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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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CASE REPORT  


Giornale Italiano di Dermatologia e Venereologia 1999 December;134(6):595-8

language: English

A ­case of mul­ti-trig­gered pem­phi­gus vul­gar­is

Satriano R. A., Fiorentini F., Tripodi Cutrì F., Muto L.

Department of Dermatology, School of Medicine and Surgery, University of Naples II, Naples


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Pemphigus vul­gar­is is a bul­lous dis­ease ­which ­affects ­skin and ker­a­ti­niz­ing, or poten­tial­ly ­such, muco­sae. Aetiology can be ­unknown or relat­ed to sev­er­al exog­e­nous fac­tors ­able to ­cause (­induced pem­phi­gus) or ­favour (trig­gered pem­phi­gus) the dis­ease in genet­i­cal­ly pre­dis­posed sub­jects. A ­case of a 24-­year-old wom­an ­with pem­phi­gus vul­gar­is ­induced by admin­is­tra­tion of pro­ges­te­rone, noram­i­dop­i­rine, acet­yl­sal­i­cil­ic ­acid and expo­sure to UV ­rays is reported. The ­patient pre­sent­ed ­with ery­them­a­tous, squa­mous, and crus­tous patch­es on the ­scalp, sev­er­al flac­cid blis­ters on appar­ent­ly nor­mal ­skin, ero­sive are­as and ­sero-emat­ic ­crusts on the ­face, ­trunk, and ­limbs, and ero­sions in the ­nasal and ­oral muco­sae. Perilesional Nikolsky’s ­sign was pos­i­tive. Cytological exam­ina­tion ­from blis­ters of the ­trunk and ­from ­oral ero­sions ­showed typ­i­cal acan­tho­lyt­ic ­cells. Biopsy of a ­fresh bul­la ­revealed suprab­a­sal acan­tho­lyt­ic detach­ments. Indirect immu­no­flu­o­res­cence test­ing ­showed inter­cel­lu­lar auto­an­ti­bod­ies at a 1:160 ­titre. HLA typ­ing detect­ed DR14 and DQ5 ­alleles, according for a genet­ic pre­dis­po­si­tion to the dis­ease. Steroid ther­a­py was start­ed, accord­ing to the con­ven­tion­al treat­ment pro­to­col, and result­ing in remis­sion of the dis­ease in 16 ­weeks, com­plete dis­ap­par­ence of inter­cel­lu­lar auto­an­ti­bod­ies in 24 ­weeks.

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