Palmoplantar Eccrine Syringofibroadenomatosis with Hypotrichosis, an Incomplete Form of Schopf Syndrome: Report of a Taiwanese Case with Occurrence of Bullous Pemphigoid

Abstract

Eccrine syringofibroadenoma is a rare benign cutaneous adnexal lesion of acrosyringeal origin. It has polymorphous clinical manifestation ranging from a solitary nodule or papule to multiple lesions, especially in the palmoplantar location. We describe a 31 year-old man with cutaneous manifestation of palmoplantar keratoderma with little or no clinical itch or pain, simultaneously accompanied with progressive alopecia and hair thinning since his early 20s. No family history or other symptoms and signs implying ectodermal dysplasia can be identified. Interestingly, 10 years after the onset of bilateral palmoplantar keratoderma, generalized bullous pemphigoid occurred at the age of 30, and was then cured with steroid treatment. A skin biopsy from the plantar foot showed hyperkeratosis, acanthosis, and epithelial anastomosing cords and strands of cuboidal cells extending down into the dermis and containing ductal structures, in association with a fibrovascular stroma. Response to occlusive steroid and keratolytic therapy was not satisfactory. The first Taiwanese case of multiple eccring syringofibroadenomas as an incomplete form of Schopf syndrome with the coexistence of bullous pemphigoid is herein presented. This diagnosis should be considered when patients present with chronic asymptomatic and refractory palmoplantar keratoderma.