[Acral melanoma in a patient with hereditary keratoderma of the palms and soles (mal de Meleda): A chance association?] / Mélanome acral chez un patient atteint de kératodermie palmoplantaire de type mal de Méléda : une association fortuite ?

ABSTRACT

INTRODUCTION: Development of acral malignant melanoma in Mal de Meleda is highly unusual. As far as we could ascertain, to date, only 10 previous cases have been published. Herein, we report a new case. OBSERVATION A 64-year-old Algerian man was followed for familial Mal de Meleda. The diagnosis was based on clinical presentation as he had a non-syndromic hereditary foul-smelling and yellowish palmoplantar keratoderma transgrediens. After the failure of acitretin, which had not prevented retractile and mutilating progression of the palmoplantar keratoderma, he had undergone surgery with graft excision of both palms. At the age of 59 years, he presented a tumor on the dorsal aspect of the 1st phalanx of the 3rd finger of the right hand in a non-grafted area. The diagnosis of acral melanoma was confirmed histologically. The radiological findings showed a specific homolateral axillary adenopathy. He underwent digital amputation of the 3rd finger, with lymph node dissection and chemotherapy involving dacarbazine. Follow-up at 5 years showed complete remission of the melanoma. DISCUSSION: Mal de Meleda is a hereditary palmoplantar keratoderma due to mutation of the SLURP1 gene. Clinical diagnosis is based on the typical phenotype in adulthood. The occurrence of acral melanoma, which is a rare form of melanoma (1 to 7%), especially in the fingers, together with an unusual palmoplantar keratoderma in a subject of type IV phototype does not appear to be a chance event. This association seems to be the outcome of immune dysregulation rather than of chronic inflammation.