P7102Propylthiouracil-induced antineutrophil cytoplasmic antibody (anti-MPOand anti-PR3) positive leukocytoclastic vasculitis in a patient with Gravesdisease presenting as purpura fulminans

Carol Cunningham, MBBCh, Salford Royal Hospital, Manchester, UnitedKingdom; Professor Louise Barnes, MBBCh, St. James’s Hospital, Dublin, Ireland

A 44-year-old woman presented with new-onset localised bullous purpura. She wasdiagnosed with Graves thyroid and eye disease 1 year previously and was started onpropylthiouracil (PTU). She did not take any other medications. After commence-ment of PTU, she developed bullous purpura of the left calf. Over the followingweek, purpura becamemore extensive and shewas admitted to hospital and seen bythe dermatology team. Multiple patches of palpable purpura with a jagged borderwere scattered on thighs, legs, buttocks, and on themaxillary areas, giving the rash a‘‘geographic’’ appearance. Lesions had black necrotic centers, an erythematous rim,and central hemorrhagic bullae with background livedo reticularis on thighsbilaterally. Although she had no symptoms or signs of meningism or septicaemia,she was presumptively treated with cefotaxime because her rash mimicked purpurafulminans which can be secondary to meningococcal septicaemia (cefotaxime wasdiscontinued when meningococcal PCR was reported negative). Differential diag-nosis included infections (bacterial and viral), HenocheSchonlein purpura, anddrug-induced vasculitis. The latter seemed initially less likely because of the 10-month lag between initiation of PTU and rash onset. Skin biopsy showedleukocytoclastic vasculitis. Both myeloperoxidase antibody (anti-MPO) (52.2EU/L) and anti-proteinase 3 antibody (anti-PR3) (90.2 EU/L) were positive.Vasculitic, thrombophilia and coagulation screens revealed no other significantabnormality. Later, ANCA remained strongly positive (1:160) with persistentlypositive antibodies to MPO and PR3. Vasculitis screen and clinical features wereotherwise negative, suggesting a localized PTU induced cutaneous vasculitis.Currently, sites of previously ulcerated purpura are healing with marked scarformation and no new areas of purpura. She underwent thyroidectomy because oftreatment failure with lithium carbonate. PTU-induced ANCA-positive vasculitis israre and most cases have been reported in nondermatologic medical journals.Management involves discontinuing PTU and treatment of lesions with topical ororal steroids. Cyclophosphamide or plasmapheresis may be considered if vasculitisis unresponsive to steroid or as an adjuvant therapy if disease is severe.

APRIL 20

cial support: None identified.

Commer

P6773The emerging phenotype of ovarian driven autoimmunity: Annular,lateralizing, and microvesicular skin diseases in women

Ramya Kollipara, University of Missouri, Kansas City, Kansas City, MO, UnitedStates; Chetna Arora, University of Missouri, Kansas City, Kansas City, MO, UnitedStates; Colleen Reisz, University of Missouri, Kansas City, Kansas City, MO,United States

Clinicians of all stripes use pattern recognition skills to process clinical situationsthat contain recognized and unrecognized components. Dermatology uses patternrecognition skills with particular emphasis on morphology and distribution. Wehave selected 4 cases of skin disease in women that feature annular or vesicularmorphology and distributive features that favor one side over the other. We discussthe utility of these clinical findings in the context of what is new in the complex andstage specific science of fertility. The ovary’s sentinel role in allergic and autoim-mune diseases may have easily recognized features in the skin. Sexual dimorphism inthe innate and adaptive immune systems has long been observed, with femalesgenerally demonstrating a more vigorous immune response than males and apropensity to develop cutaneous autoimmune diseases. Sex hormones, includingestrogen, progesterone, and prolactin, contribute to this dimorphism by promotingT and B lymphocyte autoreactivity and favoring the humoral arm of the adaptiveimmune response via intracellular receptors located on immune cells. Conversely,the immune system plays a significant role in ovarian physiology and pathology.Intricate pathways (ie, PTEN/Pi3K, ERK, FSH receptor dominance) coexist in orderto engage a single oocyte each cycle, while simultaneously sustaining the ovarianreserve. Recent research has uncovered the presence of autoantibodies directedagainst the zona pellucida and follicle stimulating hormone that contribute tofollicular atresia and subsequent premature ovarian failure. In addition, vigorousproinflammatory, vasoactive, and pigment-related cytokines emerge in the demise ofthe corpus luteum. Abnormal hormonal patterns secondary to immune attack canresult in recurring and reinforcing patterned cutaneous disease. We propose thatannular, lateralizing, and microvesicular lesions are important manifestations ofinternal inflammation. Recognition and accurate categorization of these findingsmay provide direction in diagnosis and treatment that will lead to better overallimmune and reproductive health.

cial support: None identified.

Commer

13

P6652The value of trichoscopy in differential diagnosis of isolated scalp lesionsin pemphigus

Marta Kurzeja, MD, Department of Dermatology CSK MSW, Warsaw, Poland;Adriana Rakowska, MD, PhD, Department of Dermatology CSK MSW, Warsaw,Poland; Lidia Rudnicka, MD, PhD, Department of Dermatology CSK MSW,Warsaw, Poland; Malgorzata Olszewska, MD, PhD, Department of DermatologyWarsaw Medical University, Warsaw, Poland

Differential diagnosis of scalp lesions in the course of autoimmune bullous diseasesmay be a diagnostic challenge when they are the first or sole manifestation ofdisease. The aim of the study was to analyze characteristic trichoscopy (dermatos-copy of hair and scalp) features of the three most common autoimmune bullousdiseases involving the scalp: pemphigus, pemphigoid, and dermatitis herpetiformis,and to evaluate whether trichoscopy may be helpful as an in-office supplementarydiagnostic method for differential diagnosis of isolated scalp lesions in pemphigus.The study included 30 patients with pemphigus (18with pemphigus vulgaris and 12with pemphigus foliaceus) and 32 patients with other bullous diseases. Trichoscopywas performed with a digital videodermatoscope. The working magnification was20-fold and 70-fold. In both, pemphigus vulgaris and pemphigus foliaceus,trichoscopy shows red, sharply demarcated, polygonal hemorrhagic areas, elon-gated serpentine blood vessels, dotted vessels with whitish halo, large yellow dotswith whitish halo (‘‘fried egg sign’’), and white lamellar structures. In cicatricialpemphigoid, trichoscopy showed white areas of epidermal detachment, largeyellow dots, and confluent white areas of end-stage fibrosis. In a single case ofimmunopathologically confirmed pemphigoid, follicular units with tufted hairswere observed. These follicular units contained of 5 to 8 hairs. In dermatitisherpetiformis, trichoscopy showed clusters of multiple blood vessels, which areregularly distributed, forming linear and circular structures. In conclusion, tricho-scopy may serve as a supplementary method for preliminary, in-office differentialdiagnosis scalp lesions in pemphigus.

cial support: None identified.

Commer

P6744Treatment of coexisting pemphigus foliaceus and psoriasis withadalimumab

Francisco Guimera-Martin-Neda, MD, Hospital Universitario de Canarias,Department of Dermatology, Tenerife, Spain; Eva Fagundo-Gonzalez, HospitalUniversitario de Canarias. Department of Dermatology, La Laguna, Spain; MariaJose Gonzalez-Mesa-Ponte, Hospital Universitario de Canarias, Department ofDermatology, La Laguna, Spain; Nuria Perez-Robayna, Hospital Universitario deCanarias, Department of Dermatology, La Laguna, Spain; Rosalba Sanchez-Gonzalez, Hospital Universitario de Canarias, Department of Dermatology, LaLaguna, Spain; Sorahaya Gonzalez-Hernandez, Hospital Universitario de Canarias,Department of Dermatology, La Laguna, Spain

Case report: A 39-year-old white woman with psoriasis presented with an acute5-day eruption of flaccid vesicles and bullae on his trunk and extremities. She hadpsoriatic plaques on his scalp, trunk, and extremities. Previous therapy for hispsoriasis consisted of psoralen plus ultraviolet A light therapy and topical cortico-steroids. Histologic examination revealed a subcorneal acantholytic bulla. Directimmunofluorescence studies revealed displayed epidermal intercellular stainingwith anti-IgG. Indirect immunofluorescence showed epidermal intercellular IgGconsistent with pemphigus foliaceus (PF). Initial therapy consisted of cyclosporin A4 to 5 mg/kg twice daily to suppress his bullous diseases and psoriasis. However,after 3 weeks of therapy, he developed more bullae on his trunk and lowerextremities. Cyclosporin A was discontinued, and she started on prednisone at 60mg/d to control his PF. His bullae and blisters began to resolve after the fifth day, and90% of his lesions disappeared by the 10th day. His psoriatic plaques also improved.A major concern was the potential for psoriasis symptoms to rebound, along withworsening of his bullous disease during the tapering phase of the prednisone.Therefore, adalimumab (80 mg week 0, 40 mg week 1, and later 40 mg each 2weeks) was added to his regimen as the prednisone dose was lowered anddiscontinued. The patient currently is receiving adalimumab 40 mg each 2 weeks,his PF lesions are in remission, and his PASI score is between 2 and 3. No adverseevents were evident during the combination treatment with the tapered prednisoneand adalimumab.

Discussion: Treatment of coexisting psoriasis and PF is difficult. The use andwithdrawal of systemic corticosteroids may induce rebound of psoriatic symptomsand development of pustular psoriasis. The symptoms of psoriasis and PF arebelieved to be mediated by TNF-alfa. TNF-alfa is found in the blisters and serum ofpatients with immunobullous diseases, and disease activity is associated with levelsof circulating TNF-alfa. Etanercept has been used successfully in the treatment ofother immunobullous disorders, including pemphigus vulgaris and cicatricialpemphigoid. These findings prompted us to treat our patient with adalimumab toneutralize the TNF-alfa activity. Our case suggests that adalimumab is an effectivesteroid-sparing therapy for PF, but further studies are required to establish theoptimal therapeutic regimen.

cial support: None identified.

Commer

J AM ACAD DERMATOL AB115