Angiolymphoid hyperplasia with eosinophilia (ALHE)
Synonyms: Epithelioid hemangioma and Histiocytoid hemangioma
* A non-malignant, locally proliferating lesion composed of channels of small blood vessels surrounded by lymphocytes and eosinophils, hence the name ALHE.
* Reported from different parts in the world but particularly common in Japan.
* The cause is unknown, but antigenic stimulation following insect bites has been postulated. It sometimes follows an injury (trauma).
* Age: commonly young adults, with mean age in mid-thirties.
* Affects both sex but there is a female preponderance.
* Clustered small, translucent, brown, pink or red, dome-shaped dermal papules and nodules.
* Grouped lesions merge to form plaques or grape-like clusters.
* The lesions may be asymptomatic, itchy or painful.
* The most common site is the scalp especially around the ear (retroauricular area) or the hairline, however, lesions may also involve the inside of the mouth, trunk, extremities or genitals (penis and vulva).
* Kimura’s disease is thought to be a distinct entity from ALHE. For differentiation:
1. The lesions of Kimura’s disease are deeper-seated and larger (massive subcutaneous swelling).
2. Kimura’s disease is associated with allergic conditions such as asthma, rhinitis, and eczema, and it is frequently accompanied by lymphadenopathy, peripheral blood eosinophilia, and elevated IgE level. Peripheral blood eosinophilia is more common in Kimura disease than in ALHE.
3. Skin biopsy:
ALHE: A central thick-walled vessels with hobnail endothelium. A cellular infiltrate composed mainly of lymphocytes and large numbers of eosinophils is seen around the blood vessels.
Kimura’s disease: Prominent germinal centers with eosinophils are present in the subcutaneous tissue.
* There is controversy about spontaneous regression of the lesions of ALHE!
* The response to active treatment is variable and not always successful.
* In ALHE, an underlying AV shunt is present as a result of damage to and repair of an artery or vein. The lesions may recur if this underlying AV shunt is not excised, that is why treatment with surgical excision is successful in 65% of cases. Mohs micrographic surgery, including excision of the abnormal vessels at the base of the lesion, may be more effective. There tends to be a lot of bleeding during surgery.
2. Intralesional corticosteroids.
3. Lasers: Pulsed dye, Nd:YAG, or CO2 lasers.
5. Success in some patients had been reported with: cryotherapy, electrodessication. pentoxifylline, indomethacin, imiquimod, IFN alfa-2b, isotretinoin, vinblastine, and intralesional bleomycin.