Depigmented nevus D22.L

Lesser 1884

Rare, congenital, usually solitary, rarely multiple, white patches of the skin caused by an isolated transfer defect of melanosomes into keratinocytes.

The number of melanocytes is usually normal.

• Naevus depigmentosus simplex type I < 10 cm in diameter.
• Naevus depigmentosus simplex type II > 10 cm in diameter.

Assumed to be a melanocytic functional defect leading to decreased transfer of melanonosomes into keratinocytes.

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

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.

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

• 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.

Not required.

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

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).

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