TY - JOUR
T1 - Palmoplanar eccrine syringofibroadenomatosis with hypotrichosis, an incomplete form of Schöpf syndrome
T2 - Report of a Taiwanese case with occurrence of bullous pemphigoid
AU - Huang, Shen Lin
AU - Yu, Yi Sheng
AU - Cheng, Yu Wen
AU - Huang, Chao Cheng
AU - Wu, Wei Ming
PY - 2007/9
Y1 - 2007/9
N2 - 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.
AB - 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.
KW - Bullous pemphigoid
KW - Eccrine syringofibroadenoma
KW - Schöpf syndrome
UR - http://www.scopus.com/inward/record.url?scp=34548603256&partnerID=8YFLogxK
M3 - 文章
AN - SCOPUS:34548603256
SN - 1027-8117
VL - 25
SP - 219
EP - 225
JO - Dermatologica Sinica
JF - Dermatologica Sinica
IS - 3
ER -