Angiolymphoid hyperplasia with eosinophilia L98.9

Wells and Whimster, 1969

Angiolymphoid hyperplasia with eosinophilia aka epithelioid hemangioma is a rare vascular proliferative neoplasm of unknown cause. It was first described by Wells and is characterized by solitary or multiple lesions that most commonly occur on the scalp, face and ear. Multiple lesions occur in about half of these patients, but are almost always grouped in the same body region. In addition to the vascular proliferations, an accompanying eosinophilic or lymphocytic component is usually present. The formation of germinal centers is also possible. Most authors distinguish between systemic Kimura's disease (lymphadenopathy, eosinophilia) and localized angiolymphoid hyperplasia with eosinophilia on the basis of clinical and histological criteria. Apparently, overlaps between the two clinical pictures are observed. However, the separate entity appears to be certain.

Panethnic among men and women of all ages. Unlike M. Kimura, no preference for the Asian population.

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

Adults, mostly 20-50. LJ.

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

In Kimura's disease, the scalp and neck region are preferentially affected.

Solitary, more rarely grouped, asymptomatic or painful, also itchy, red or brown-red, brown, firm, about 0.5-0.8 cm in size, papules and nodules with a smooth surface. Also occurring as deep nodules.

Single or multiple, 0.2-4.0 cm large, hemispherical, red to reddish-brownish, more rarely purple or ulcerated nodules and nodes. Rarely disseminated occurrence or lymphadenopathy. Usually asymptomatic or painful, also itchy (pruritus is absent in Kimura's disease!). In Kimura's disease, larger subcutaneous (also intramuscular) non-itching nodules are observed.

Eosinophilia possible in 20-30% of patients.

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.

Atheroma; epidermal cysts; cylindroma; hemangioma; granuloma teleangiectaticum; angiosarcoma; Kaposi's sarcoma; granuloma eosinophilicum faciei; lymphadenosis cutis benigna.

These are individual case reports. Larger experiences are not available due to the rarity of the disease.

Excision in case of longer duration. Risk of recurrence! Risk of bleeding!

CO2 laser may also be used.

Recurrence treatment in patients > 65 years, if necessary with X-rays (GD: 20-30 Gy). Combinations of CO2 laser irradiation with intralesional glucocorticoid application such as triamcinolone acetonide crystal suspension (e.g. Volon A diluted 1:3 with a local anaesthetic, e.g. Scandicaine) have also been described as successful.

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