Angiohistiocytoma with giant cells D23.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

multinucleate cell angiohistiocytoma

History
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Smith and Wilson Jones, 1985

Definition
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Rare, mostly asymptomatic, slowly growing, benign tumour of the skin of unknown etiology and pathogenesis.

Occurrence/Epidemiology
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Very rare (less than 50 cases have been reported in the literature worldwide).

Etiopathogenesis
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Unknown. Triggering by a primary inflammatory event is discussed.

Manifestation
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Mainly middle-aged women (w:m=3:1).

Localization
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Mainly acra, legs, less often face.

Clinical features
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Solitary or multiple, occasionally also disseminated or generalized, 0.3-3.0 cm in size, pale reddish to livid, firm, flat, asymptomatic (mild itching is rarely described) papules with smooth surface, which tend to confluence.

Histology
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  • Beneath a slightly acanthotic epidermis there is a varying density, partly perivascular, partly diffuse infiltrate of lymphocytes, spindle-shaped cells and bizarre, large, multinucleated giant cells as well as numerous vessel incisions.
  • Endothelial cells: positive staining with factor VIII related antigen, Ulex europaeus I lectin, CD31, CD 34.
  • Giant cells (multinuclear histiocytes): positive staining with vimentin.
  • Mononuclear histiocytes: positive staining with CD 68, lysozyme, alpha-1 antitrypsin; negative for S100 protein and CD1a.

Differential diagnosis
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Since the clinical picture is very heterogeneous (solitary and multiple, isolated or confluent papules, no symptoms) the diagnosis is based on the histological findings. As clinical differential diagnosis the following diseases are possible: A clinical evaluation is not made.

Therapy
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Lesions arranged in groups can be ablated with the laser (CO2 laser), individual lesions can be removed surgically, e.g. by punching. Recurrence rate apparently low (?).

Literature
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  1. Jones EW et al (1990) Multinucleate cell angiohistiocytoma: an acquired vascular anomaly to be distinguished from Kaposi's sarcoma. Br J Dermatol 122: 651-663
  2. Puig L (2002) Multinucleate cell angiohistiocytoma: a fibrohistiocytic proliferation with increased mast cell numbers and vascular hyperplasia. J Cutan catheter 29: 232-237
  3. Smith NP, Wilson Jones E (1985) Multinucleate cell angiohistiocytoma: a new entity. Br J Dermatol 113: 15
  4. Vakeva L et al (2003) Multinucleate cell angiohistiocytoma: a report of four cases in Finland. Acta Derm Venereol 83: 222-223
  5. Weinstabl A et al (2010) Angiohistiocytoma with giant multinucleated cells. JDDG 8: 951-952

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

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Last updated on: 29.10.2020