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INTRODUCTION

KEY POINTS

  • Erythema dyschromicum perstans (EDP) primarily affects Latin Americans but is also seen in darker-pigmented people and Asians; it is rarely seen in non-Hispanic Caucasians.

  • EDP is also known as dermatitis cenicienta (“ashy dermatosis”), los cenicientos (“the ashy ones”), and erythema chronicum figuratum melanodermicum, and pintoid.

  • The pathogenesis is elusive, and often no etiologic cause can be assigned to the disease.

  • EDP is characterized by asymptomatic, progressive, and often symmetrically distributed hyperpigmented macules and patches.

  • Modest success has been reported with clofazimine and dapsone therapy.

SYNONYMS

  • Erythema dyschromicum perstans

  • Ashy dermatosis

  • Dermatosis cenicienta

  • Erythema chronicum figuratum melanodermicum

  • Pintoid

INTRODUCTION

Erythema dyschromicum perstans (EDP) is a chronic, acquired, rare pigmentary disorder most commonly reported in Hispanics, but it also occurs worldwide, especially in those with darker-pigmented skin.

This condition was first reported by Ramirez in El Salvador in 1957.1 He called these patients los cenicientos, meaning “ashy ones.” In Spanish, the word cenicienta refers to the folklore character Cinderella, who had an ash-dirtied face due to constantly sitting close to the fireplace at home.2 It is also known as ashy dermatosis because of the ashy gray color exhibited by the Tyndall effect due to pigment incontinence in the dermis. The term erythema dyschromicum perstans was coined by Venezuelan physician Marion Sulzberger to highlight the word erythema because an erythematous border is observed in the early lesions and also to suggest the variety and persistence of the dyschromia.3,4 In South Africa, EDP is also referred to as erythema chronicum figuratum melanodermicum.

Although EDP is a distinct entity, little progress has been made in elucidating its pathogenesis or finding effective treatments. In the past, many considered it a controversial entity and regarded it as a form of lichen planus or lichen planus actinicus.5,6,7 A proposed clinical classification has been devised, dividing ashy dermatosis from EDP, with the former lacking the erythematous borders, and then adding a third category for simulators such as the lichen planus variety and medication-induced melanodermas.8

CLINICAL PRESENTATION

Individuals with Fitzpatrick skin types III to VI are predominantly affected. Most diagnosed patients are Latin American; however, the disease has been described in Asians and darker-pigmented people, but rarely in non-Hispanic Caucasians. It has been reported in most regions of the world. There is no clear sex predilection, although several reviews relate a slightly higher incidence in women.4,9,10 The disease predominantly affects adults between the second and third decades of life but can occur at any age, including in prepubertal children as young as 2 years of age [Table 30-1].11

TABLE 30-1Clinical presentation of erythema dyschromicum perstans

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