Angioma serpiginosum L81.7

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Angiektasia serpiginosum Hutchinson; Angioma serpiginosum Crocker; Angioma serpiginosum Hutchinson; infective angioma Hutchinson

History
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Hutchinson, 1889; Radcliffe Crocker, 1893

Definition
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Rare, superficial, nevoid vascular neoplasms, occurring predominantly in girls and young women. Occasionally associations with systemic diseases have been described (Sjögren's syndrome, primary biliary cirrhosis, paraproteinemia), without a pathogenetic explanation for this association.

Etiopathogenesis
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Unknown, probably X-linked dominant inherited mosaic.

Manifestation
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Mostly occurring in childhood (80% of patients are affected before the age of 18). Rarely present at birth. Gynecotropy: Women are affected about 9 times as often as men.

Localization
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Thighs, buttocks, mamma, arms, trunk parts near the extremities.

Clinical features
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Disseminated, 1-2 mm in size, bright red to purple-coloured, sometimes non-removable, dot-shaped spots. Occasionally also telangiectasias. Serpiginous, gyrated or linear patterns are produced by grouped arrangement. Occurring on one or both sides (rather symmetrical). Palms, soles and the mucous membranes close to the skin remain free.

Manifestation along the Blaschko lines is described but rather the rarity.

Histology
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Convolute dilated capillaries with or without thickening of the vessel wall in the papillary stratum, sometimes also capillary proliferation and erythrocyte extravasations. No inflammatory infiltrates.

Differential diagnosis
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Therapy
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Laser therapy(e.g. pulsed dye laser), if necessary diathermy needle sclerotherapy, cosmetic covering.

Progression/forecast
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Healing without atrophy possible, usually after years.

Literature
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  1. Al Hawsawi K et al (2003) Linear angioma serpiginosum. Pediatric dermatol 20: 167-168
  2. Chavaz P, Laugier P (1981) Angioma serpiginosum Hutchinson: ultrastructural study (author's transl). Ann Dermatol Venereol 108: 429-436
  3. Gerbig A et al (1995) Angioma serpiginosum, a skin change along the Blaschko lines. dermatologist 46: 847-849
  4. Katta R et al (2003) Angioma serpiginosum with extensive cutaneous involvement. J Am Acad Dermatol 42: 384-385
  5. Namazi MR et al (2003) Angioma serpiginosum. Dermatol Online J 9: 19
  6. Radcliffe-Crocker H (1893) Diseases of the skin: their description, pathology, diagnosis, and treatment, with special reference to the skin eruptions of children. P. Blakiston, son & Co, Philadelphia, S. 646
  7. Sandhu K, Gupta S (2005) Angioma serpiginosum: report of two unusual cases. J Eur Acad Dermatol Venereol 19: 127-128

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Last updated on: 29.10.2020