Penile Erosions as First Manifestation of Pemphigus Vulgaris: A Misleading Presentation

Abstract

Pemphigus vulgaris (PV) is a potentially devastating autoimmune bullous disease that affects the mucosa and skin. It is characteristically associated with intraepidermal blistering and cell-cell dissociation due to the presence of autoantibodies targeting desmoglein (dsg) 3 and dsg 1, i.e. transmembrane adhesion molecules of the desmosomal cadherin protein family (1-3). In most patients, PV characteristically starts with and affects the oral mucosa, although involvement of other mucosae may also occur (4-10). We have recently observed 2 patients, in whom the first clinical manifestation of PV consisted of chronic painful penile erosions, which represented a diagnostic challenge. CASE REPORTSCase 1. A 59-year-old man presented with painful penile erosions of 3-months duration. Microbiological cultures and serology for Treponema pallidum were negative. Under treatment with local antibiotics and disinfectants the erosive lesions further progressed and affected the entire glans penis and part of the shaft. Only after the patient developed painful aphthoid lesions in the oral mucosa, was a biopsy specimen from the oral mucosa obtained, which showed suprabasal blistering with acantholysis. Direct immunofluorescence studies of perilesional oral mucosa demonstrated intercellular epithelial deposits of immunoglobulin G (IgG) and complement (C3). Enzyme-linked immunoassay-desmoglein 3 (ELISA-dsg 3) (136 U/ml, normal < 7) and ELISA-dsg 1 (62 U/ ml, normal < 14) were both positive. The patient was first given prednisone 0.5 mg/kg body weight. However, under this regimen he further developed multiple vesicles and bullae on the neck, trunk and extremities. Prednisone was hence increased to 1 mg/ kg body weight/day and associated with azathioprine 2.5 mg/ kg/day. Genital lesions were concomitantly treated with topical betamethasone associated with topical antibiotics and disinfectants. Thereafter, both mucous and cutaneous lesions started healing. Four weeks later, at the end of the consolidation phase, prednisone was tapered gradually. After 12 months, the patient remained in complete remission under therapy (Fig. 1). Case 2. A 35-year-old man was referred for evaluation of a 3-month history of painful penile erosions. Topical treatments with disinfectants and topical antibiotics had no effect. Two months after appearance of the genital lesions the patient also developed painful oral lesions. On examination, the patient had multiple erythematous erosions on the glans penis and sulcus coronarius (Fig. 2). In the oral cavity, the patient exhibited asymptomatic erosions in the region of the palatal arch and the lower lip. Histology from the glans penis showed an intraepidermal bulla with acantholytic cells. Direct immunofluorescence microscopy studies demonstrated deposits of IgG and C3 on the cytoplasmic membrane of the keratinocytes. ELISA-dsg 3 was strongly positive (146 U/ml, normal <7), whereas ELISA-dsg 1 was negative. The patient was treated with prednisone 1 mg/ kg body weight and azathioprine 150 mg/day. Furthermore, he...