Palmoplanar eccrine syringofibroadenomatosis with hypotrichosis, an incomplete form of Schöpf syndrome: Report of a Taiwanese case with occurrence of bullous pemphigoid

Shen Lin Huang, Yi Sheng Yu, Yu Wen Cheng, Chao Cheng Huang, Wei Ming Wu*

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

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 eccrine syringofibroadenomas as an incomplete form of Schöpf 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.

Original languageEnglish
Pages (from-to)219-225
Number of pages7
JournalDermatologica Sinica
Volume25
Issue number3
StatePublished - 09 2007

Keywords

  • Bullous pemphigoid
  • Eccrine syringofibroadenoma
  • Schöpf syndrome

Fingerprint

Dive into the research topics of 'Palmoplanar eccrine syringofibroadenomatosis with hypotrichosis, an incomplete form of Schöpf syndrome: Report of a Taiwanese case with occurrence of bullous pemphigoid'. Together they form a unique fingerprint.

Cite this