Amicrobial pustulosis of the folds mimicking Hailey-Hailey disease in a patient with systemic lupus erythematosus and Sjögren’s syndrome

Abstract

Sir, Amicrobial pustulosis of the folds (APF) is a rare disease of neutrophilic dermatosis involving the scalp, ears, axillae, umbilicus, groin, and buttocks [1–3]. Herein, we describe a case of APF involving the bilateral axillae mimicking Hailey-Hailey disease in association with systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SjS). A 63-year-old female had been undergoing treatment for SLE and SjS for six years. She had been taking prednisolone at a dose of 4 mg per day on the first visit to us. She also had a history of Crohn’s disease in remission. She complained of recurrent, erosive erythema on the axillae and beneath the breasts, which worsened in summer. A physical examination revealed infiltrative erythema with superficial erosions on the bilateral axillae (Figs. 1a and 1b). A laboratory examination revealed normal liver and kidney function, yet positive results for serum antinuclear antibodies (1:1280), anti-double strand DNA antibody (128 IU/mL), anti-Sm antibody (index: 6.7), anti-SS-A antibody (index: 131.5), and anti-SS-B antibody (index: 93.7). The erythrocyte sedimentation rate was high (32 mm in the first hour), and the CRP level was slightly increased (1.19 mg/dL). Bacterial cultures were positive for group G streptococci and the Corynebacterium species. A biopsy specimen did not reveal acantholysis of the epidermis, yet mild epidermal acanthosis without intense infiltration of inflammatory cells. Hailey-Hailey disease was excluded, and the patient was followed under treatment with topical corticosteroid ointment. During the course, the skin lesions exacerbated, and new pustules and erosive erythemas appeared on the groin and the anogenital areas (Fig. 1c). A histopathological examination revealed subcorneal neutrophilic abscesses in the epidermal and dermal perifollicular areas (Figs. 2a – 2d).