Six Cases of Erythrodermic Pemphigus Foliaceus: A Case Report

Abstract

Pemphigus foliaceus (PF) is one of the causes of erythroderma; however, to date, there have been relatively few reported cases. We herein describe 6 cases of erythrodermic PF. In all 6 cases, PF was a direct cause of erythroderma because the patients had not undergone any medical treatments and neither had any other skin diseases nor were taking any drugs that typically cause erythroderma. Serum levels of IgE and thymus and activation-regulated chemokine were elevated in 5 of the 6 cases, whereas soluble interleukin-2 receptor and squamous cell carcinoma-related antigen were markedly increased in all cases, suggesting that those markers are strong indicators of skin surface damage. All patients were treated with predonisolon (PSL), of which PSL pulse was added in 4 patients and intravenous immunoglobulin was added in 4 patients. Furthermore, all patients except for one were older adults, among whom 2 cases developed Kaposi's varicelliform eruption, and died, and another 2 patients, respectively, died of gastrointestinal bleeding and sepsis. Kaposi's varicelliform eruption is a complication of erythrodermic PF associated with poor prognosis, and thus caution is necessary when considering the diagnosis. Furthermore, elderly people are more likely to have complications due to PSL, which may result in death. Inappropriate treatment and delay in treatment may cause erythroderma, so early diagnosis and treatment are necessary.

Keywords: Erythroderma; Kaposi's varicelliform eruption; Pemphigus.

Fig. 1

a–fErythrodermic condition consisting of erythemas, scales, and erosions with profuse exudates all over the patient's body. gHistopathology showing infiltration of numerous neutrophils in the subcorneal layer. Acantholytic cells were observed in granular layer (hematoxylin-eosin stain, ×100). hDirect immunofluorescence staining detected serum IgG antibodies against the intercellular cell surface.