Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature

Abstract

This entity was established 40 years ago, and around 100 patients have been reported worldwide. It is important to be aware of this particular form of pemphigus because clinical presentation, course of the disease and therapeutic approach are different from conventional forms of pemphigus.

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

“…The initial search yielded 371 publications: 284 from the PubMed database and 87 from the Ovid Medline database. We identified 96 publications (88 from PubMed and 63 from Ovid Medline [55 duplicates between the two databases]) reporting 158 individual cases that fulfilled the inclusion criteria. We also report an additional three unpublished cases diagnosed at our institution.…”

Pemphigus herpetiformis masquerading as tinea corporis

“…Therefore, a delay in the diagnosis is common. [2] We herein report a case of PH with annular pruritic erythematous plaques and bullous lesions mimicking tinea corporis. A 45-year-old female presented with chief complaints of itchy red annular lesions on the inframammary area, back, abdomen, and lower limbs since 2 months.…”

“…Pemphigus herpetiformis is a rare entity and sporadic variant of pemphigus. [2] The incidence of PH is approximately 6% to 7.3%. [1345] It mainly affects adults with no gender predilection.…”

“…[35] Presentation is different from the classical forms of pemphigus. [2] Jablonska et al [1] and Floden and Gentele[6] described this entity in early 1955 and named it as dermatitis herpetiformis with acantholysis. PH has atypical presentation and a benign course.…”

“…[15] In majority of cases mucous membranes are spared. [1234] Pruritus often accompanies skin lesions, sometimes it might be severe. [134] Eosinophilia is found in peripheral blood and had been reported in 37.5% of cases by Laws et al [3] Histopathologically, PH shows the presence of intraepidermal bullae,[34] or pustules[35] mostly in the subcorneal, and occasionally suprabasal or in the spinous layer.…”