Hypermelanosis nevoid, striped and vertebralL81.4

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Linear and whorled nevoid hypermelanosis; Nevoid stripe- and vertebral hypermelanosis; Progressive cribriform and zosterifom hyperpigmentation; Striped and vertebral hypermelanosis; Striped and vertebral nevoidal hypermelanosis

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HistoryThis section has been translated automatically.

Kalter et al. 1988

DefinitionThis section has been translated automatically.

Mostly sporadically occurring pigment anomaly with stripe- and reticular, partly also vortical arrangement, following the Blaschko lines.

Occurrence/EpidemiologyThis section has been translated automatically.

Rarely, both sexes are equally affected

EtiopathogenesisThis section has been translated automatically.

Mosaic dermatosis with mutation of the KITLG gene located on chromosome 12q21.12-q22 (Sorlin A et al. 2017). The KIT ligand gene and its receptor kit control processes in hematopoiesis, melanogenesis and gametogenesis.

Clinical featuresThis section has been translated automatically.

Manifestation within the first two years of life. Typical are linear or spiral, often also reticular brownish hyperpigmentations arranged in the Blaschko lines, in which no recognizable inflammatory phase precedes (distinction to Incontinentia pigmenti).

Systemic involvement associated with this cutaneous appearance is described: atrial septal defect, deafness, mental retardation, tooth, eye and skeletal changes, T-cell functional defects, blood eosinophilia and pseudohermaphroditism.

HistologyThis section has been translated automatically.

Hyperpigmentation of the epithelium; pigment incontinence.

Differential diagnosisThis section has been translated automatically.

Incontinentia pigmenti; systematized epidermal nevus; nevus fuscocoeruleus deltoideoacromialis.

TherapyThis section has been translated automatically.

Brightening with dye laser.

Note(s)This section has been translated automatically.

clinically it is the hypermelanotic counterpart of the naevus Ito.

LiteratureThis section has been translated automatically.

  1. Amyere M et al (2011) KITLG mutations cause familial progressive hyper- and hypopigmentation.
    J Invest Dermatol 131:1234-1239.
  2. Hofmann U, Wagner N, Grimm T, Bröcker EB, Hamm H (1998) Linear and whorled nevoid hypermelanosis. Case report and review of the literature. dermatologist 49: 408-412
  3. Megarbane A et al (2002) Linear and whorled nevoid hypermelanosis with bilateral giant cerebral aneurysms. On J Med Genet 112: 95-98
  4. Mendiratta V et al (2001) Linear and whorled nevoid hypermelanosis. J Dermatol 28: 58-59
  5. Sorlin A et al (2017) Mosaicism for a KITLG mutation in Linear and Whorled Nevoid Hypermelanosis.
    J Invest Dermatol 137:1575-1578.

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer