Atrophodermia linearis L90.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Atrophodermia linearis Moulin; Linear atrophoderma of Moulin

History
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Moulin, 1992

Definition
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In the Blaschko lines running, symptomless skin atrophy (atrophodermia) without alteration of specific laboratory parameters.

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Etiopathogenesis
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The cause is unknown. Somatic mutations in the early embryonic phase are discussed. The mutated cell clone migrates and could represent a locus minoris resistentiae in which an unknown manifestation factor later triggers the disease.

Manifestation
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With children and young adults.

Clinical features
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Multiple skin lesions, up to 5 cm in diameter, partly round or oval, partly confluent, brownish-livid, sunken skin lesions. No induration. No focal signs of inflammation. Arrangement along the Blaschko lines.

Histology
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Basal hyperpigmentation of the epidermis, ballooned keratinocytes, perivascularly increased lymphocytes in the upper dermis. No inflammatory cells or inflammatory infiltrates, no melanophages; collagenous and elastic tissue of the dermis inconspicuous.

Direct Immunofluorescence
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Deposition of IgG, IgA and IgM in the dermis (probably unspecific pattern).

Differential diagnosis
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Therapy
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  • So far, no effective therapy is known. Often improvement of the untreated course over several years.
  • Used but unsuccessful therapies: glucocorticoids, penicillin, bath-PUVA. Successes with potassium aminobenzoate (Potaba) are described in individual case reports.

Progression/forecast
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All in all a favourable prognosis but cosmetically disturbing.

Note(s)
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If skin changes make one think of a morphea, but follow the Blaschko lines, there is an atrophodermia linearis.

Literature
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  1. Baumann et al (1994) Atrophodermia linearis Moulin. A new disease picture, following the Blaschko Lines. dermatologist 45: 231-236
  2. Gansz B et al (2003) Linear symptomless skin atrophies in a 14-year-old girl. JDDG 1: 319
  3. Miteva L et al (2002) An unusual manifestation of linear atrophoderma of Moulin. Acta Derm Venereol 82: 479-480
  4. Moulin G et al (1992) Acquired atrophic pigmented band-like lesions following Blaschko's lines. Ann Dermatol Venereol 119: 729-736
  5. Rompel R et al (2000) Linear atrophoderma of Moulin. Eur J Dermatol 10: 611-613

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Last updated on: 29.10.2020