Erythema dyschromicum perstans L81.02

Ramirez, 1957 (Ramirez described the affected Central American patients as "Los cenicientos" the "ash-colored ones" because of their skin colorations); Vegas et Rodriguez 1960; Convit et al. 1961;

Rare, acquired, chronic or chronically recurrent, with characteristic, dull, greyish-brown discolouration accompanied by slight or non-itching, large spots (patches), inflammatory skin disease histologically characterized by a discrete interface dermatitis with pronounced pigment incontinence. Since the disease occurs mainly in dark-skinned people, the combination of dark epidermis and dark melanin deposits in the dermis leads to the characteristic "ash-like" discoloration of the skin.

Preferably found in Latin America in dark-skinned individuals; less frequently in light-skinned Caucasians, less frequently in Asians

Unexplained. The entity of the disease is questionable.

Since the disease occurs mainly in dark-skinned people, it is conceivable that a previously developed dermatitis is masked by the dark skin and only the postinflammatory hyperpigmented final state is noticed.

Thus, the condition is considered by some authors to be the "end state of a lichen ruber exanthematicus"(lichen planus exanthematicus) or a lichenoid drug exanthema.

Discussed further are:

• postinflammatory hyperpigmentation due to drug intolerance (omeprazole)
• contact with environmental toxins, detergents or photoprotective agents
• focal events, viral infections (hepatitis B).
• endocrine and immunological influences as well as a genetic disposition.

Variable age: 3-80 years; average in larger collectives: 35 years; rare in children.

In adulthood without annual limit.

w>m

In larger collectives, the following distribution pattern emerged:

• Trunk 69%
• Neck: 47%
• arms: 40%
• Face: 30%
• legs: 20%
• Exclusion of capillitium, palms, soles and mucous membranes.

Initially small spots 0.1-0.5 cm in size, roundish to oval, also bizarrely configured, blurred, slightly itchy grey-brown or greyish-bluish (ashy-dermatosis) spots.

These can be aligned along the skin tension lines.

Unilateral or linear distribution patterns are rare.

Up to 5.0 cm or larger patches result from appositional growth or confluence. No desquamation.

Occasionally, especially when viewed from the side, a slight violet erythema is visible in the lesions in addition to the predominant brown tone.

Picture of"interface dermatitis" with rather thinning, subepidermal, lymphocytic infiltrate with focal exocytosis, hydropic degeneration of the basal cell series. Pronounced pigment incontinence; round cell infiltrates in the papillary body. In a late phase of the inflammation, only melanophages may appear.

• Clinical:
  • inversedplanuspigmentosuslichen
  • Hypomelanosis Ito;
  • Lichen planus atrophicans;
  • Incontinentia pigmenti, Bloch-Sulzberger type;
  • incontinentia pigmenti,
  • Erythema in malignant visceral tumours and rheumatic infections;
  • drug reaction, fixed.
• Histological:
  • lichen planus
  • lichen-planus type keratosis
  • Lichenoid graft-versus-host disease (GvHD)
  • Lupus erythematosus with lichenoid infitrate

A detailed anamnesis with a subtle medication survey is important. Discontinuation or conversion of all medications in question.

Local therapy with topical glucocorticoids (class I).

Bland care local therapeutics. Textile sunscreen.

Alternative: Covering paste or camouflage (e.g. with Dermacolor). If necessary, therapy of the underlying disease.

Alternative: Attempts with bleaching agents such as 3% (up to 4%) hydroquinone cream are rather frustrating.

Alternative: Topical retinoids.

Alternative: topical Tacrolimus

Therapeutic success with clofazimine (Lamprene) for several months has been described on the basis of some single case studies (not recommended).

Chronic, years-long course. Slow regression possible.

The clinical picture described by Degos in 1978 as "Pigmentatio maculosa eruptiva idiopathica" may be considered a variant of the erythema dyschromicum perstans in fair-skinned people.

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3. Cutrì FT et al (2011) Lichen planus pigmentosus-like ashy dermatosis. Dermatol Reports 6:e46.
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11. Ramirez CO (1957) Los cenicientos: problema clinico. Report of the First Central American Congress of Dermatology. San Salvador, pp. 122-130
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13. Szliska C et al (1991) Erythema dyschromicum perstans (Ashy Dermatosis). Dt Dermatol 39: 1561-1563