Angiolymphoid hyperplasia with eosinophilia L98.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

epithelioid hemangioma; hemangioma epitheloides; histiocytic hemangioma; Histiocytoid hemangioma; papular angioplasia

History
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Wells and Whimster, 1969

Definition
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Rare acquired, acquired, dermal or subcutaneously located, benign, nodular vascular neoplasia associated with eosinophilia, the pathogenesis of which is still unclear. The first describers evaluated their findings as late stage of M. Kimura.

However, the own entity seems to be secured.

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Occurrence/Epidemiology
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Women > Men

Etiopathogenesis
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Connections with arterio-venous fistulas, vascular malformations and reactive proliferations after trauma are discussed.

Manifestation
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Adults, mostly 20-50. LJ.

Localization
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Head, especially ear region, forehead and capillitium, neck; more rarely extremities and genital area, vulva or penis.

Clinical features
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Solitary, more rarely grouped in plural, symptomless or painful, also itchy, red or brown-red, brown, firm, about 0.5-0.8 cm large, papules and nodes with smooth surface. Also occurring as deep knots.

Laboratory
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Eosinophilia possible in 20-30% of patients.

Histology
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Either in the dermis or subcutis, dense solid cell proliferates with smaller roundish capillary cavities. Centrally located larger vascular spaces lined by prominent "epithelioid" endothelials (cobblestone aspect). These endothelial cells were responsible for the synonymous term "epitheloid hemangioma". Variably dense infiltrate of dense lymphocyte turf partially with lymph follicles, eosinophilic granulocytes, plasma cells and mast cells.

Therapy
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Excision (danger of recurrence), cryosurgery or laser therapy( dye laser). These are individual case reports. Due to the rareness of the disease, there is no greater experience available.

Literature
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Abrahamson TG et al (2003) Angiolymphoid hyperplasia with eosinophilia responsive to pulsed dye laser. J Am Acad Dermatol 49: S195-196
Asadi AK et al (2002) Angiolymphoid hyperplasia with eosinophilia. Dermatol Online J 8: 10
Becker-Weimann SY (2017) Angiolymphoid hyperplasia with eosinophilia. Nude Dermatol 44: 468-469
Kempf W et al (2002) Angiolymphoid hyperplasia with eosinophilia: evidence for a T-cell lymphoproliferative origin. Hum Pathol 33: 1023-1029
Tsuboi H et al (2001) Angiolymphoid hyperplasia with eosinophilia in the oral mucosa. Br J Dermatol 145: 365-366
Wells GC, Whimster IW (1969) Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 81: 1-14