Dystrophia bullosa hereditaria - type maculatus seu amsterdam Q81.8

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Definition
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X-linked recessive variant of a dystrophic epidermolysis with early loss of scalp hair, subepidermal blisters, intermittent spotty hyper- and depigmentation of the face and extremities. Proportional dwarfism, microcephaly, mental retardation.

Differential diagnosis
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Incontinentia pigmenti.

Therapy
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Symptomatic therapy, see below Epidermolysis bullosa group.

Progression/forecast
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Death usually before the age of 25.

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Incoming links (1)

Hypotrichosis;

Outgoing links (2)

Epidermolysis bullosa hereditaria (overview); Pigment incontinence;

Disclaimer

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

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer Prof. Dr. med. Peter Altmeyer

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