Raj Radhika Cg, Patil Rajesh
Department of Surgery, Goa Medical College, Bambolim, Goa, India.
Goa Medical College, Bambolim, Goa, India.
Indian J Dermatol. 2015 Mar-Apr;60(2):217. doi: 10.4103/0019-5154.152585.
Familial atypical multiple mole melanoma syndrome (FAMMMS) is an autosomal dominant genodermatosis characterized by multiple melanocytic nevi, usually more than 50, and a family history of melanoma. It is known to be associated with carcinoma of pancreas and other malignancies involving gastrointestinal tract, breast, lung, larynx, and skin in the kindred. There is no published report of FAMMMS in dark-skinned individuals. We report a case of FAMMMS in a dark-skinned adult Indian male, who had multiple extensive nevi all over the body and oral mucosa; associated with malignant melanoma, squamous cell carcinoma (Marjolin's ulcer), and carcinoma of pancreas. His father had died of carcinoma of lung and his sister had a partial phenotypic expression. The clinical presentation of the case is discussed with review of literature.
家族性非典型多发性痣黑色素瘤综合征（FAMMMS）是一种常染色体显性遗传性皮肤病，其特征为多发性黑素细胞痣，通常超过50个，并有黑色素瘤家族史。已知在该家族中，它与胰腺癌及其他涉及胃肠道、乳腺、肺、喉和皮肤的恶性肿瘤有关。尚无关于深色皮肤个体患FAMMMS的报道。我们报告一例深色皮肤的成年印度男性患FAMMMS的病例，该患者全身及口腔黏膜有多处广泛的痣；伴有恶性黑色素瘤、鳞状细胞癌（Marjolin溃疡）和胰腺癌。他的父亲死于肺癌，他的妹妹有部分表型表达。结合文献复习对该病例的临床表现进行了讨论。
