Diffuse dermal angiomatosis D18.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 05.08.2021

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Synonym(s)

Diffuse dermal angiomatosis: diffuse cutaneous angiomatosis

History
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Krell, 1994

Definition
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Rare, acquired, benign, skin-limited vascular proliferations with painful, often centrally exuded plaques and diffuse infiltrative growth pattern.

Etiopathogenesis
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Controversial. Discussed are the reactive, ischemia-induced proliferation of cutaneous capillaries, peripheral atherosclerosis and the formation of antibody-antibodies.

Localization
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Extremities, trunk, breast (mammae)

Clinical features
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Solitary or multiple, painful, often centrally ulcerated, brownish-livid, blurred, bizarre or flame-figure-like plaques. The lesions arise subacutely and spontaneously and may regress rapidly even without therapy. They are often found near surgical scars or dialysis shunts; also in areas with chronic venous insufficiency.

Diffuse dermal angiomatosis of the female breast occupies a special pathogenetic position (see Angiomatosis diffuse cutaneous of the female breast below).

Histology
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Dense network of regularly structured capillary vessels that run through the entire corium. The vessels are formed by CD31- and CD34-positive endothelia and are surrounded by smooth muscle actin-positive pericytes in a cuff-shaped manner. Glomerular vascular structures and atypical endothelial mitoses are absent.

Diagnosis
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Clinic, Histology

Therapy
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Surgical restoration of the arterial blood supply. Trial with systemic glucocorticoids such as prednisolone (e.g. Decortin H) in medium dosages.

Progression/forecast
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Complete regression after restoration of the arterial blood supply. More rarely also spontaneous regression.

Literature
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  1. Frizzell MR et al (2020) Diffuse dermal angiomatosis localized to abdominal striae. Proc (Bayl Univ Med Cent) 33:419-421.
  2. Kim S et al (2002) Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with peripheral vascular atherosclerosis. Arch Dermatol 138: 456-458
  3. Kimyai-Asadi A (1999) Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with atherosclerosis. J Am Acad Dermatol 40: 257-259
  4. Krell JM et al (1994) Diffuse dermal angiomatosis. A variant of reactive cutaneous angiomatosis. J Cutan Pathol 21: 363 - 370
  5. Kutzner H et al (2002) Diffuse dermal angiomatosis. Dermatologist 53: 808-812
  6. Pichardo RO et al. (2002) What is your diagnosis? Diffuse dermal angiomatosis secondary to anticardiolipin antibodies. Am J Dermatopathol 24: 502-503
  7. Reusche R et al (2015) Diffuse dermal angiomatosis of the breast: a series of 22 cases from a single institution. Gland Surg 4:554-560.

  8. Sriphojanart Tet al (2015) Diffuse dermal angiomatosis: A Clue to the Diagnosis of Atherosclerotic Vascular Disease. Case Rep Dermatol 7:100-106.

Disclaimer

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

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Last updated on: 05.08.2021