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Desmoplastic hairless hypopigmented nevus (DHHN). A distinct variant of giant melanocytic nevus
European Journal of Dermatology. Volume 15, Number 6, 451-3, November-December 2005, Genes and skin
Free Article
Author(s) : María del Carmen Boente, Raúl A Asial
Summary : Desmoplastic hairless hypopigmented nevus (DHHN) is the name Ruiz-Maldonado et al. gave to a new variant of giant congenital melanocytic nevus characterized clinically by a hard ligneous consistency, absence of hair and progressive loss of pigment. Histologically, dermal fibrosis consistent with desmoplasia is a predominant feature. We describe a 6-year-old boy with a hard hairless pigmented congenital nevus involving the lumbosacral area, buttocks, perineum and scrotum. During the first years of life, the nevus showed a progressive reduction in colour, size and consistency. These changes continued until the age of four when a well-demarcated tumour appeared, within the nevus, on the right buttock. Resection of this outgrowth was performed. Histologically, nevus cells of normal appearance between thick collagen bundles were present. Immunostaining revealed S100 +, Vim +, HMB45 – results. The nevus has continued to involute to date. An immune response against the melanocytes of the nevus may explain this type of evolution.
Keywords : congenital melanocytic nevus, desmoplastic hairless hypopigmented nevus, nevus

Desmoplastic hairless hypopigmented nevus (DHHN). A distinct variant of giant melanocytic nevus