A kindred with familial progressive hyperpigmentation-like disorder: Implication of fibroblast-derived growth factors in pigmentation

Giorgia Cardinali, Daniela Kovacs, Micol Del Giglio, Carlo Cota, Nicaela Aspite, Ada Amantea, Giampiero Girolomoni, Mauro Picardo

Research output: Contribution to journalArticle

Abstract

Two patients with a generalized, progressive dyschromatosis disorder are described and investigated as a model to study the role of fibroblast-derived mediators on skin pigmentation. The patients (father and daughter) had had a widespread hyperpigmentation since early life which then progressively worsened with the appearance of hyperpigmented macules, café-au-lait macules and freckles, also involving the lips, palms and soles, intermixed with small hypopigmented spots. These features resembled those of familial progressive hyperpigmentation (FPH). Histology revealed a normal epidermis with pronounced keratinocyte hyperpigmentation and the presence of dermal melanophages. Ultrastructural analysis showed basal and suprabasal keratinocytes enriched in melanosome complexes. Immunohistochemical staining displayed an increased expression of hepatocyte growth factor (HGF), stem cell factor (SCF) and keratinocyte growth factor (KGF) in fibroblast-like cells of the upper dermis in hyperpigmented lesions of both patients, compared to control healthy skin. Our data suggest that a persistent activation of fibroblasts abnormally stimulating melanocyte functions is involved in hyperpigmentation disorders.

Original languageEnglish
Pages (from-to)469-473
Number of pages5
JournalEuropean Journal of Dermatology
Volume19
Issue number5
DOIs
Publication statusPublished - Sep 2009

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Keywords

  • Fibroblasts
  • Hyperpigmentation
  • Melanogenic growth factors

ASJC Scopus subject areas

  • Dermatology

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