A case of amicrobial pustulosis of the folds in a patient with Sjogren's disease / 대한내과학회지

ABSTRACT

Amicrobial pustulosis of the folds (APF) is a rare pustular eruption, which predominantly involves the cutaneous folds, including the external auditory canals, scalp, and palms. It occurs in patients with a wide spectrum of autoimmune abnormalities. Systemic lupus erythematosus is the autoimmune disorder observed most commonly in patients with APF. The etiology of APF is unclear, but all reported cases have involved women of reproductive age when the eruptions first occurred. This report describes a 34-year-old woman who presented with an acute pustular rash. Her medical history was unremarkable, except for transitory dry eyes, dry mouth, and polyarthralgia for 1 year. Coalescing pustular lesions arising on erythematous skin, forming crusted and eroded plaques, were observed, which predominantly affected the anterior chest, mainly the cutaneous folds. The periorificial regions were also involved, including the nasal alae, mouth angles, and external auditory canals. Our patient had the characteristic clinical and histopathological features of APF, in association with primary Sjogren's syndrome (pSS). In conclusion, we present a patient having APF associated with pSS.