HistoryThis section has been translated automatically.
Ramirez, 1957; Ramirez described the affected Central American patients as "Los cenicientos" the "ash-coloured" because of their skin colouring. Convit et al. 1961
DefinitionThis section has been translated automatically.
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.
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Occurrence/EpidemiologyThis section has been translated automatically.
Preferably found in Latin America in dark-skinned individuals; less frequently in light-skinned Caucasians, less frequently in Asians
EtiopathogenesisThis section has been translated automatically.
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:
ManifestationThis section has been translated automatically.
Variable age: 3-80 years; average in larger collectives: 35 years; rare in children.
In adulthood without annual limit.
LocalizationThis section has been translated automatically.
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.
Clinical featuresThis section has been translated automatically.
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.
HistologyThis section has been translated automatically.
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 diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
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).
Progression/forecastThis section has been translated automatically.
Chronic, years-long course. Slow regression possible.
Note(s)This section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- 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
- Chun JS et al (2009) A case of unilateral ashy dermatosis. Ann Dermatol 21:432-434.
- Cutrì FT et al (2011) Lichen planus pigmentosus-like ashy dermatosis. Dermatol Reports 6:e46.
- Degos R et al (1978) La pigmentation maculeuse eruptive idiopathique. Ann Dermatol Venereol 105: 177-182
- Imanishi H et al (2011) Two cases of unilateral ashy dermatosis. Case Rep Dermatol. 3:1-4
- Keisham C et al (20134) Ashy dermatosis in an 8-year-old Indian child. Indian Dermatol Online J 4:30-32
- Lee SJ et al (1999) Erythema dyschromicum perstans in early childhood. J Dermatol 26: 119-121
- Naik NS (2003) Erythema dyschromicum perstans and vitiligo. Dermatol Online J 9: 25
- Phay KL et al (1987) Erythema dyschromicum perstans (Ashy dermatosis). J Dermatol 14: 502-505
- Ramirez CO (1957) The ashy dermatosis (erythema dyschromicum perstans): epidemiological study and report of 139 cases. Cutis 3: 244-247
- Ramirez CO (1957) Los cenicientos: problema clinico. Report of the First Central American Congress of Dermatology. San Salvador, pp. 122-130
- Silverberg NB et al (2003) Erythema dyschromicum perstans in prepubertal children. Pediatric Dermatol 20: 398-403
- Szliska C et al (1991) Erythema dyschromicum perstans (Ashy Dermatosis). Dt Dermatol 39: 1561-1563
Incoming links (11)Ashy dermatosis; Cenicienta dermatosis; Dyschromatosis universalis hereditaria; Erythema; Erythema perstans faciei; Hyperpigmentation, circumscribed; Interface dermatitis; Lichen planus pigmentosus (inversus); Pigment incontinence; Pinta; ... Show all
Outgoing links (18)Camouflage; Clofazimine; Drug reaction fixe; Erythema; Glucorticosteroids topical; Graft-versus-host disease; Hydroquinone; Hyperpigmentation; Incontinentia pigmenti (Bloch-Sulzberger); Interface dermatitis; ... Show all
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