Erythema Dyschromicum Perstans: Report of a New Case and Critical Review of the Literature

Abstract

Erythema dyschromicum perstans (EDP), described by Convit et al. in 1961, is a rare dermatosis. Its relationship with ashy dermatosis (AD), described by Ramirez in 1957, is still a matter of debate. We report a typical case of EDP. The patient, of North African origin, had a dyschromic (hypo- and hyperpigmented) eruption on the chest and limbs for 2 years. The lesions were occasionally surrounded by a papular border which spread slowly and centrifugally. Histological examination showed a lichenoid infiltrate. A carcinoma of the lung was simultaneously discovered. No treatment was given, EDP is infrequent and often considered identical to ashy dermatosis in the literature. However, the clinical aspects of the two diseases differ. The main features of these two diseases are reviewed and compared on the basis of a literature review. We conclude that EDP and AD are distinct clinical entities.

Erythema dyschromicum perstans: the continuing enigma of Cinderella or ashy dermatosis

“…The therapeutic options are many, but few have been effective,5,6,8,12–14,24,32,42–47 except possibly clofazimine 13,32 . Its beneficial effect may be mediated by anti‐inflammatory and immunomodulatory actions 32 .…”

Segmental neurofibromatosis is caused by somatic mutation of the neurofibromatosis type 1 (NF1) gene

“…The strictly defined segmental NF was termed NF5 in Riccardi's clinical classification of the neurofibromatoses into eight groups. 4 Broader definitions were given by Roth 5 and Combemale et al 6 Their classification schemes, however, which include heritable and non-heritable clinically distinguishable subtypes were not in agreement with genetic knowledge. A classification for neurofibromatoses into NF1, NF2, alternate and related forms of either NF1 or NF2 was proposed by Viskochil and Carey, 7 according to the present understanding of the molecular aspects of neurofibromatoses.…”

Acquired Dermal Macular Hyperpigmentation

“…Ramirez in 1957 described a condition called “dermatosis cenicienta” (ashy dermatosis),[ 29 ] which presented with small greyish macules, and later in 1961, Convit et al . [ 30 ] described a dermatosis from Venezuela consisting of five cases characterized by large greyish macules with a papular, slowly extending erythematous border.…”