Pemphigus vegetans in association with human immunodeficiency virus.

Abstract

A 23‐year‐old black man presented with a 2‐year history of blistering eruptions involving the skin and mucous membranes. Risk factors for human immunodeficiency virus (HIV) infection included sexual encounters with prostitutes. He had no additional past medical history and denied any family history of autoimmune blistering disorders. Over the last 2 years the patient had undergone multiple admissions at various medical centers with a diagnosis of pemphigus vegetans (PV). He was treated with a variety of topical and systemic medications, including steroids and antimicrobials, but he always relapsed within a few months. The patient presented to the Metropolitan Hospital Center with malaise, blisters, and vegetative lesions of several days’ duration.On physical examination, erosions, pustules, nodules, and verrucous, vegetating papules were noted in the axillae, groin ( Fig. 1), scrotal, and anal areas. There were erosions and crusts on the lower lips. The tongue showed mild desquamation and had a cerebriform appearance ( Fig. 2). Paronychial crust and scale were noted on the hands and feet and an intact vesicle was noted on one finger. Inguinal, submandibular, cervical, and axillary lymphadenopathy was present. Hyperpigmented macules and patches were noted on the trunk and extremities.Erosions, crust, and verrucous papules in the groinimageCerebriform appearance of the tongue in association with crusts and erosions on the lipsimageA biopsy specimen of a verrucous papule from the left axilla showed marked spongiosis, intraepidermal acantholysis, and numerous eosinophils within the acantholytic vesicle ( Fig. 3). In addition, there was a marked superficial dermal infiltrate with eosinophils, plasma cells, and lymphocytes. A biopsy specimen of a pustule in the left axilla showed marked spongiosis, intraepidermal vesicle formation, superficial dermal edema, perivascular infiltration with lymphocytes and plasma cells, and exocytosis of neutrophils and eosinophils in the dermis and epidermis.Histopathology of vegetating lesion showing acantholysis and a dense eosinophilic infiltrate (hematoxylin and eosin, ×100)imageDirect immunofluorescence showed immunoglobulin G (IgG) and weak C3 on the intercellular junctions in the epidermis. Indirect immunofluorescence showed a high titer (>1 : 1280) of circulating antibodies with an intercellular pattern. Viral culture from the mouth and skin was negative for herpes simplex virus. Bacterial culture from the skin of the groin was positive for Proteus mirabilis. Blood cultures were negative. Laboratory tests on admission showed a normocytic anemia, and the CD4 count was 372 cells/mm3 (normal, 537–1571 cells/mm3 ). The urine on admission was positive for opiates and cocaine. The results of HIV‐1 testing by enzyme‐linked immunoabsorbent assay and immunoblotting were positive.