Case of intractable scalp pustulosis with alopecia diagnosed with amicrobial pustulosis of the folds

Miyakawa, Mami; Yamaguchi, Yukie; Hirokado, Michiko; Aihara, Michiko

doi:10.1111/1346-8138.15142

The Journal of Dermatology

2019

DOI: 10.1111/1346-8138.15142

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Case of intractable scalp pustulosis with alopecia diagnosed with amicrobial pustulosis of the folds

Mami Miyakawa

Yukie Yamaguchi

Michiko Hirokado

et al.

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2022

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“…It mainly affects the scalp and ear canals, as well as the intertriginous areas such as the axillary, groin, and perianal regions. If it involves the scalp, it typically results in alopecia [4]. APF is characterized by small, sterile pustules and erythemas and, histopathologically, subcorneal or intraepidermal neutrophil infiltration sometimes forming spongiform pustules, parakeratosis, and cellular infiltrates in the upper dermis.…”

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

Hanami¹,

Yamamoto²

2022

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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).

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confidence: 99%

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

Hanami¹,

Yamamoto²

2022

Our Dermatol Online

View full text Add to dashboard Cite

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Case of intractable scalp pustulosis with alopecia diagnosed with amicrobial pustulosis of the folds

Cited by 1 publication

References 5 publications

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

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

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