Angiolymphoid hyperplasia with eosinophiliaL98.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 04.08.2021

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Synonym(s)

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

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HistoryThis section has been translated automatically.

Wells and Whimster, 1969

DefinitionThis section has been translated automatically.

Multicentric, benign lymphoproliferative hyperplasia, clinically characterized by dermal nodules and nodules, but also by subcutaneously located nodules with edema, lymphocytic and eosinophilic infiltrates, formation of germinal centers, fibrosis, and hyperplasia of blood vessels. Most authors separate Kimura's disease from angiolymphoid hyperplasia with eosinophilia on the basis of clinical and histological criteria. Obviously, overlaps of both syndromes are observed. However, the separate entity appears to be secure.

Occurrence/EpidemiologyThis section has been translated automatically.

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

EtiopathogenesisThis section has been translated automatically.

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

ManifestationThis section has been translated automatically.

Adults, mostly 20-50. LJ.

LocalizationThis section has been translated automatically.

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.

Clinical featuresThis section has been translated automatically.

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

Solitary or multiple, 2-40 mm, hemispherical, red, to reddish-brown, rarely purplish or ulcerated nodules and nodules. Rarely disseminated appearance or lymphadenopathy. Usually asymptomatic or painful, also pruritic (pruritus is absent in Kimura's disease!). In Kimura's disease, larger subcutaneous (also intramuscular) non-pruritic nodules are observed.

LaboratoryThis section has been translated automatically.

Eosinophilia possible in 20-30% of patients.

HistologyThis section has been translated automatically.

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.

Differential diagnosisThis section has been translated automatically.

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

TherapyThis section has been translated automatically.

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.

LiteratureThis section has been translated automatically.

  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 Ill.)
  8. Kurihara Y et al (2012) Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in zosteriform distribution. Indian J Dermatol 57:401-403.
  9. Mukherjee B et al (2015) Angiolymphoid hyperplasia with eosinophilia of the orbit and adnexa. Ocul Oncol Pathol 2:40-47.
  10. 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.
  11. Tsuboi H et al (2001) Angiolymphoid hyperplasia with eosinophilia in the oral mucosa. Br J Dermatol 145: 365-366
  12. Wells GC, Whimster IW (1969) Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 81: 1-14

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Last updated on: 04.08.2021