Pemphigus Vegetans

Abstract

Two cases of pemphigus vegetans are presented, one of the Neumann type and one of the Hallopeau type. The differences between the two subsets are clinical presentation and course. Patients with the Hallopeau type often have a relatively benign disease, require lower doses of systemic corticosteroids, and usually have a prolonged remission. Patients with the Neumann type have a course similar to pemphigus vulgaris, need higher doses of systemic corticosteroids, and have relapses and remissions. The histologic findings in the vegetating lesions are similar in both types. The immunopathologic features of both types are indistinguishable and similar to pemphigus vulgaris. IgM staining of eosinophilic microabscesses was observed in one case.

Pemphigus vegetans: An unusual presentation

“…[3] Two subtypes – Neumann and Hallopeau types are recognized, which are differentiated based on their clinical presentation, course, and response to treatment. [45] Unlike pemphigus vulgaris which has a classical clinical picture and is easy to diagnose most of the time, pemphigus vegetans, especially the Hallopeau variant, has no clinical resemblance to a vesiculobullous disorder. It presents as heaped up vegetating and verrucous plaques mainly in the flexures.…”

Oral Lesions in Autoimmune Bullous Diseases: An Overview of Clinical Characteristics and Diagnostic Algorithm

“…Mucocutaneous PV is more common than mucosal dominant PV [76]. Oral involvement in pemphigus vegetans, a clinical variant of PV, is reported in 60–80% of the cases [77]. Skin lesions typically consist of flaccid blisters that easily rupture, leaving erosions and crusts, and are commonly distributed on the face, scalp, and upper chest [3].…”

Pemphigus Vegetans of Neumann Type on Lip: A Case Report

“…According to the clinical manifestations, there are two subtypes: (1) the Neu-mann, and (2) Hallopeau subtypes. The Neumann type is characterized by bullae that extend and coalesce; later progressing to vegetating masses or plaques, which become dry, hyperkeratotic, and fissured (10).…”