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Dermatology > BENIGN NEOPLASMS
Multinucleate Cell Angiohistiocytoma
Article Last Updated: Mar 29, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Diane M Scott, MD, Dermatologist and Dermatopathologist, Palm Beach Dermatology
Diane M Scott is a member of the following medical societies: American Academy of Dermatology
Coauthor(s):
Daniel Davis, MD, Associate Professor, Departments of Dermatology, Otolaryngology, and Pathology, University of Arkansas for Medical Sciences;
Frances Ramos-Ceballos, Staff Physician, Department of Dermatology, University of Arkansas for Medical Sciences
Editors: Abdul-Ghani Kibbi, MD, Chairman and Professor, Department of Dermatology, American University of Beirut Medical Center, Lebanon; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Paul Krusinski, MD, Director of Dermatology, Professor, Department of Internal Medicine, Fletcher Allen Health Care, University of Vermont; Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
benign fibrohistiocytic vascular proliferation, vascular tumor, connective tissue disorder, connective-tissue disorder
Background
Multinucleate cell angiohistiocytoma is a benign fibrohistiocytic and vascular proliferation that was first reported by Smith and Wilson Jones in 1985. It occurs predominantly on the extremities of middle-aged and elderly women.
Pathophysiology
The pathogenesis of multinucleate cell angiohistiocytoma is unknown. Some authors consider it a connective-tissue disorder, while others categorize it as a vascular tumor. Wilson Jones concluded that multinucleate cell angiohistiocytoma is a chronic inflammatory disorder of unknown cause manifested by vascular hyperplasia and connective-tissue cells that have lost their function after prolonged stimulation.
Frequency
United States
Multinucleate cell angiohistiocytoma is a rare disorder. Fewer than 50 cases have been reported as of January 2001.
Mortality/Morbidity
No morbidity or mortality is associated with this disorder.
Race
The frequency of multinucleate cell angiohistiocytoma in various races has not been reported.
Sex
The female-to-male ratio of persons affected with this disorder is approximately 5:1.
Age
Reported cases of multinucleate cell angiohistiocytoma have occurred in patients aged 24-74 years. Most cases occur in middle-aged and elderly women.
History
Multinucleate cell angiohistiocytoma presents as insidiously developing asymptomatic papules that usually develop on acral surfaces and tend to persist indefinitely.
Physical
- Multiple, grouped, well-circumscribed papules are red to violaceous to brownish in color.
- Lesions are smooth and firm, and they may be either dome shaped or flat topped.
- A few cases of lesions have been in an annular distribution, mimicking granuloma annulare.
- Lesions of multinucleate cell angiohistiocytoma occur most commonly on the extremities.
- Acral areas such as the dorsal aspects of the hands, fingers, wrists, and legs are the most frequent sites.
- Unusual locations include the forehead, the upper lip, the chest, and the orbit.
- Lesions usually are unilateral, but a few bilateral cases and one generalized case have been reported.
- Lesions are usually asymptomatic to pruritic.
Causes
Multinucleate cell angiohistiocytoma is an acquired disorder of unknown cause. Puig et al propose the existence of an interaction between mast cells and factor XIIIa–positive fibrohistiocytic cells, which might contribute to vascular proliferation by the release of various proangiogenic cytokines. Shapiro postulates that the condition may be related to trauma because it tends to occur on the dorsal aspects of the hands and around the knees.
Dermatofibroma
Granuloma Annulare
Insect Bites
Kaposi Sarcoma
Lichen Planus
Lymphocytoma Cutis
Sarcoidosis
Other Problems to be Considered
Angiofibroma
Procedures
- Lesions of multinucleate cell angiohistiocytoma often mimic other conditions. A biopsy is the best way to exclude more serious disorders (eg, sarcoidosis, Kaposi sarcoma).
Histologic Findings
Multinucleate cell angiohistiocytoma has a distinctive histologic appearance characterized by a vascular proliferation and bizarre multinucleated cells. In the upper and mid dermis, a proliferation of small blood vessels occurs, some of which have dilated lumina. The vessels have the histologic appearances of capillaries and small vessels. The endothelial cells lining the vessels are plump but lack atypia. A sparse perivascular lymphocytic infiltrate may be present. In addition, an increased number of mast cells has been reported. The vascular proliferation is embedded in a fibrous stroma rich in fibrohistiocytic cells. The collagen bundles are slightly thickened and arranged haphazardly or parallel to the epidermis. Bizarre multinucleated cells are amongst the interstitial cells. These cells have 3-10 hyperchromatic nuclei that are closely aggregated or arranged in a ringlike fashion. The cytoplasm of these cells is often angulated or scalloped in outline (see Images 1-2). Immunohistochemical studies may be useful in confirming the diagnosis of multinucleate cell angiohistiocytoma and in differentiating it from other vascular and fibrous proliferations. The lesional vessels label with factor VIII–related antigen (factor VIII-RA), EN-4, BMA-120, PAL-E, and Ulex europaeus agglutinin I (UEA-I). These findings indicate a capillary and venular nature of the vessels. Polymerase chain reaction analysis reveals no evidence of human herpesvirus-8 (HHV-8) infection, as may be found in Kaposi sarcoma. The multinucleated cells stain with vimentin, but they do not label with the macrophage markers lysozyme, MAC 387, and alpha-1-antitrypsin. The multinucleated cells are also negative for S-100 protein, factor XIII, and human leukocyte antigen DR (HLA-DR). The mononuclear interstitial cells label with vimentin, factor XIIIa, and CD68.
Medical Care
Multinucleate cell angiohistiocytoma has not been shown to respond to any medical therapy.
Surgical Care
- Surgical excision may be recommended for cosmetic reasons.
- Laser therapy has resulted in clinical resolution of the lesions with not scarring. Kopera et al reported successful treatment with an argon laser.
- Perez et al reported a case with a good response to cryosurgery.
No medical therapy is available for this condition.
Further Outpatient Care
- Further follow-up care is not needed because multinucleate cell angiohistiocytoma is not known to be associated with any systemic diseases and because it has a biologically benign course.
Complications
- No morbidity or mortality is associated with this disorder.
Prognosis
- Multinucleate cell angiohistiocytoma is a benign condition. Spontaneous resolution is uncommon.
Medical/Legal Pitfalls
- Failure to differentiate this entity from other conditions that require treatment, most notably Kaposi sarcoma, is a potential medicolegal pitfall.
| Media file 1:
Biopsy specimen of multinucleate cell angiohistiocytoma. A vascular proliferation is embedded in a stroma rich in fibroblasts and histiocytes, some of which are multinucleated. |
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Media type: Photo
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| Media file 2:
Bizarre multinucleated cells are present in the stroma surrounding the vascular proliferation. |
 | View Full Size Image | |
Media type: Photo
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Multinucleate Cell Angiohistiocytoma excerpt Article Last Updated: Mar 29, 2007
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