenz ray therapy. Naildisease can respond to systemic agents, topical retinoids, localtriamcinolone injections, and topical 5-uorouracil. The latteragent can cause onycholysis if applied to the free edge ofthe nail.

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Reactive arthritis with conjunctivitis/urethritis/diarrhea (Reiter syndrome)

Reiter syndrome is a characteristic clinical triad of urethritis,conjunctivitis, and arthritis. The disease occurs chiey inyoung men of HLA-B27 genotype, generally following a boutof urethritis or diarrheal illness. Systemic involvement caninclude the gastrointestinal tract, kidneys, central nervoussystem, and cardiovascular system. As few patients presentwith the classic triad, the American College of Rheumatologyrecognizes criteria for limited manifestations of the syndrome,including peripheral arthritis of more than 1 months durationin association with urethritis, cervicitis, or bilateralconjunctivitis.

Hans Reiter was a Nazi war criminal, involved with orhaving knowledge of involuntary sterilization, as well as astudy of an experimental typhus vaccine that resulted in hun-dreds of deaths of concentration camp internees. Severalauthors have suggested that he no longer be afforded the rec-ognition of using his name to designate the syndrome.

Clinical features

Any part of the triad may occur rst, often accompanied byfever, weakness, and weight loss. Although the inciting ure-thritis may be bacterial, later manifestations include a nonbac-terial urethritis with painful urination and pyuria. Cystitis,prostatitis, and seminal vesiculitis may be accompaniments.Vulvar ulceration has been reported. About one-third ofpatients develop conjunctivitis, which may be bulbar, tarsal,or angular. Keratitis is usually supercial and extremelypainful. Iritis is common, especially in recurrent cases.Infrequently, optic neuritis may occur. An asymmetric arthri-tis affects peripheral joints, especially those that are weight-bearing. Its onset is usually sudden. Pain in one or both heelsis a frequent symptom. Sacroiliitis may develop in up to two-thirds of patients, most of whom are of HLA-B27 type.

The skin involvement commonly begins with small, guttate,hyperkeratotic, crusted or pustular lesions of the genitals (Fig.10-12), palms, or soles. Involvement of the glans penis (balani-tis circinata) occurs in 25% of patients. Lesions on the soles andtrunk often become thickly crusted or hyperkeratotic. Theeruption on the soles is known as keratoderma blennorrhagi-cum (Fig. 10-13), and occurs in 10% of patients. The buccal,palatal, and lingual mucosa may show painless, shallow, rederosions. The nails become thick and brittle, with heavy sub-ungual keratosis. Children are much more likely to have thepost-dysenteric form, often with conjunctivitis and arthritis asthe most prominent complaints.

Fig. 10-12 Genital involvement in reactive arthritis.

Fig. 10-13Keratoderma blennorrhagicum.

The syndrome generally follows an infectious urethritis diarrheal illness. Implicated organisms include ChlamydShigella, Salmonella, Yersinia, Campylobacter, Ureaplasma, BorreCryptosporidium, gonococci, and bacillus CalmetteGu

(BCG). Chlamydia trachomatisand Ureaplasma urealyticumhabeen isolated from the synovial uid of affected joints, asome patients respond to antibiotic therapy. Reiter syndrohas also been observed in HIV disease, but may not be direcrelated to the virus, as it frequently occurs under treatmentthe immune response improves. The disease has also betriggered by adalimumab and leunomide in the setting ankylosing spondyloarthropathy and Crohn disease.

The syndrome involves both infection and the resultiimmunologically mediated tissue injury in a geneticapredisposed patient. HLA-B27 is present in about 80% cases. A positive family history is often noted.



200


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10of the remaining cases. Some patients have responded to sulfapyridine therapy. Acitretin, narrow-band UVB ptherapy, colchicine, azithromycin, biologicals, and tetracwith niacinamide may also be effective.

BediMKL:Successful treatment of long-standing, recalcitrant subcorneal pustular dermatosis with etanercept. Skinmed 2007 Sep6(5):245247.BliziotisI, et al:Regression of subcorneal pustular dermatosis tyIgA pemphigus lesions with azithromycin. J Infect 2005; 51:E31.ChengS, et al:Subcorneal pustular dermatosis: 50 years on. ClinDermatol 2008; 33(3):229233.HowellSM, et al:Rapid response of IgA pemphigus of the subco

pustular dermatosis subtype to treatment with adalimumab andmycophenolate mofetil. J Am Acad Dermatol 2005; 53:541.

Eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis (EPF) was rst describ1970 by Ofuji but is also referred to as sterile eosinopustulosis. It occurs more commonly in males, and is mreported in Asia. The mean age of onset is 35. It is charized by pruritic, follicular papulopustules that m12 mm. The lesions tend to be grouped and plaquesmonly form. New lesions may form at the edges of the plleading to peripheral extension, while central clearingplace. The most frequent site is the face, particularly ovcheeks. The trunk and upper extremities are comaffected, and 20% have palmoplantar pustules. The distion is commonly asymmetrical, and the typical couone of spontaneous remissions and exacerbations lseveral years. The condition must be distinguishedHIV-associated eosinophilic folliculitis, which is discin Chapter 19. A similar condition has occurred in assocwith hepatitis C virus infection, with allopurinol, and dpregnancy.

Histologically, there is spongiosis and vesiculation follicular infundibulum and heavy inltration with ephils. Follicular mucinosis may be present. There is a peral eosinophilia in half the cases, and pulmonary eosinohas been described. The cause is unknown; but num

studies have implicated chemotactic substances, ICAM-cyclooxygenase-generated metabolites. Tryptase-positivchymase-negative mast cells have also been implicated

Indomethacin is effective in the vast majority of paTopical and intralesional corticosteroids, clofazimine, cycline, isotretinoin, UVB therapy, dapsone, colchcyclosporine, and cetirizine have also been reporteffective.

Childhood cases have been described. This subset from the typical cases in Asian males. Pediatric padevelop sterile pustules and papules preferentially ovscalp, although scattered clusters of pustules may occuthe trunk and extremities. Leukocytosis and eosinoare often present. Recurrent exacerbations and remiusually occur, with eventual spontaneous resolution.

potency topical steroids are the treatment of choice in pepatients.

GulU, et al:Eosinophilic pustular folliculitis: the first case associawith hepatitis C virus. J Dermatol 2007; 34(6):397399.KusS, et al:Eosinophilic pustular folliculitis (Ofujis disease)exacerbated with pregnancies. J Eur Acad Dermatol Venereol 2020(10):13471348.SufyanW, et al:Eosinophilic pustular folliculitis. Arch Pathol Lab 2007; 131(10):15981601.YonedaK, et al:Eosinophilic pustular folliculitis associated withpulmonary eosinophilia. J Eur Acad Dermatol Venereol 2007 Se21(8):11221124.

Peripheral leukocytosis of 10 00020 000/mm3and elevatedsedimentation rate are the most consistent ndings. There isno specic test for Reiter syndrome.

The differential diagnosis includes rheumatoid arthritis,ankylosing spondylitis, gout, psoriatic arthritis, gonococcalarthritis, acute rheumatic fever, chronic mucocutaneous can-didiasis, and serum sickness. The presence of associatedmucocutaneous lesions establishes the diagnosis. Some casesof Lyme disease overlap with the syndrome. Individual skinlesions may be indistinguishable from those in psoriasis.Hyperkeratotic lesions generally have a thicker scale crustthan most psoriatic plaques, but are otherwise identical.

Mucocutaneous lesions are generally self-limited and clearwith topical steroids. Joint disease is managed with rest andNSAIDs. Antibiotics, such as doxycycline, have been effectivein some cases. Immunosuppressive agents, such as methotrex-ate, are commonly employed for refractory joint disease.Iniximab has been successful in treating severe disease.Refractory skin lesions are treated like refractory psoriasis,and severely affected patients have responded to acitretin orcyclosporine.

PanushRS, et al:Retraction of the suggestion to use the term Reiterssyndrome sixty-five years later: the legacy of Reiter, a war criminal,should not be eponymic honor but rather condemnation. ArthritisRheum 2007; 56(2):693694.Sampaio-BarrosPD, et al:Frequency of HLA-B27 and its alleles inpatients with Reiter syndrome: comparison with the frequency in otherspondyloarthropathies and a healthy control population. Rheumatol Int2008; 28(5):483486.ThielenAM, et al:Reiter syndrome triggered by adalimumab(Humira) and leflunomide (Arava) in a patient with ankylosingspondylarthropathy and Crohn disease. Br J Dermatol 2007;156(1):188189.TownesJM:Reactive arthritis after enteric infections in the UnitedStates: the problem of definition. Clin Infect Dis 2010; 50(2):247242.WallaceDJ, et al:The physician Hans Reiter as prisoner of war inNuremberg: a contextual review of his interrogations (19451947).Semin Arthritis Rheum 2003; 32:208.YuD, et al:Role of bacteria and HLA-B27 in the pathogenesis ofreactive arthritis. Rheum Dis Clin North Am 2003; 29:21.

Subcorneal pustular dermatosis(SneddonWilkinson disease)

In 1956, Sneddon and Wilkinson described a chronic pustulardisease, which occurred chiey in middle-aged women. Thepustules are supercial and arranged in annular and serpigi-nous patterns, especially on the abdomen, axillae, and groins.Cultures from the pustules are sterile. Oral lesions are rare.Some cases are associated with a monoclonal gammopathy(usually IgA). The condition is chronic, with remissions ofvariable duration.

Histologically, the pustules form below the stratum corneum,as in impetigo. Acantholysis is absent, but spongiform pus-tules may be noted in the upper epidermis. The histologicdifferential diagnosis includes pustular psoriasis, and super-

cial fungal and bacterial infections. Some cases will showupper epidermal intercellular IgA staining.

IgA pemphigus shows signicant overlap with subcornealpustular dermatosis. Presentations of IgA pemphigus includesubcorneal pustular dermatosis and intraepidermal neu-trophilic IgA dermatosis types. Using immunoblotting tech-niques, Hashimoto et al have shown that human desmocollin1 is an autoantigen for the subcorneal pustular dermatosistype of IgA pemphigus.

Localized cases may respond well to topical corticosteroids.Dapsone, 50200 mg/day (for an adult), is effective for most



Recalcitrant palmoplantar eruptions

Dermatitis repens

Dermatitis repens, also known as acrodermatitis continua andacrodermatitis perstans, is a chronic inammatory disease ofthe hands and feet. It usually remains stable on the extremities,but in rare cases generalized pustular ares may occur. Thedisease usually begins distally on a digit, either as a pustulein the nailbed or as a paronychia. Extension takes place byeruption of fresh pustules with subsequent hyperkeratosis and

crusting. The disease is usually unilateral in its beginning andasymmetrical throughout its entire course. As the diseaseprogresses, one or more of the nails may become dystrophicor oat away on lakes of pus. Anonychia is common in chroniccases. Some have used the term dermatitis repens to refer tomore indolent involvement of the distal ngers.

Involvement of the mucous membranes may occur, evenwhen the eruption of the skin is localized. Painful, circular,white plaques surrounded by inammatory areolae are foundon the tongue and may form a brinous membrane. Fissuredor geographic tongue may occur.

Histologically, intraepithelial spongiform pustules identicalto those of psoriasis are seen in the acute stage. Later stagesshow hyperkeratosis with parakeratosis or atrophy.

Numerous treatment options have been used, including

topical corticosteroids, calipotriene, dapsone, sulfapyridine,methotrexate, PUVA, acitretin, cyclosporine, and topicalmechlorethamine. The decision regarding which agent to useshould take into consideration the severity of disease, and thepatients age and functional impairment.

Palmoplantar pustulosis (pustular psoriasisof the extremities)

Chronic palmoplantar pustulosis is essentially a bilateral andsymmetrical dermatosis (Fig. 10-14). The favorite locations arethe thenar or hypothenar eminences or the central portion ofthe palms and soles. The patches begin as erythematous areasin which minute intraepidermal pustules form. At the begin-

ning these are pinhead-sized; then they may enlarge and coa-lesce to form small lakes of pus. As the lesions resolve, denudedareas, crusts, or hyperkeratosis may persist. Palmoplantar pus-tulosis is strongly associated with thyroid disorders and ciga-rette smoking. Medications such as lithium, which aggravatepsoriasis, have also been reported to induce palmoplantar pus-tular psoriasis.

In 1968, Kato described the rst case of bilateral clavicularosteomyelitis with palmar and plantar pustulosis. In 1974,Sonozaki described persistent palmoplantar pustulosis andsternoclavicular hyperostosis. These conditions belong to thespectrum of skin and joint involvement designated by Kahnas the SAPHO syndrome (synovitis, acne, pustulosis, hyper-ostosis, and osteitis). Common features include palmoplantarpustulosis, acneiform eruption, and pain and swelling of a

sternoclavicular joint, or sternomanubrial or costochondraljunctions. There is shoulder, neck, and back pain, and limita-tion of motion of the shoulders and neck is common. Brachialplexus neuropathy and subclavian vein occlusion may occur.The lumbar spine and sacroiliac joints are usually spared.Chronic multifocal osteomyelitis in children may be a pediat-ric variant. Others have described an association betweenpalmoplantar pustulosis and arthritis or osteitis. SAPHOsyndrome may coexist with features of Behets disease. Theknees, spine, and ankles may be involved. Ivory vertebraehave been described.

Fig. 10-14 A. Plantas pustulosis; B. Pustules and hyperkeratosis atypical.

A

B

The disease is commonly resistant to treatment. Topical stoids, retinoids, calcipotriene, or macrolactams are of sobenet. Acitretin is generally extremely effective at a dose1 mg/kg/day, although rebound occurs more quickly thwith etretinate. Low-dose cyclosporine, in doses ranging fro1.25 to 5 mg/kg/day, has also been very effective, but it is nsuitable for long-term treatment. Dapsone, colchicine, leunmide, and mycophenolate mofetil may be effective. O8-methoxypsoralen and high-intensity UVA irradiation soak PUVA can both be helpful, and Grenz ray therapy cinduce prolonged remissions in some patients. Chronic ostemyelitis in SAPHO syndrome has been reported to respondbisphosphonates.

BlancoJF, et al:Ivory vertebra and palmoplantar pustulosis. JRheumatol 2007 Apr; 34(4):896899.Hurtado-NedelecM, et al:Characterization of the immune response inthe synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO)syndrome. Rheumatology (Oxford) 2008; 47(8):11601167.NikkelsAF, et al:Breaking the relentless course of Hallopeausacrodermatitis by dapsone. Eur J Dermatol 1999; 9:126.Piquero-CasalsJ, et al:Using oral tetracycline and topical betamethasone valerate to treat acrodermatitis continua of Hallopeau. Cutis2002; 70:106.ProenaNG:Acropustulosis repens. Int J Dermatol 2006;45(4):389393.SkovL, et al:IL-8 as antibody therapeutic target in inflammatorydiseases: reduction of clinical activity in palmoplantar pustulosis. JImmunol 2008; 181(1):669679.YabeH, et al:Two cases of SAPHO syndrome accompanied by classfeatures of Behets disease and review of the literature. ClinRheumatol 2008; 27(1):133135.



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10

Fig. 10-15 Acropustulosis of infancy. (Courtesy of Curt SamlasMD)

Pustular bacterid

Pustular bacterid was rst described by George Andrews. It ischaracterized by a symmetric, grouped, vesicular, or pustulareruption on the palms and soles, marked by exacerbations andremissions over long periods. Andrews regarded the discov-ery of a remote focus of infection, and cure on its elimination,as crucial to the diagnosis.

The primary lesions are pustules. Tiny hemorrhagic punctaintermingled with the pustules are frequently seen. Whenlesions are so numerous as to coalesce, they form a honeycomb-like structure in the epidermis. The disease usually begins onthe mid-portions of the palms or soles, from which it spreadsoutwardly until it may eventually cover the entire exoraspects of the hands and feet. There is no involvement of thewebs of the ngers or toes, as in tinea pedis.

When the eruption is fully developed, both palms and solesare completely covered, and the symmetry is pronounced.During fresh outbreaks, the white blood count may show aleukocytosis that ranges from 12 000 to 19 000/mm 3with 6580% neutrophils. As a rule, scaling is present in fully evolvedlesions, and the scales are adherent, tough, and dry. Duringexacerbations, crops of pustules or vesicles make their appear-ance, and there is often severe itching of the areas. Tendernessmay be present. Many regard this condition as a variant ofpsoriasis, triggered by infection.

Bacharach-BuhlesM, et al:The pustular bacterid (Andrews): are thereclinical criteria for differentiating from psoriasis pustulosa palmaris etplantaris? Hautarzt (Germany) 1993; 44:221.WorretWI:Pustular bacterid of Andrews. Am J Dermatopathol 1985;7:200.

Infantile acropustulosis

Infantile acropustulosis is an intensely itchy vesicopustulareruption of the hands and feet (Fig. 10-15). Most cases beginby 10 months of age. Lesions often predominate at the edgesof the palms and soles. Individual crops of lesions clear in afew weeks, but recurrences may continue for months or years.Scabies, tinea, and herpetic infection can produce similarlesions, and must be excluded.

Histologically, a subcorneal pustule with neutrophils isnoted. Eosinophils may be numerous. As the lesions are easilypunctured to produce smears of the inammatory cells, biop-sies are seldom employed.

Fig. 10-1Seborrheic dermatitis.

Fig. 10-2Seborrheic dermatitis involving the chest and axillae.

Fig. 10-3Psoriasis.

Fig. 10-4Psoriasis plaque, red plaque with silver scale on the kn

Fig. 10-5Inverse psoriasis.

Fig. 10-6Nail pitting and distal onycholysis in psoriasis.

Fig. 10-7Fissured and geographic tongue in a patient withgeneralized pustular psoriasis.

Fig. 10-8Nailbed involvement in acrodermatitis continua.

Fig. 10-9Anonychia in acrodermatitis continua.

Fig. 10-10Erythrodermic psoriasis.

Fig. 10-11Hyperkeratotic lesions of the reactive arthritis syndrom

Fig. 10-12Plantar pustulosis.

Fig. 10-13Psoriasis.

Bonus images for this chapter can be found online

http://www.expertconsult.com

Lesions often respond to topical corticosteroids. Refrlesions may respond to dapsone at doses of 12 mg/kg

VicenteJ, et al:Are eosinophilic pustular folliculitis of infancy andinfantile acropustulosis the same entity? Br J Dermatol 1996; 13WagnerA:Distinguishing vesicular and pustular disorders in theneonate. Curr Opin Pediatr 1997; 9:396.

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Chapter 10 Seborrheic Dermatitis, Psoriasis, Recalcitrant Palmoplantar Eruptions, Pustular Dermatitis, And Erythroderma