Erythroderma with pemphigus immunofluorescence. An erythrodermic variant of pemphigus?

Emanuele Cozzani, Sonia Balestrero, Aurora Parodi, Alfredo Rebora

Research output: Contribution to journalArticle

Abstract

A 79-year-old woman had suffered from a severe pruritic eruption for 2 years which worsened whenever she was given nonsteroidal anti-inflammatory drugs (NSAIDs). Treatment with itraconazole, 100 mg/day for 16 days, was of no benefit, while partial remission was obtained with methylprednisolone 4 mg/day. On examination, she was in good general condition and exhibited several pruritic polycyclic urticarial lesions (Fig. 1). In a week, from their initial localization at the trunk and limbs, her lesions spread centrifugally to eventually involve the whole body. Oral lesions were not present. Routine laboratory tests, including those directed to the detection of a possible neoplasm, were normal. Eosinophils were 800/mL. Three biopsies were taken 1 week apart. All revealed only a mild epidermal hyperplasia and a lymphocytic infiltrate in the superficial dermis, with some neutrophils and eosinophils. No acantholysis or spongiosis was observed. Direct immunofluorescence (DIF) performed on lesional skin disclosed deposits of both immunoglobulin G (IgG) and C3 in the intercellular spaces (ICS) of the entire epidermis (Fig. 2), and deposits of both IgM and C3 in the vessel walls. Three serum specimens were taken simultaneously with biopsies to detect anti-ICS antibodies. Anti-ICS antibodies were not found in the serum, by indirect immunofluorescence (IIF) with monkey esophagus as substrate and by immunoblotting (IB) on epidermal extract. Because of the DIF features, the patient was diagnosed as having pemphigus and given 2 mg/kg/day prednisone. The lesions cleared after 1 month. During disease remission, however, IIF became positive disclosing IgG anti-ICS antibodies at a serum dilution of 1/40 and, 6 months later, when the patient was taking 5 mg/day prednisone, the IgG titer rose to 1/80. After a further 2 months, when the patient was in clinical remission, IIF was again negative.

Original languageEnglish
Pages (from-to)207-208
Number of pages2
JournalInternational Journal of Dermatology
Volume38
Issue number3
Publication statusPublished - 1999

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ASJC Scopus subject areas

  • Dermatology

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