Linear and whorled nevoid hypermelanosis

General Information (adopted from Orphanet):

Synonyms, Signs: LWNH HYPERPIGMENTATION, PROGRESSIVE CRIBRIFORM AND ZOSTERIFORM, INCLUDED
PCZH, INCLUDED
Number of Symptoms 15
OrphanetNr: 79150
OMIM Id: 614323
ICD-10: L81.4
UMLs: C1304501
MeSH:
MedDRA:
Snomed: 403803002

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant inheritance
[Omim]
Age of onset: Infantile onset
[Omim]

Disease classification (adopted from Orphanet):

Parent Diseases: Genetic hyperpigmentation of the skin
 -Rare genetic disease
Hyperpigmentation of the skin
 -Rare skin disease

Symptom Information: Sort by abundance 

1
(HPO:0007572) Hyperpigmented streaks 2 / 7739
2
(HPO:0001880) Eosinophilia 35 / 7739
3
(OMIM) Mild eosinophilia may occur 1 / 7739
4
(OMIM) No incontinence of pigment 1 / 7739
5
(OMIM) Increased melanin in the basal layer 1 / 7739
6
(OMIM) Mild elongation of the rete ridges 1 / 7739
7
(HPO:0000006) Autosomal dominant inheritance 2518 / 7739
8
(OMIM) Reticulate hyperpigmentation composed of 1- to 5-mm light brown spots with clearly defined borders that may coalesce 1 / 7739
9
(OMIM) Hyperpigmented streaks following lines of Blaschko 1 / 7739
10
(OMIM) Normal or mildly increased numbers of basal melanocytes 1 / 7739
11
(HPO:0003593) Infantile onset 249 / 7739
12
(OMIM) Hyperpigmented whorls 1 / 7739
13
(OMIM) Lesions appear on the trunk and limbs but the face, palms, soles, and mucosa are spared 1 / 7739
14
(OMIM) No melanophages 1 / 7739
15
(OMIM) Increased numbers of melanosomes in keratinocytes 1 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Linear and whorled hypermelanosis (LWNH) is a benign skin condition characterized by onset in infancy of hyperpigmented regions composed of small light brown spots that coalesce with age and follow the lines of Blaschko on the trunk and ...
Clinical Description OMIM Iijima et al. (1987) reported 2 unrelated Japanese children with early-onset reticulate hyperpigmentation distributed in a zosteriform pattern along the lines of Blaschko on the trunk and limbs, but sparing the face, palms, and soles. The first girl ...