Lee Eun Ah, Kim Hei Sung, Kim Hyung Ok, Park Young Min
Department of Dermatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Ann Dermatol. 2009 Nov;21(4):426-8. doi: 10.5021/ad.2009.21.4.426. Epub 2009 Nov 30.
Focal acral hyperkeratosis (FAH) is a rare genodermatosis with an autosomal dominant pattern of inheritance; however, it may also be sporadic. FAH is characterized by late-onset crateriform keratotic papules, some coalescing into plaques, along the borders of the hands and feet. We herein report a case of FAH in a 47-year-old male with a family history of similar lesions in three generations. The histological findings revealed focal areas of orthohyperkeratosis over an area of depressed but otherwise normal epidermis. The dermis showed no specific changes, which distinguished this case from acrokeratoelastoidosis, which shows elastorrhexis of clinically similar lesions.
局限性肢端角化过度症(FAH)是一种罕见的遗传性皮肤病,呈常染色体显性遗传模式;不过,它也可能是散发性的。FAH的特征是在手足边缘出现迟发性火山口状角化性丘疹,部分融合成斑块。我们在此报告一例47岁男性的FAH病例,其家族三代均有类似皮损病史。组织学检查结果显示,在表皮凹陷但其他方面正常的区域出现局灶性正角化过度。真皮未见特异性改变,这将该病例与肢端角化性弹性组织变性区分开来,后者在临床类似皮损处可见弹性纤维离解。