Papulosquamous Disorders: Papulosquamous Disorders: Seb Derm, Psoriasis, Palmoplantar, Pust Seb Derm, Psoriasis, Palmoplantar, Pust

Derm, ErythrodermaDerm, Erythroderma

Chris Weyer D.O.Chris Weyer D.O.

Dermatology Resident, PGY-1Dermatology Resident, PGY-1

Northeast Regional Medical Northeast Regional Medical CenterCenter

Seborrheic DermatitisSeborrheic Dermatitis

2-5 % of the population2-5 % of the population Chronic, superficial, inflammatory disease Chronic, superficial, inflammatory disease

caused by the yeast, caused by the yeast, Malassezia globosaMalassezia globosa Most commonly affects the scalpMost commonly affects the scalp Also, affects the eyebrows, ears, eyelids, Also, affects the eyebrows, ears, eyelids,

nasolabial creases, lips, sternal area, nasolabial creases, lips, sternal area, axillae, submammary folds, umbilicus, axillae, submammary folds, umbilicus, groin, and gluteal creasegroin, and gluteal crease

Characterized by itchy, pink, yellow or Characterized by itchy, pink, yellow or erythematous patches that may be dry, erythematous patches that may be dry, moist, or greasymoist, or greasy

Infantile Seborrheic Infantile Seborrheic DermatitisDermatitis

Presents around 1 Presents around 1 week after birthweek after birth

AKA “Cradle cap”AKA “Cradle cap” Superimposed Superimposed

Candida Candida may occurmay occur

Adult Seborrheic Adult Seborrheic DermatitisDermatitis

Erythematous, Erythematous, greasy yellow scalegreasy yellow scale

SymmetricalSymmetrical If extensive, think If extensive, think

about about immunosuppressioimmunosuppression: HIV, diabetes, n: HIV, diabetes, and Parkinson’s and Parkinson’s diseasedisease

Histology of Seb DermHistology of Seb Derm Epidermis is acanthoticEpidermis is acanthotic Focal scale crust Focal scale crust

adjacent to follicular adjacent to follicular ostiaostia

Slight spongiosisSlight spongiosis Adult seb derm has Adult seb derm has

superficial perivascular superficial perivascular & perifollicular & perifollicular lymphocytic infiltrate lymphocytic infiltrate composed mainly of composed mainly of lymphocyteslymphocytes

Older lesions show Older lesions show irregular acanthosis & irregular acanthosis & focal parakeratosisfocal parakeratosis

Histo features are Histo features are combination of combination of psoriasis & spongiotic psoriasis & spongiotic dermderm

Treatment-Adolescent & Treatment-Adolescent & AdultAdult

Regular use of medicated shampoos -- over-Regular use of medicated shampoos -- over-the-counter: sulfide, tar, zinc, pyrithionatethe-counter: sulfide, tar, zinc, pyrithionate

Nizoral shampoo & creamNizoral shampoo & cream Loprox shampoo & solutionLoprox shampoo & solution Elidel creamElidel cream Corticosteroids for quick control or flaresCorticosteroids for quick control or flares Key: leave shampoo on scalp for 3 minutes Key: leave shampoo on scalp for 3 minutes

daily; apply daily; apply beforebefore going into shower, as going into shower, as patient will not leave on shampoo for 3 patient will not leave on shampoo for 3 minutes while in showerminutes while in shower

Infantile seborrheic Infantile seborrheic dermatitisdermatitis

Responds well to topical therapyResponds well to topical therapy Hydrocortisone 1% on the face & Hydrocortisone 1% on the face &

skin foldsskin folds This may be combined with an This may be combined with an

antifungal agent for skin foldsantifungal agent for skin folds Remove scale after softening with an Remove scale after softening with an

oil preparation or a weak keratolytic oil preparation or a weak keratolytic agentagent

ReassuranceReassurance

PsoriasisPsoriasis

A common, chronic, recurrent, A common, chronic, recurrent, inflammatory disease of the skin inflammatory disease of the skin characterized by round, characterized by round, circumscribed, erythematous, dry, circumscribed, erythematous, dry, scaling plaques of various sizes, scaling plaques of various sizes, covered by grayish white or covered by grayish white or silvery white, imbricated and silvery white, imbricated and lamellar scaleslamellar scales

Predilection for the scalp, nails, Predilection for the scalp, nails, extensor surfaces, elbows, knees, extensor surfaces, elbows, knees, umbilical, and sacral regionumbilical, and sacral region

Psoriasis Psoriasis nail nail changeschangesonycholysis, nail onycholysis, nail pitting, and oil pitting, and oil spot phenomenonspot phenomenon

Psoriasis clinical Psoriasis clinical featuresfeatures

Koebner’s phenomenonKoebner’s phenomenon – the – the appearance of typical lesions of psoriasis appearance of typical lesions of psoriasis at sites of injuryat sites of injury

Auspitz’s sign – pinpoint bleeding when Auspitz’s sign – pinpoint bleeding when the psoriatic scale is forcibly removed, the psoriatic scale is forcibly removed, this occurs because of severe thinning of this occurs because of severe thinning of the epidermis over the tips of the dermal the epidermis over the tips of the dermal papillapapilla

Woronoff ring – is concentric blanching Woronoff ring – is concentric blanching of the erythematous skin at or near the of the erythematous skin at or near the periphery of the healing psoriatic plaqueperiphery of the healing psoriatic plaque

PathogenesisPathogenesis

Hyperproliferative disorder driven Hyperproliferative disorder driven by inflammatory mediatorsby inflammatory mediators

TH1 cytokines- IL-2,6,8,12; IFN-g, TH1 cytokines- IL-2,6,8,12; IFN-g, TNF-aTNF-a

IL-8: accumulation of neutrophilsIL-8: accumulation of neutrophils IL-12: Primary TH1 signal, promotes IL-12: Primary TH1 signal, promotes

IFN-g productionIFN-g production

InheritenceInheritence

Heredity has a high influence, Heredity has a high influence, incidence increases with incidence increases with generationsgenerations

Evidence that susceptibility to Evidence that susceptibility to psoriasis is linked to class I and II psoriasis is linked to class I and II MHC chromo 6MHC chromo 6

Genetic loci: PSORS1 (6); PSORS2 Genetic loci: PSORS1 (6); PSORS2 (17q)(17q)

HLA asso: early onset -Cw6, -B57, HLA asso: early onset -Cw6, -B57, and -DR7 and -DR7 late onset –Cw2late onset –Cw2

Inheritance contInheritance cont

It is also believed that any individual It is also believed that any individual that has that has

-B13 or –B17 has a fivefold risk of -B13 or –B17 has a fivefold risk of developing psoriasis developing psoriasis

pustular psoriasis: HLA-B27pustular psoriasis: HLA-B27 guttate and erythrodermic psoriasis: -guttate and erythrodermic psoriasis: -

B13,-B17B13,-B17 Palmarplantar pust: -B8, -Bw35, -Cw7, Palmarplantar pust: -B8, -Bw35, -Cw7,

-DR3-DR3

Epidemiology of Epidemiology of psoriasispsoriasis

Seen in 1-2% of US populationSeen in 1-2% of US population Equal frequency in both sexesEqual frequency in both sexes Mean age of onset is 27 yearsMean age of onset is 27 years Sunlight may improve psoriasis by Sunlight may improve psoriasis by

decreasing T-cellsdecreasing T-cells Emotional stress, smoking, and Emotional stress, smoking, and

alcohol aggravates psoriasisalcohol aggravates psoriasis Increased incidence of celiacs, Increased incidence of celiacs,

lymphomalymphoma

Drug-induced psoriasisDrug-induced psoriasis

May be induced by many drugs: beta May be induced by many drugs: beta blockers, lithium, and antimalarials, blockers, lithium, and antimalarials, terbinafine, calcium channel terbinafine, calcium channel blockers, captopril, glyburide, and blockers, captopril, glyburide, and lipid lowering agents, such as lipid lowering agents, such as gemfibrozilgemfibrozil

PathologyPathology Regular epidermal Regular epidermal

hyperplasia with long, test-hyperplasia with long, test-tube-shaped rete ridgestube-shaped rete ridges

Thinning of dermal papillaeThinning of dermal papillae Granular layer is thin or Granular layer is thin or

absentabsent Overlying parakeratosisOverlying parakeratosis Small collections of Small collections of

neutrophils (Munro neutrophils (Munro microabcesses) may be microabcesses) may be present in the stratum present in the stratum corneumcorneum

There is a perivascular There is a perivascular mononuclear cell infiltratemononuclear cell infiltrate

Types of PsoriasisTypes of Psoriasis Seborrheic-Like, “sebopsoriasis”Seborrheic-Like, “sebopsoriasis” InverseInverse NapkinNapkin ArthritisArthritis GuttateGuttate Generialized Pustular (von Zumbusch)Generialized Pustular (von Zumbusch) Acro dermatitis Continua of HallopeauAcro dermatitis Continua of Hallopeau Impetigo HerpetiformisImpetigo Herpetiformis Keratoderma BlennorrhagicaKeratoderma Blennorrhagica ErythrodermicErythrodermic

Inverse PsoriasisInverse Psoriasis

Flexural or Flexural or intertrigenous intertrigenous locationlocation

Shiny erythematous plaques in the axilla that lack scale.

““Napkin” PsoriasisNapkin” Psoriasis

Infants 2-8 moInfants 2-8 mo Erythematous sharply demarcated in Erythematous sharply demarcated in

diaper areadiaper area Lesions typically clear w/ topical txLesions typically clear w/ topical tx Infants may be at risk for psoriasis in Infants may be at risk for psoriasis in

adulthoodadulthood

Five clinical patterns of Five clinical patterns of psoriatic arthritispsoriatic arthritis

Asymmetrical DIP joint involvement with Asymmetrical DIP joint involvement with nail damage, 16%nail damage, 16%

Arthritis mutilans with osteolysis of Arthritis mutilans with osteolysis of phalanges and metacarpals, 5%phalanges and metacarpals, 5%

Symmetrical polyarthritis-like rheumatoid Symmetrical polyarthritis-like rheumatoid arthritis, with claw hands, 15%arthritis, with claw hands, 15%

Oligoarthritis with swelling and Oligoarthritis with swelling and tenosynovitis of one or a few hand joints, tenosynovitis of one or a few hand joints, 70%70%

Ankylosing spondylitis alone or with Ankylosing spondylitis alone or with peripheral arthritis, 5%peripheral arthritis, 5%

Radiographic findings of Radiographic findings of psoriatic arthritispsoriatic arthritis

Erosion of terminal phalangeal tufts; Erosion of terminal phalangeal tufts; tapering of phalanges or metacarpals; tapering of phalanges or metacarpals; “cupping’ of proximal ends of phalanges; “cupping’ of proximal ends of phalanges; bony ankylosis; osteolysis of metatarsals; bony ankylosis; osteolysis of metatarsals; predilection for distal and proximal predilection for distal and proximal interphalangeal joints; paravertebral interphalangeal joints; paravertebral ossification; asymmetrical sacroiliitis; and ossification; asymmetrical sacroiliitis; and “bamboo spine”“bamboo spine”

Nearly half the patients with psoriatic Nearly half the patients with psoriatic arthritis have HLA-B27arthritis have HLA-B27

Tx- biologics, MTX, cyclosporin- dz Tx- biologics, MTX, cyclosporin- dz modifying prevent deformitymodifying prevent deformity

Guttate PsoriasisGuttate Psoriasis Usually, occurs as an abrupt eruption Usually, occurs as an abrupt eruption

following an acute infection, such as following an acute infection, such as streptococcal pharyngitis streptococcal pharyngitis

Occurs mostly in patients under age 30Occurs mostly in patients under age 30 Recurrent episodes are likelyRecurrent episodes are likely This type of psoriasis is usually rapidly This type of psoriasis is usually rapidly

responsive to topical steroids or UVBresponsive to topical steroids or UVB Tx strep infxTx strep infx

Guttate Psoriasis

A: Small papules and plaques of guttate psoriasis; note Koebner phenomenon

B: Numerous papules due to Koebner phenomenon after sunburn

Generalized pustular Generalized pustular psoriasispsoriasis

(von Zumbusch)(von Zumbusch) Typical patients have Typical patients have plaque psoriasis and plaque psoriasis and often psoriatic arthritisoften psoriatic arthritis

The onset is sudden, The onset is sudden, with formation of lakes with formation of lakes of pus periungally, on of pus periungally, on the palms, and at the the palms, and at the edge of psoriatic edge of psoriatic plaquesplaques

Pruritus, pain, fever, Pruritus, pain, fever, and malaiseand malaise

Fetid odor developsFetid odor develops

Generalized pustular Generalized pustular psoriasispsoriasis

(von Zumbusch)(von Zumbusch) Etiology unclearEtiology unclear Iodides, coal tar, steroid withdrawal, Iodides, coal tar, steroid withdrawal,

terbinafine, minocycline, terbinafine, minocycline, hydroxychloroquine, acetazolamide, hydroxychloroquine, acetazolamide, and salicylates may trigger the and salicylates may trigger the attacksattacks

May occur in infantsMay occur in infants Acitretin is drug of choice, with a Acitretin is drug of choice, with a

rapid and predictable responserapid and predictable response Isotretinoin, cyclosporine, Isotretinoin, cyclosporine,

methotrexate, dapsonemethotrexate, dapsone

Acrodermatitis Continua Acrodermatitis Continua of Hallopeauof Hallopeau

Acral erythematious plaques with Acral erythematious plaques with nail involvmentnail involvment

Fingernails float away on lakes of Fingernails float away on lakes of pus: anonychiapus: anonychia

Fingers taper to long keratotic Fingers taper to long keratotic pointspoints

Impetigo HerpetiformisImpetigo Herpetiformis

Generalized pustular psoriasis of Generalized pustular psoriasis of pregnancypregnancy

Retinoids not indicatedRetinoids not indicated Prednisone: controversial, may Prednisone: controversial, may

induce pustular flare, also matures induce pustular flare, also matures fetal lungfetal lung

Early delivery strongly encouragedEarly delivery strongly encouraged

Treatment- TopicalsTreatment- Topicals Corticosteroids- class I- 2wks, pulse, occlusion; Corticosteroids- class I- 2wks, pulse, occlusion;

rapid return when stoppedrapid return when stopped Calcipotriene- keratinocyte differentiation; Calcipotriene- keratinocyte differentiation;

plaque and scalp typeplaque and scalp type Tars- oils and shmp; stinks (literally)Tars- oils and shmp; stinks (literally) Macrolactams (tacrolimus, pimicrolimus)- Macrolactams (tacrolimus, pimicrolimus)-

prevent steroid atrophy/acneprevent steroid atrophy/acne Salicylic acid- keratolyticSalicylic acid- keratolytic Tazorac- modulate differentiation/proliferationTazorac- modulate differentiation/proliferation Anthralin- SCAT, supresses neut superoxide, Anthralin- SCAT, supresses neut superoxide,

inhib mono IL-6,8/TNF-ainhib mono IL-6,8/TNF-a

Treatment- LightTreatment- Light

NB-UVB (311-313nm), MED, 70% NB-UVB (311-313nm), MED, 70% responseresponse

Goeckerman Tech- Goeckerman Tech- 2-5% tar bath QD + UV; clear 18 d w/ long 2-5% tar bath QD + UV; clear 18 d w/ long

term responseterm response Ingram- Ingram-

tar bath QD/carbonisdetergens/ UV/ tar bath QD/carbonisdetergens/ UV/ anthalin/talcum/stocking dressinganthalin/talcum/stocking dressing

PUVA- 2x/wk, clear 20-25 tx; risk PUVA- 2x/wk, clear 20-25 tx; risk cataracts, SCC, MMcataracts, SCC, MM

MethotrexateMethotrexate First effective systemic drug for First effective systemic drug for

psoriasis and is the standard for psoriasis and is the standard for systemic therapysystemic therapy

Blocks synthesis of deoxyribonucleic Blocks synthesis of deoxyribonucleic acid, which decreases cell divisionacid, which decreases cell division

Indications- erythroderma, arthritis, Indications- erythroderma, arthritis, pustular, lg BSApustular, lg BSA

MTXMTX

For patients with no risk factors For patients with no risk factors for liver disease, the first liver for liver disease, the first liver biopsy should be obtained at biopsy should be obtained at approximately 1.5g of cumulative approximately 1.5g of cumulative methotrexate methotrexate

Weekly dosing for oral or IM – Weekly dosing for oral or IM – divided in three doses, 12 hours divided in three doses, 12 hours apartapart

IM injections have a much lower IM injections have a much lower risk of hepatotoxicityrisk of hepatotoxicity

CyclosporineCyclosporine

May down-modulate May down-modulate proinflammatory epidermal proinflammatory epidermal cytokinescytokines

Rapid clearing and returnRapid clearing and return Low risk of renal tox w/ <6mo txLow risk of renal tox w/ <6mo tx Follow BP, CrFollow BP, Cr

DietDiet

Most recent trials have demonstrated Most recent trials have demonstrated the antiinflammatory effects of fish oils the antiinflammatory effects of fish oils rich in n-3 polyunsaturated fatty acids rich in n-3 polyunsaturated fatty acids in rheumatoid arthritis, inflammatory in rheumatoid arthritis, inflammatory bowel disease, psoriasis and asthmabowel disease, psoriasis and asthma

Gluten-free dietGluten-free diet Koo J., Lee E., Lee C.S., Lebwohl M.. Koo J., Lee E., Lee C.S., Lebwohl M..

PsoriasisPsoriasis. J Am Acad Dermatol . J Am Acad Dermatol 2004;50:613-22. 2004;50:613-22.

Oral antimicrobial Oral antimicrobial therapytherapy

Recent evidence suggest that Recent evidence suggest that Staphylococcus aureusStaphylococcus aureus and and streptococci secrete a large family of streptococci secrete a large family of exotoxins that are superantigens, exotoxins that are superantigens, producing massive T-cell activationproducing massive T-cell activation

Oral antibiotic for psoriasis patients Oral antibiotic for psoriasis patients infected with these organisms is infected with these organisms is imperativeimperative

RetinoidsRetinoids Treatment with 13-cis-retinoic acid can Treatment with 13-cis-retinoic acid can

produce good results, especially in produce good results, especially in pustular psoriasispustular psoriasis

It is a potent teratogenIt is a potent teratogen Combinations of retinoic acids with Combinations of retinoic acids with

photochemotherapy for chronic plaque photochemotherapy for chronic plaque psoriasis may also be very affectivepsoriasis may also be very affective

Etretinate- long ½ life, EtOH changes Etretinate- long ½ life, EtOH changes acitretinacitretin

Acitretin – avoid pregnancy for up to 3 Acitretin – avoid pregnancy for up to 3 years following cessation of therapyyears following cessation of therapy

DapsoneDapsone

Use is limited largely to Use is limited largely to palmoplantar eruptionspalmoplantar eruptions

22ndnd-3-3rdrd line therapy line therapy

Combination therapyCombination therapy

Patient on methotrexate may have Patient on methotrexate may have their dose minimized with their dose minimized with concomitant topical steroid useconcomitant topical steroid use

PUVA with acitretin, cyclosprine, PUVA with acitretin, cyclosprine, or methotrexateor methotrexate

Topical Dovonex with Acitretin, Topical Dovonex with Acitretin, cyclosporine, methotrexate, and cyclosporine, methotrexate, and phototherapyphototherapy

Biologic AgentsBiologic Agents

Alefacept (Amevive)Alefacept (Amevive) Efalizumab (Raptiva)Efalizumab (Raptiva) Etanercept (Enbrel)Etanercept (Enbrel) Infliximab (Remicaide)Infliximab (Remicaide) Adalimumab (Humira)Adalimumab (Humira) These are indicated for moderate-to-These are indicated for moderate-to-

severe plaque psoriasissevere plaque psoriasis

Journal of the American Academy of DermatologyVolume 53 • Number 2 • August 2005Copyright © 2005 American Academy of Dermatology, Inc. Biologics in psoriasis: A quick reference guide Valencia D. Thomas, MD F. Clarissa Yang, MD Joseph C. Kvedar, MD

Reiter’s syndromeReiter’s syndrome

Triad: urethritis, conjunctivitis, and Triad: urethritis, conjunctivitis, and arthritisarthritis Can’t see, can’t pee, can’t climb a tree!Can’t see, can’t pee, can’t climb a tree!

May also be other features that involve May also be other features that involve the skin , mucous membranes, GI tract, the skin , mucous membranes, GI tract, and cardiovascular systemand cardiovascular system

American Rheumatism Association - American Rheumatism Association - criterion of peripheral arthritis of more criterion of peripheral arthritis of more than 1 month duration, in association than 1 month duration, in association with urethritis and/or cervicitiswith urethritis and/or cervicitis

Young men of HLA-B27 genotypeYoung men of HLA-B27 genotype

Clinical featuresClinical features May also have fever, May also have fever,

weakness, and weight weakness, and weight lossloss

A nonbacterial urethritis A nonbacterial urethritis may develop with may develop with painful and bloody painful and bloody urination and pyuria urination and pyuria

About 1/3 of patients About 1/3 of patients develop conjunctivitisdevelop conjunctivitis

Keratitis is usually Keratitis is usually superficial and very superficial and very painful. Iritis is painful. Iritis is common.common.

Reiter’s syndromeReiter’s syndrome

An asymmetric An asymmetric arthritis afflicts arthritis afflicts synovial joints, synovial joints, especially those that especially those that are weight bearingare weight bearing

Onset is sudden with Onset is sudden with heat and tenderness heat and tenderness and swellingand swelling

Pain in one or both Pain in one or both heels is a frequent heels is a frequent symptomsymptom

Cutaneous lesions of Cutaneous lesions of Reiter’sReiter’s

The skin lesions start as multiple, The skin lesions start as multiple, small, yellowish vesicles that break, small, yellowish vesicles that break, become confluent, and form become confluent, and form superficial erosionssuperficial erosions

Develop frequently on the genitals Develop frequently on the genitals and palmsand palms

Eruption on the glans penis occurs Eruption on the glans penis occurs in 25% of patientsin 25% of patients

Cutaneous featuresCutaneous features The eruption is known The eruption is known

as keratoderma as keratoderma blennorrhagicumblennorrhagicum

Penile lesions are Penile lesions are frequentfrequent Balanitis circinataBalanitis circinata

Characterized by Characterized by perimeatal balanitisperimeatal balanitis

Similar lesion are Similar lesion are seen on the vaginal seen on the vaginal mucosa of womenmucosa of women

EtiologyEtiology

Syndrome has been attributed to many Syndrome has been attributed to many different agents, such as: different agents, such as: Shigella flexneri, Shigella flexneri, Salmonella spp., yersinia spp., Salmonella spp., yersinia spp., Ureaplasma urealyticum, Borrelia Ureaplasma urealyticum, Borrelia burgdorferi, Cryptosporidia,burgdorferi, Cryptosporidia, and and Campylobacter fetusCampylobacter fetus

May be responsible for the infectious May be responsible for the infectious enteritis that precedes onset in a small enteritis that precedes onset in a small percentage of patientspercentage of patients

In cases that follow an infection of the In cases that follow an infection of the genitourinary tract, genitourinary tract, Chlamydia Chlamydia trachomatis trachomatis may be associatedmay be associated

HLA-B27 positivity is present in 80%HLA-B27 positivity is present in 80%

Laboratory findingsLaboratory findings

Non-specificNon-specific A leukocytosis of 10, 000 to 20, A leukocytosis of 10, 000 to 20,

000/mm3 and an elevated sed rate000/mm3 and an elevated sed rate

HistopathologyHistopathology

Identical to psoriasisIdentical to psoriasis

Differential diagnosisDifferential diagnosis

May be confused with rheumatoid May be confused with rheumatoid arthritis, ankylosing spondylitis, arthritis, ankylosing spondylitis, gout, psoriatic arthritis, gonococcal gout, psoriatic arthritis, gonococcal arthritis, acute rheumatic fever, arthritis, acute rheumatic fever, chronic mucocutaneous candidiasis, chronic mucocutaneous candidiasis, and serum sicknessand serum sickness

TreatmentTreatment

Usually mucocutaneous lesions are Usually mucocutaneous lesions are self- limited and clear within a few self- limited and clear within a few monthsmonths

Topical steroidsTopical steroids NSAIDsNSAIDs MethotrexateMethotrexate Cyclosporine Cyclosporine AcitretinAcitretin

Subcorneal pustular Subcorneal pustular dermatosisdermatosis

(Sneddon-Wilkinson Disease(Sneddon-Wilkinson Disease)) Chronic pustular disease, Chronic pustular disease,

which occurs chiefly in which occurs chiefly in middle-aged womenmiddle-aged women

Pustules are superficial and Pustules are superficial and arranged in annular and arranged in annular and serpiginous patterns on the serpiginous patterns on the abdomen, axillae and groinabdomen, axillae and groin

Vesicles may be presentVesicles may be present Cultures from pustules are Cultures from pustules are

sterilesterile Oral lesions are rareOral lesions are rare Some cases occur in Some cases occur in

association with an IgA association with an IgA monoclonal gammopathymonoclonal gammopathy

Desmocollin 1 autoantigen Desmocollin 1 autoantigen for Iga pemphigusfor Iga pemphigus

Subcorneal pustular Subcorneal pustular dermatosisdermatosis

(Sneddon-Wilkinson Disease)(Sneddon-Wilkinson Disease) Dapsone appears to be Dapsone appears to be

effective in most caseseffective in most cases SulfapyridineSulfapyridine AcitretinAcitretin NB-UVBNB-UVB Corticosteroids, Corticosteroids,

colchicine, and TCN colchicine, and TCN with niacinamidewith niacinamide

Without treatment this Without treatment this is a chronic condition is a chronic condition with remissions of with remissions of variable durationvariable duration

Pathology of Sneddon-Pathology of Sneddon-WilkinsonWilkinson

Eosinophilic pustular Eosinophilic pustular folliculitisfolliculitis

Also referred to as Also referred to as sterile sterile eosinophilic pustulosiseosinophilic pustulosis

AKA Ofuji’s diseaseAKA Ofuji’s disease Males, AsiaMales, Asia HIVHIV Characterized by pruritic, Characterized by pruritic,

follicular papulopustules arranged follicular papulopustules arranged in groupsin groups

Histo: eos in or around folliclesHisto: eos in or around follicles

Eosinophilic pustular Eosinophilic pustular folliculitisfolliculitis

Distribution is usually asymmetrical – Distribution is usually asymmetrical – affects face, trunk, and upper affects face, trunk, and upper extremitiesextremities

Cause is unknown – numerous studies Cause is unknown – numerous studies have implicated chemotactic have implicated chemotactic substances, ICAM-1, and substances, ICAM-1, and cyclooxygenase-generated metabolitescyclooxygenase-generated metabolites

Typical course – spontaneous Typical course – spontaneous remissions and exacerbations for a few remissions and exacerbations for a few months to several yearsmonths to several years

Eosinophilic pustular Eosinophilic pustular folliculitisfolliculitis

Dapsone or systemic steroids are the Dapsone or systemic steroids are the treatment of choicetreatment of choice

Success with intralesional steroids, Success with intralesional steroids, clofazimine, minocycline, clofazimine, minocycline, isotretinoin, UVB therapy, isotretinoin, UVB therapy, indomethacin, colchicine, indomethacin, colchicine, cyclosporine, and cetirizinecyclosporine, and cetirizine

Recalcitrant Recalcitrant Palmoplantar EruptionsPalmoplantar Eruptions

Recalcitrant pustular eruptions of Recalcitrant pustular eruptions of the hands and feet are often the hands and feet are often examples of psoriasisexamples of psoriasis

Need to then search for lesions Need to then search for lesions elsewhere on the body(e.g., scalp, elsewhere on the body(e.g., scalp, ears, glans penis)ears, glans penis)

Search also for a family history to Search also for a family history to confirm your suspicionconfirm your suspicion

Dermatitis RepensDermatitis Repens Aka- acrodermatitis continua and Aka- acrodermatitis continua and

acrodermatits perstansacrodermatits perstans It’s a chronic inflammatory disease of It’s a chronic inflammatory disease of

hands and feethands and feet Rarely, can become generalizedRarely, can become generalized Usually, as a pustule or paronychiaUsually, as a pustule or paronychia

Dermatitis RepensDermatitis Repens Occasionally, mucous membranes are Occasionally, mucous membranes are

involvedinvolved Nails are often dystrophic or destroyedNails are often dystrophic or destroyed Lesions cause skin atrophyLesions cause skin atrophy Crusted, eczematoid, and psoriasiform Crusted, eczematoid, and psoriasiform

lesions may occur, and there may be lesions may occur, and there may be moderate itchingmoderate itching

It is essentially unilateral in its beginning It is essentially unilateral in its beginning and asymmetrical throughout its entire and asymmetrical throughout its entire coursecourse

Dermatitis RepensDermatitis Repens HistologyHistology

similar to those seen in psoriasissimilar to those seen in psoriasis the primary lesion is epidermalthe primary lesion is epidermal An intraepithelial spongiform pustule is An intraepithelial spongiform pustule is

formed by infiltration of pmn’sformed by infiltration of pmn’s TreatmentTreatment

topical mechlorethamine, topical steroids, topical mechlorethamine, topical steroids, PUVA, fluorouracil, and sulfapyridinePUVA, fluorouracil, and sulfapyridine

Acitretin, low dose cyclosporine, Acitretin plus Acitretin, low dose cyclosporine, Acitretin plus calcipotriolcalcipotriol

Palmoplantar PustulosisPalmoplantar Pustulosis

AKA pustular psoriasisAKA pustular psoriasis In contrast to dermatitis repens it is In contrast to dermatitis repens it is

essentially bilateral and symmetricalessentially bilateral and symmetrical Locations include: Locations include:

thenar/hypothenar eminences or thenar/hypothenar eminences or central portion of the palms and central portion of the palms and solessoles

Palmoplantar Palmoplantar PustulosisPustulosis

Patches begin as Patches begin as erythematous erythematous areas in which areas in which pustules formpustules form

Start as pinhead-Start as pinhead-sized, enlarge sized, enlarge and coalesce to and coalesce to form small lakes form small lakes of pusof pus

In the course of a week, In the course of a week, they tend to dry up, they tend to dry up, leaving punctate brown leaving punctate brown scabs that eventually scabs that eventually exfoliateexfoliate

Stages of quiescence and Stages of quiescence and exacerbation characterize exacerbation characterize the conditionthe condition

Meds, such as lithium, Meds, such as lithium, have been reported to have been reported to induceinduce

Palmoplantar PustulosisPalmoplantar Pustulosis Nails may become malformed, ridged, stippled, Nails may become malformed, ridged, stippled,

pitted and discoloredpitted and discolored May be associated with psoriasis vulgarisMay be associated with psoriasis vulgaris Some regard palmoplantar pustulosis as a form Some regard palmoplantar pustulosis as a form

of psoriasis, while others consider it a separate of psoriasis, while others consider it a separate entityentity

Female predominance; lack of seasonal variation; Female predominance; lack of seasonal variation; different histopathologic features anddifferent histopathologic features and

Associated with thyroid disorders and cigarette Associated with thyroid disorders and cigarette smokingsmoking

Palmoplantar PustulosisPalmoplantar Pustulosis May be predisposed May be predisposed

to joint disease and to joint disease and possibly SAPHO possibly SAPHO syndrome-syndrome-SSynovitis, ynovitis, AAcne, cne, PPustulosis, ustulosis, HHyperostosis and yperostosis and OOsteoarthritissteoarthritis

It’s resistant to most It’s resistant to most treatmentstreatments

Acitretin is reportedly Acitretin is reportedly effective(1mg/kg/day)effective(1mg/kg/day)

Low-dose cyclosporine Low-dose cyclosporine (1.25mg/kg/day-(1.25mg/kg/day-3.75mg/kg/day)3.75mg/kg/day)

Intramuscular Intramuscular Kenalog (40-Kenalog (40-60mg)may be 60mg)may be effective for short-effective for short-term reliefterm relief

Palmoplantar PustulosisPalmoplantar Pustulosis

Pustular Pustular BacteridBacterid

Characterized by a symmetric, grouped, vesicular Characterized by a symmetric, grouped, vesicular or pustular eruption on palms and solesor pustular eruption on palms and soles

Marked by exacerbations and remissions over long Marked by exacerbations and remissions over long periodsperiods

No involvement of webs of fingers or toes or No involvement of webs of fingers or toes or flexion creases of toes flexion creases of toes

WBC may be elevatedWBC may be elevated Scaling is usually presentScaling is usually present Etiology is thought to be a remote focus of Etiology is thought to be a remote focus of

infection; infection needs to be treated before infection; infection needs to be treated before resolution will occurresolution will occur

Juvenile Plantar Juvenile Plantar DermatosisDermatosis

• Usually begins as a patchy, symmetrical, smooth, Usually begins as a patchy, symmetrical, smooth, red, glazed macule on great toes, sometimes with red, glazed macule on great toes, sometimes with fissuring and desquamation in children aged 3-13fissuring and desquamation in children aged 3-13

• Toe webs are rarely involved; fingers may beToe webs are rarely involved; fingers may be• Histologically, there is psoriasiform acanthosis Histologically, there is psoriasiform acanthosis

and a sparse, lymphocytic infiltrate in the upper and a sparse, lymphocytic infiltrate in the upper dermisdermis

• Spongiosis is commonly presentSpongiosis is commonly present• Tx: bed rest, cotton socks and topical steroidsTx: bed rest, cotton socks and topical steroids• Spontaneous resolution within 4 yrs is the ruleSpontaneous resolution within 4 yrs is the rule

Infantile AcropustulosisInfantile Acropustulosis

Intensely itchy vesicopustular Intensely itchy vesicopustular eruption of hands and feeteruption of hands and feet

Begins at any age up to 10 months, Begins at any age up to 10 months, clearing in a few weeks and recurring clearing in a few weeks and recurring repeatedly until final resolution at 6 – repeatedly until final resolution at 6 – 36 months of age36 months of age

Dapsone at 2mg/kg/day may helpDapsone at 2mg/kg/day may help Potent topical steroids aid in Potent topical steroids aid in

symptomatic reliefsymptomatic relief

Should prompt an extensive workup Should prompt an extensive workup to eliminate serious infectious to eliminate serious infectious causes (i.e., Tzanck prep, gram causes (i.e., Tzanck prep, gram stain, KOH prep of pustule)stain, KOH prep of pustule)

Some suspect that this condition Some suspect that this condition may be a persistent reaction to prior may be a persistent reaction to prior scabiesscabies

Infantile AcropustulosisInfantile Acropustulosis

Acropustulosis of infancyAcropustulosis of infancy

THE ENDTHE END