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Multinucleate cell angiohistiocytoma - Skin
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Multinucleate cell angiohistiocytoma - Skin

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Contributors: Laurie Good MD, Whitney A. High MD, JD, MEng, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Multinucleate cell angiohistiocytoma (MCA) is a rare benign fibrohistiocytic and vascular cutaneous proliferation. It is characterized by well-circumscribed papules occurring in groups that vary in color from red-brown to violaceous and are typically 2-10 mm in size. MCAs are usually found on the dorsum of the hands, wrists, and legs but may also occur on the face of middle-aged to elderly women.

The etiology of this acquired proliferation is unknown. Some believe that trauma plays a role in development, while others postulate a complicated interaction between mast cells and fibrohistiocytic cells (factor XIIIa positive cells) that leads to proangiogenic cytokine release and development of lesions.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
21985009 – Fibrohistiocytic proliferation of the skin

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Clinically, MCAs may resemble:
  • Angiofibromas – Usually on the face and often associated with tuberous sclerosis.
  • Dermatofibromas – Usually on the leg, and dimple centrally with lateral compression (Fitzpatrick's sign).
  • Granuloma annulare – Usually on the extremities; often forms annular lesions without scale or other epidermal changes. When broadly disseminated, granuloma annulare may be associated with diabetes while MCAs are not.
  • Kaposi's sarcoma (AIDS associated and non-AIDS associated) – Classic forms on the legs of elderly men, and epidemic forms associated with HIV infection; Kaposi's sarcoma is caused by infection with human herpesvirus type 8 (HHV-8). Usually more purple.
  • Sarcoidosis – Most common in African Americans from the southern US, sarcoidosis may be associated with pulmonary or other systemic involvement, whereas MCA is not.
A skin biopsy is necessary to differentiate MCAs from these entities, as they can be clinically indistinguishable.

Best Tests

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Therapy

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References

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Last Updated: 09/09/2011
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Multinucleate cell angiohistiocytoma - Skin
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Multinucleate cell angiohistiocytoma : Grouped configuration, Red color, Smooth papules
Clinical image of Multinucleate cell angiohistiocytoma
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