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

汗腺汗管纖維腺瘤是一個以末端汗管為起源的罕見良性皮膚附屬器病灶，它多型多樣的臨床表現可以從一個單獨的結節或丘疹到數個特別是位於手腳掌的病灶。我們描述一個31歲的男性以不痛也不癢們於手掌及腳掌的角皮症，同時伴隨從20處是期逐漸掉?及頭?變細的情形，沒有相關的家族史或有顯?外層發育異常的症狀可以辨識。特別是在出現手掌及腳掌角皮症10年以後，全身類天瘡在他30歲時發生，之後使用類固醇而治癒。皮膚切片顯示過度角化、棘層肥厚、在表皮由立方型細胞連結成索狀並往下延伸真皮層－索狀表皮中含有管狀結構、周圍並有纖維血管組織，病人對局部類固醇及角質溶解藥物沒有令人滿意的進步。我們?告台灣第一個以數個汗腺汗管纖維腺瘤?呈現的不完全型Sch?pf徵候群，同時合併有類天 瘡，而臨床若出現慢性無症狀且治療反應不好的手掌腳掌角皮症，此病也順列入鑑別診斷之一。
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 Sch?pf 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.