Naevus D22.L

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 08.01.2023

Dieser Artikel auf Deutsch


Birthmark; Hamartoma of the skin; Melanocyte nevus; Times

This section has been translated automatically.

The term "nevus" has 2 meanings:

  1. As a pathogenetic term equivalent to " hamartoma of the skin". A nevus is a visible, sharply defined, often congenital, but also postnatally manifesting, long-term malformation of the skin or mucosa, which is characterized by excess, rarely also by underdevelopment or malformation, of one or more components of skin or mucosa, and which aetiopathogenetically represents a cutaneous mosaic (see also under malformation).
  2. Furthermore, the term "nevus" also finds a general use for a true, benign, melanocytic tumor(melanocytic nevus - formerly also called nevus cell nevus).

This section has been translated automatically.

Hamartomas of the skin are caused by abnormal development of the epidermis with increased or decreased pigmentation and/or of the skin appendages, vessels (vascular hamartomas), nerves or connective tissue(connective tissue nevus). Various combinations of malformations are possible (organoid nevi). They are named after the predominant tissue type (e.g. sweat gland nevus).

Systematized hamartomas of the skin are those that are distributed over a body region. Systematized hamartomas of the skin usually follow the Blaschko lines in their arrangement (classification see Tab. 1).

Differential diagnosis
This section has been translated automatically.

The term nevus is to be distinguished from the term malformation as a defective development from an embryonic anlage (e.g. branchial remnants in lateral neck cysts).

In clinical terminology, the term "nevus" is used synonymously with an acquired pigmentary tumor ( melanocytic nevus). However, most melanocytic nevi are not nevi in the sense of a "hamartoma of the skin" but are true acquired pigment cell tumors. For differential diagnosis see below. Melanocytic nevus.

This section has been translated automatically.

Classification of the nevi (n. Altmeyer 2015)

Melanocytic hamartomas

Melanocytic hamartomas


Café-au-lait spots

Nevus spilus


Nevus bleu

Mongolian spot

Nevus fuscocoeruleus ophthalmomaxillaris

Nevus fuscocoeruleus deltoideoacromialis

Melanocytic hamartoma


Congenital melanocytic nevi

Melanosis neurocutanea

BK-mole syndrome

Non-melanocytic hamartomas

Epithelial hamartomas (are called "epidermal nevi")

Nevus verrucosus


Keratosis areolae mammae naeviformis

CHILD syndrome

Nevus sebaceus

Nevus, sweat gland nevus

Curly hair nevus

Hair nevus

Connective tissue hamartomas

Lumbosacral connective tissue nevus

coarse nodular disseminated connective tissue nevus

Nevus elasticus

Vascular malformations

Nevus flammeus

Angioma serpiginosum

Nevus araneus

Sturge-Weber-Krabbe syndrome

Parkes-Weber syndrome

Teleangiectasia hereditaria haemorrhagica

Nevus anaemicus

Hamartomas of the adipose tissue

Nevus lipomatosus

Familial lipomas

This section has been translated automatically.

  1. Blum A et al (2003) The dermoscopic classification of atypical melanocytic naevi (Clark naevi) is useful to discriminate benign from malignant melanocytic lesions. Br J Dermatol 149: 1159-1164
  2. Dawson HA et al (1996) A prospective study of congenital melanocytic nevi: progress report and evaluation after 6 years. Br J Dermatol 134: 617-623
  3. Happle R (2004) Cutaneous mosaics: patterns and molecular mechanisms. Dt Medical Journal 101: 1575-1580


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


Last updated on: 08.01.2023