Answer 1

Angiolymphoid hyperplasia with eosinophilia was first described by Wells and Whimster in 1969 as a late stage of Kimura's disease.^[1] It is now generally accepted that these two are separate entity.^[2]

Angiolymphoid hyperplasia with eosinophilia (ALHE) typically presents as papules or nodules tan-brown, pink or dull-red color, located predominantly in the head and neck region, especially around the ears, forehead and scalp, generally grouped in the same area. ALHE can be asymptomatic or it can be painful, pruritic, or pulsatile.^[3]

Histopathologically ALHE characterized by capillary proliferation with lumenlined with swollen plump protruding endothelial cells. These cells develop epithelioid or histiocytoid morphology, are the principal characteristic of ALHE. An interstitial or perivascular inflammatory infiltrate consisting of lymphocytes, mastocytes, and eosinophils are seen.^[4]

The local tissue eosinophilia is associated with peripheral blood eosinophilia has been reported in approximately 20% of the patients.^[5]

The lesions were treated with topical steroid for 4 weeks without any improvement, the patient lost for follow-up.

Answer 2

The etiology and pathogenesis of ALHE are unclear, but considered to be a reactive phenomenon. History of trauma may be elicited in some cases.^[3] Owing to tissue and peripheral eosinophilia and elevated IgE levels, a hypersensitive reaction to an unknown stimulus has been implicated. The lymphoid infiltrate is a prominent component of the lesion and few studies have demonstrated T-Cell Receptor (TCR)-gene rearrangement on the lymphoid cells supporting the monoclonal nature of the infiltrate.^[6],[7]

Answer 3

The important differential diagnosis is Kimura's disease, which affects younger patients, present as deep painless subcutaneous masses covered by normal skin located on the head and neck region, ranging from 2 to 10 cm in size and associated with lymphadenopathy, sometimes affects the salivary glands. Histologically there is a predominance of lymphoid follicles with well formed germinative centre and a high percentage of eosinophils that occasionally form abscesses. The eosinophilia and high IgE levels are characteristic features of Kimura's disease, in ALHE these changes are generally absent.^[8]

Other conditions clinically mimicks ALHE are lymphoma cutis, pyogenic granuloma, Kaposi's sarcoma, granuloma faciale, cavernous hemangioma, and angiomatous lymphoid hamartoma.

Histologically angiosarcoma because of its vascular proliferation associated with lymphocytic infiltration mimicks ALHE, but the presence of eosinophils and absence of nuclear atypia in ALHE distinguishes it from angiosarcoma.^[9] Retiform haemangioendothelioma having typical “hobnail” pattern of endothelial cells with tall narrow and protrude inside the lumen, instead of being plump and swollen occluding the lumen as in ALHE.^[10]

Answer 4

Although ALHE is considered a benign disease, it can be resistant to multiple therapies. Various therapeutic approaches are proposed including surgery, electrodessication, cryosurgery, laser ablation, intralesional triamcinolone, and radiotherapy with variable results. Topical imiquimod has been reported effective.^[11] Spontaneous regression has also been reported, ALHE may be observed for several months before it is treated aggressively.^[12]

Though ALHE is not a very rare condition, a high index of suspicion is required to diagnose and differentiate it from other common conditions and for its proper management.