Angiohistiocytoma with giant cells D23.9

Smith and Wilson Jones, 1985

Rare, usually asymptomatic, slow-growing, benign tumor of the skin of unknown etiology and pathogenesis.

Very rare (less than 200 cases have been reported in the literature worldwide to date - Frew JW 2015).

Unknown. Triggering by a primary inflammatory event is discussed.

Mainly women in middle age (w:m=3:1). In a retrospective analysis of 142 cases (Frew JW 2015), the average age at onset of the disease was 50.1 years.

Mainly acra, legs, less often face.

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.

Under a slightly acanthotic epidermis there is a variably dense, partly perivascular, partly diffuse infiltrate of lymphocytes, spindle-shaped cells and bizarre, large, multinucleated giant cells as well as numerous vascular 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.

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:

• Kaposi's Sarcoma
• Histiocytoma(s)
• Anulare granuloma.
• Lichen planus
• Arthropod reaction (see below pseudolymphomas of the skin)

A clinical evaluation is not made.

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 (?).

1. Frew JW (2015) Multinucleate cell angiohistiocytoma: clinicopathological correlation of 142 cases with insights into etiology and pathogenesis. Am J Dermatopathol 37:222-228.

2. Grgurich E et al (2019) Multinucleate cell angiohistiocytoma: case report and literature review. J Cutan Pathol 46:59-61.

3. 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
4. Puig L (2002) Multinucleate cell angiohistiocytoma: a fibrohistiocytic proliferation with increased mast cell numbers and vascular hyperplasia. J Cutan Pathol 29: 232-237
5. Smith NP, Wilson Jones E (1985) Multinucleate cell angiohistiocytoma: a new entity. Br J Dermatol 113: 15
6. Vakeva L et al (2003) Multinucleate cell angiohistiocytoma: a report of four cases in Finland. Acta Derm Venereol 83: 222-223
7. Weinstabl A et al (2010) Angiohistiocytoma withz multinucleate giant cells. JDDG 8: 951-952