Depigmented nevus D22.L

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Dr. Elisabeth Hanf

All authors of this article

Last updated on: 09.01.2021

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Synonym(s)

achromatic nevus; achromic nevus; Hypomelanotic nevus; nevus achromicus; nevus albus; Nevus hypomelanotic

History
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Lesser 1884

Definition
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Rare, congenital, mostly solitary, more rarely multiple, white patches of the skin caused by an isolated transfer defect of the melanosomes into the keratinocytes. The number of melanocytes is usually normal.

Classification
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A distinction is made between:
  • Naevus depigmentosus simplex type I < 10 cm in diameter.
  • Naevus depigmentosus simplex type II > 10 cm in diameter.

Occurrence/Epidemiology
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About 1% of newborns are affected.

Etiopathogenesis
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A melanocytic functional defect is assumed, which leads to a reduced transfer of melanin into the keratinocytes.

Localization
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Ubiquitously occurring, more often on the trunk and the upper and lower extremities. Rare face.

Clinical features
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One distinguishes the following forms:

  • Solitary: Roundish or angular, also bizarrely configured, bright or white-yellow spot.
  • Segmental patterns are described; also systematized (aligned according to the Blaschko lines) following the Blaschko lines, bizarrely configured, white spot patterns may occur. The combination with a nevus spilus is unusual.
  • S.a.u. Incontinentia pigmenti achromians; s.a. Hypomelanosis Ito.

Histology
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Normal or reduced number of melanocytes, which typically contain fewer melanosomes

Differential diagnosis
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  • Vitiligo (segmental vitiligo): acquired, not congenital. Histologically melanocytes are absent.
  • Naevus anaemicus: On rubbing no reactive hyperemia of the skin occurs(in contrast to the nevus depigmentosus).
  • Ash-leaf-like hypopigmentations (mainly on the trunk and the proximal extremity) in Pringle-Bourneville phacomatosis.
  • Pityriasis alba: Common in atopic eczema. The skin is usually scaly and dry.
  • Piebaldism: This pigment anomaly is described as a congenital, autosomal dominant inherited, circumscribed, systematized white spot on the skin (complete absence of melanocytes in lesional skin). Whether there is a difference between the two diseases is still unclear.

Therapy
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Not required.

Experimental: Approaches involving the implantation of cultured melanocytes should be considered experimental.

Progression/forecast
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No regression. No progression.

Note(s)
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The name naevus "depigmentosus" is somewhat confusing in that the leukoderms are generally not completely depigmented but only hypopigmented. It can be assumed that the depigmentosus nevus is a minus variant (no systemic involvement) of the Blaschko-linear hypomelanosis (synonym: hypomelanosis Ito).

Literature
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  1. Böhm M (2015) Differential diagnosis of hypomelanosis. dermatologist 66: 945-958
  2. Chokoeva A et al (2015) Nevus depigmentosus associated with nevus spilus: first report in the world literature. Georgian Med News 248:73-76
  3. Cohen J et al (2014) Analysis of 36 cases of Blaschkoid dyspigmentation: reading between the lines of Blaschko. Pediatric Dermatol 31:471-476
  4. Mulekar SV et al (2011) Nevus depigmentosus treated by melanocyte-keratinocyte transplantation. J Cutan Aesthet Surgery 4:29-32
  5. Oiso N et al (2011) The diagnostic usefulness of dermoscopy for nevus depigmentosus. Eur J Dermatol 21:639-640
  6. Oiso N et al (2011) Melanocytic nevi in nevus depigmentosus in a region of the body exposed to sunlight. J Eur Acad Dermatol Venereol 25:491-492
  7. of Geel N et al (2010) Long-term results of noncultured epidermal cellular grafting in vitiligo, halo naevi, piebaldism and naevus depigmentosus. Br J Dermatol 163: 1186-1193

Disclaimer

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

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Last updated on: 09.01.2021