Angiolymphoid hyperplasia with eosinophilia L98.9

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.

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.

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.

1. Abrahamson TG et al. (2003) Angiolymphoid hyperplasia with eosinophilia responsive to pulsed dye laser. J Am Acad Dermatol 49: S195-196
2. Asadi AK et al. (2002) Angiolymphoid hyperplasia with eosinophilia. Dermatol Online J 8: 10
3. Bangal S et al. (2011) Orbital extension of supraorbital angiolymphoid hyperplasia with eosinophilia. Australas Med J 4:111-113.
4. Becker-Weimann SY (2017) Angiolymphoid hyperplasia with eosinophilia. Act Dermatol 44: 468-469
5. Hussain JA et al. (2018) Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the external auditory canal, an unusual presentation in an adult female: a case report. J Surg Case Rep 2018(7):rjy145.
6. Kempf W et al. (2002) Angiolymphoid hyperplasia with eosinophilia: evidence for a T-cell lymphoproliferative origin. Hum Pathol 33: 1023-1029
7. Kim SM et al. (2010) Angiolymphoid hyperplasia with eosinophilia on the palm. Ann Dermatol 22:358-361
8. Kim G et al. (2023) Multiple Eruptive Epithelioid Hemangiomas on the Right Upper Extremity in an Asian Man. Ann Dermatol 35(Suppl 2): 252-S255.
9. Kurihara Y et al. (2012) Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in zosteriform distribution. Indian J Dermatol 57:401-403.
10. Mukherjee B et al. (2015) Angiolymphoid Hyperplasia with Eosinophilia of the Orbit and Adnexa. Ocul Oncol Pathol 2:40-47.
11. Santosa C et al. (2018) Angiolymphoid hyperplasia with eosinophilia with clinical pictures of keratoacanthoma: A rare case report. Clin Case Rep. 2018 Dec 4;7(1):189-192.
12. Tsuboi H et al. (2001) Angiolymphoid hyperplasia with eosinophilia in the oral mucosa. Br J Dermatol 145: 365-366
13. Wells GC, Whimster IW (1969) Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 81: 1-14