Angiokeratoma corporis diffusum, idiopathic E75.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.05.2024

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Synonym(s)

Diffuse idiopathic angiokeratoma corporis; Idiopathic diffuse angiokeratoma corporis

Definition
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In contrast to angiokeratoma corporis diffusum, this is a rare disease with angiokeratomas of the skin and associated malformations that is not associated with a metabolic disorder. An intellectual disability does not usually occur.

Clinical features
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Herd-shaped, blue-blackish vascular lesions occurring in childhood, signs of dysmorphia and in individual cases mental retardation (possibly asphyxia at birth).

Capillaroscopy: Torqued and tufted capillaries of normal size, partly with halos. Small bleedings on individual fingers on both sides, partly avascular fields, capillary density reduced by 6/mm2. Plexus Visibility Score 0, no evidence of vascular changes in internal organs.

Laboratory
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No detection of pathological metabolic products (lipids, water-soluble oligosaccharides) or enzymes.

Histology
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Pronounced ectasia of the vessels in the upper corium, reaching right under the epidermis.

No abnormal lysosomes in lesional endothelial cells and fibroblasts in 5 patients examined by electron microscopy.

Literature
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  1. Gerbig AW et al (1995) Angiokeratoma corporis diffusum without associated metabolic disorder. dermatologist 46: 785-788
  2. Laxmisha C et al (2003) Cutaneous variant of angiokeratoma corporis diffusum. Dermatol Online J 9: 13

Outgoing links (1)

Angiokeratomas (overview);

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Last updated on: 19.05.2024