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ALHE is currently considered to be a benign vascular proliferation. It is more common than Kimura disease and affects subjects between the third and fourth decades of life with no predilection for any ethnic group or sex. Its occurrence after a trauma or during pregnancy is often reported. Clinically, it is characterized by erythematous or deep purple papules or nodules that are pseudo-angiomatous, sometimes crusted, or associated with bleeding and pruritus (15%). Subcutaneous nodules are rare and always small. There may be a solitary lesion or a multilobulated lesion with a “grapevine” pattern. The most commonly affected areas are the head (forehead, temples, ears) (50%) and the neck. Other sites (trunk, extremities: hands, palms) are infrequent . Extracutaneous involvement is also rare: localised or regional unspecific lymphadenopathy is present in 20% of cases. The presence of vascular malformations (arteriovenous shunts) underlying or close to the lesions is quite common and must be searched [2, 3]. There is no renal involvement. Hypereosinophilia is present in only 20% of cases and immunoglobulin E level is normal. Histologically, there is a proliferation of blood vessels in the superficial and mid-dermis, typically with turgescent endothelial cells with intracytoplasmic vacuoles referred to as “histiocytoid” or “epithelioid” with a “hobnail” shape, within a polymorphous infiltrate made up of lymphocytes, histiocytes, and eosinophils with no true lymphoid follicle formation.
Spontaneous regression is rare but possible. Treatment consists mainly of surgery or laser therapy. Other anecdotal treatments have been reported.
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