Ashy dermatosis 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 chronic-relapsing, with characteristic, dull, grey-brown patches, slightly or non-itchy, large-spotted (patches), inflammatory skin disease, which is characterized histologically by a discrete interface dermatitis with pronounced pigment incontinence. As the disease mainly occurs in dark-skinned people, the combination of a dark complexion and dark melanin deposits in the dermis leads to the characteristic "ashy" 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.

As the disease mainly occurs in dark-skinned people, it is conceivable that a previous dermatitis is masked by the dark skin and only the post-inflammatory hyperpigmented final condition is noticed.

Some authors consider the disease to be the "final stage of a lichen ruber exanthematicus"(lichen planus exanthematicus) or a lichenoid drug exanthema.

Also discussed are:

• post-inflammatory hyperpigmentation in drug intolerance (X-ray contrast agents, omeprazole, ethambutol, fluoxetine and chlorothalonil (Nguyen K et al.2019)
• Contact with environmental toxins, detergents or light stabilizers
• Focal events, parasitic and viral infections (hepatitis B).
• endocrine and immunological influences as well as a genetic disposition.0

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

In adulthood without annual limit.

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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

It is important to take a detailed medical history with a subtle medication survey. Discontinuation or conversion of all possible medication.

Local therapy with topical glucocorticoids (class I)

Blande nourishing local therapeutics. Textile sun protection.

Alternatively: 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) taken for several months has been described on the basis of a few individual case studies (not recommended). Dapsone, minocycline, tranexamic acid, pentoxyfulline and macrolides have been mentioned as further systemic therapies (Burlando M et al.2022).

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.

1. Burlando M et al. (2022)* Erythema dyschromicum perstans (ashy dermatosis) under dimethyl fumarate. J Dtsch Dermatol Ges 20:1502-1504.
2. 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
3. Chun JS et al (2009) A case of unilateral ashy dermatosis. Ann Dermatol 21:432-434.
4. Cutrì FT et al (2011) Lichen planus pigmentosus-like ashy dermatosis. Dermatol Reports 6:e46.
5. Degos R et al (1978) La pigmentation maculeuse eruptive idiopathique. Ann Dermatol Venereol 105: 177-182
6. Imanishi H et al. (2011) Two cases of unilateral ashy dermatosis. Case Rep Dermatol. 3:1-4
7. Keisham C et al (20134) Ashy dermatosis in an 8-year-old Indian child. Indian Dermatol Online J 4:30-32
8. Lee SJ et al (1999) Erythema dyschromicum perstans in early childhood. J Dermatol 26: 119-121
9. Naik NS (2003) Erythema dyschromicum perstans and vitiligo. Dermatol Online J 9: 25
10. Nguyen K et al.(2019)* Ashy dermatosis: a review. Dermatol Online J. 25(5):13030/qt44f462s8.
11. Phay KL et al (1987) Erythema dyschromicum perstans (ashy dermatosis). J Dermatol 14: 502-505
12. Ramirez CO (1957) The ashy dermatosis (erythema dyschromicum perstans): epidemiological study and report of 139 cases. Cutis 3: 244-247
13. Ramirez CO (1957) Los cenicientos: problema clinico. Report of the First Central American Congress of Dermatology. San Salvador, pp. 122-130
14. Silverberg NB et al (2003) Erythema dyschromicum perstans in prepubertal children. Pediatr Dermatol 20: 398-403
15. Szliska C et al (1991) Erythema dyschromicum perstans (Ashy Dermatosis). Dt Dermatol 39: 1561-1563