A Rare Case of Hallopeau-Type Pemphigus Vegetans with Premalatha Sign: A Case Report and Literature Review

Abstract

Pemphigus vegetans (PVeg) is the rarest clinical variant in the pemphigus group of vesiculobullous autoimmune diseases, accounting  for less than 2% of all pemphigus cases.¹ It is classified into two subtypes: Hallopeau and Neumann, which differ in severity and prognosis. PVeg presents with flaccid bullae or pustules that break down into thickened, papillated plaques, mainly involving the skin folds, scalp, face, and mucous membranes. In some cases, oral lesions may resemble the surface of the brain, a distinctive feature known as the Premalatha sign. The diagnosis of PVeg relies on a combination of clinical presentations, histopathological findings, and laboratory investigations.

We reported a case of PVeg characterized by involvement of skin folds, oral ulcers, and a cerebriform tongue (Premalatha sign). A tissue sample taken from a lesion in the right groin revealed papillomatous hyperplasia of the epidermis, suprabasal separation with acantholytic cells, and intraepidermal abscesses, consistent with a classic presentation of PVeg. Direct immunofluorescence revealed IgG deposits in the intercellular spaces of the epidermis, while indirect immunofluorescence detected a high titer of pemphigus IgG autoantibodies.  Additionally, the anti-desmoglein 3 antibody test was positive. The patient received a three-month regimen of prednisolone and azathioprine, leading to successful treatment without relapse. This outcome confirmed the diagnosis of Hallopeau-type PVeg, which is linked to a more favorable prognosis.