Psoriasiform pemphigus foliaceus: a report of two cases

Abstract

Pemphigus foliaceus (PF) represents an autoimmune blistering disease characterized by the disruption of epidermal intercellular adhesion proteins. Clinical findings include superficial crusted erosions in a seborrheic distribution; however, the disease can rarely present as an exfoliative erythroderma. Histopathologic findings include acantholysis with cleavage within the granular layer. Direct immunofluorescence studies show intercellular IgG and complement deposition. We present two patients, to our dermatology department, with a previous diagnosis of psoriasis, with an exfoliative erythroderma, which ultimately proved to be because of PF based on histopathological features, direct immunofluorescence results and levels of antibodies against desmoglein 1. Both patients responded well to oral prednisone and rituximab. This variant of PF should be entertained in both the clinical differential diagnosis of psoriasiform erythroderma and in the microscopic differential diagnosis of psoriasiform epidermal hyperplasia with focal acantholysis, particularly in patients for whom the clinical history is not classic for psoriasis or for whom conventional psoriasis therapies have not proven beneficial.

Dermatolojide Rituksimab Kullanımı

“…Connelly ve ark ise jeneralize eritrodermik pemfigus foliaseuslu çocuk bir olguyu rituksimabla tedavi edebilmiştir (85). Grekin ve ark ise psoriasiform pemfigus foliaseuslu 2 olguda ri-tuksimab ve steroid kombinasyonunu başarılı şe-kilde uygulamıştır (95).…”

Non‐endemic erythrodermic pemphigus foliaceus: a case with delayed diagnosis and response to rituximab

“…Für die korrekte Diagnose und ein angemessenes Therapiemanagement ist die Immunfl uoreszenz obligatorisch [ 2,3 ] [ 6 ] . An einen erythrodermischen PF sollte auch bei Patienten ohne Psoriasis-Anamnese gedacht werden, oder wenn die Erythrodermie auf konventionelle Psoriasistherapien nicht anspricht [ 5 ] . Vorsicht ist darüber hinaus geboten, da auch das gleichzeitige Auftreten einer anulären Psoriasis und eines PF beschrieben wurde [ 7 ] .…”

“…Über seine Rolle bei der Behandlung des Pemphigus-Spektrums wurde in der Literatur eingehend berichtet [ 2,9,10 ] . Jedoch gibt es nur wenige Fallberichte (insgesamt vier mit dem vorliegenden Fall) über nichtendemischen erythrodermischen PF, der erfolgreich mit Rituximab behandelt wurde [ 5,11 ]…”

Avaliação da expressão do fator de crescimento endotelial vascular e da endoglina nos pacientes eritrodérmicos com pênfigo foliáceo

“…• A eritrodermia adquirida em adultos é uma manifestação clínica exuberante compartilhada por dermatoses autoimunes, doenças inflamatórias, tumorais e por hipersensibilidade a drogas 38,106,107 . A maioria das publicações em língua inglesa na literatura médica a respeito dessa apresentação do PF corresponde a relatos de casos [108][109][110][111][112][113][114][115][116] . Há também séries de casos de eritrodermia com descrição das etiologias mais comumente a ela associadas 30,37,38 .…”

“…A eritrodermia foi descrita em 2 a 6,25% dos pacientes com PF. Por outro lado, nos pacientes eritrodérmicos o PF é a causa da eritrodermia em 0,76% a 6,25% dos casos 30,37,38 , sendo menos frequente do que a associação com PSO e eczema 38,106,107 [108][109][110][111][112]114,115,117 ; em 3 casos essa informação não foi divulgada 28,113,116 .…”