Erythema dyschromicum perstans L81.02

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

ashy dermatitis; Ashy dermatosis; c; cenicienta dermatosis; cinderella dermatitis; cinderella dermatosis; La pigmentation maculeuse eruptive idiopathique; Los cenicientos; melanosis lenticularis generalisata; Pigmentatio idiopthica maculosa eruptiva acquisita

History
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Ramirez, 1957; Ramirez described the affected Central American patients as "Los cenicientos" the "ash-coloured" because of their skin colouring. Convit et al. 1961

Definition
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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.

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

Etiopathogenesis
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Unsolved. Since the disease occurs mainly in dark-skinned people, it is conceivable that a previously expired dermatitis is masked by the dark skin and only the post-inflammatory hyperpigmented final state is noticed. Thus the disease is regarded by some authors as "final state of a lichen ruber exanthematicus"(lichen planus exanthematicus) or a lichenoid drug lxanthema.

Discussions continue:

  • post-inflammatory hyperpigmentation in drug intolerance (omeprazole)
  • Contact with environmental noxins, detergents or light stabilizers
  • Focus events, viral infections (hepatitis B).
  • endocrine and immunological influences and a genetic disposition.

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

In adulthood without annual limit.

w>m

Localization
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In larger collectives the following distribution pattern was found:

  • Fuselage 69%
  • Neck/neck: 47
  • Arms: 40%
  • Face: 30%
  • Legs: 20%.
  • Recessing of capillitium, palms, soles and mucous membranes.

Clinical features
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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.

Histology
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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.

Differential diagnosis
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Therapy
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  • A detailed anamnesis with a subtle collection of medication is important. Discontinuation or conversion of all possible medicines.
  • Local therapy with topical glucocorticoids (class I)
  • Blande nourishing local therapeutics. Textile sun protection.
  • Alternative: concealer paste or camouflage (e.g. with Dermacolor). If necessary therapy of the basic disease.
  • Alternative: Trials with bleaching agents like 3% (to 4%) hydroquinone cream are rather frustrating.
  • Alternative: Topical retinoids.
  • Alternative: topical tacrolimus
  • Therapeutic successes with the use of clofazimine (Lamprene) over several months are described on the basis of a few individual case studies (not recommended).

Progression/forecast
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Chronic, years-long course. Slow regression possible.

Note(s)
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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.

Literature
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  1. Chang SE det al (2015) Clinical and histological aspect of erythema dyschromicum perstans in Korea: A review of 68 cases. J Dermatol 42:1053-1057
  2. Chun JS et al (2009) A case of unilateral ashy dermatosis. Ann Dermatol 21:432-434.
  3. Cutrì FT et al (2011) Lichen planus pigmentosus-like ashy dermatosis. Dermatol Reports 6:e46.
  4. Degos R et al (1978) La pigmentation maculeuse eruptive idiopathique. Ann Dermatol Venereol 105: 177-182
  5. Imanishi H et al (2011) Two cases of unilateral ashy dermatosis. Case Rep Dermatol. 3:1-4
  6. Keisham C et al (20134) Ashy dermatosis in an 8-year-old Indian child. Indian Dermatol Online J 4:30-32
  7. Lee SJ et al (1999) Erythema dyschromicum perstans in early childhood. J Dermatol 26: 119-121
  8. Naik NS (2003) Erythema dyschromicum perstans and vitiligo. Dermatol Online J 9: 25
  9. Phay KL et al (1987) Erythema dyschromicum perstans (Ashy dermatosis). J Dermatol 14: 502-505
  10. Ramirez CO (1957) The ashy dermatosis (erythema dyschromicum perstans): epidemiological study and report of 139 cases. Cutis 3: 244-247
  11. Ramirez CO (1957) Los cenicientos: problema clinico. Report of the First Central American Congress of Dermatology. San Salvador, pp. 122-130
  12. Silverberg NB et al (2003) Erythema dyschromicum perstans in prepubertal children. Pediatric Dermatol 20: 398-403
  13. Szliska C et al (1991) Erythema dyschromicum perstans (Ashy Dermatosis). Dt Dermatol 39: 1561-1563

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020