Berlin syndrome Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Congenital generalized melanoleukoderma; ectodermal dysplasia Berlin type; Melanoleukoderma congenital generalized

History
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Berlin, 1961

Definition
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Extremely rare congenital ectodermal dysplasia with reduced hairiness, dry atrophic skin with patchy hyperpigmentation, palmoplantar keratoses.

Clinical features
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  • Skin changes: Hyperpigmentation of the extremities with small spots (so-called leopard skin), tendency to atrophic scarring. Anetodermia, poikiloderma. Palmoplantar keratoses with hyperhidrosis. Teleangiectasia of the lips. Lack of lanugo hair, underdeveloped sebaceous gland apparatus in the area of the hairy head.
  • Extracutaneous manifestations: delayed tooth growth, short stature, stilt-shaped legs, mental retardation, hypogonadism in male patients.

Therapy
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Caring and refatting ointments (e.g. linola grease), covering creams or pastes can provide visual help. Keratotic changes can be treated with urea-containing or salicylic acid-containing ointments or oils.

Literature
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  1. Berlin CI (1961) Congenital generalized melanoleucoderma associated with hypodontia, hypotrichosis, stunted growth and mental retardation occurring in two brothers and two sisters. dermatologica 123: 227-243

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Last updated on: 29.10.2020