Primary localized cutaneous amyloidosis intermingled with papulopustular lesions in a patient with Behçet’s disease

Abstract

Primary localized cutaneous amyloidosis (PLCA) sometimes shows overlapping with collagen vascular diseases; however, association with Behçet’s disease (BD) is rare. We herein report a case of PLCA developing intermingled with papulopustular lesions associated with BD. A 57-year-old woman visited our hospital, complaining of recalcitrant multiple oral aphthae. Further detailed physical examination revealed folliculitis-like small pustules with surrounding erythema on the upper back. Also, she stated past histories of genital ulcers 10 years ago and erythema nodosum on the lower legs 4 years ago. A biopsy specimen from the pustular lesion on the back showed neutrophilic abscess in the epidermis with a number of perivascular inflammatory cells in the upper dermis. Congo-red and Dylon stain revealed amyloid deposition in the papillary dermis. Although association of systemic amyloidosis with BD is occasionally found in the literature, co-occurrence of cutaneous amyloidosis and papulopustular lesions of BD is extremely rare.