Angiolymphoid hyperplasia with eosinophilia and Kimura disease overlap, with evidence of diffuse visceral involvement

Abstract

A relationship between Kimura disease (KD) and angiolymphoid hyperplasia with eosinophilia (ALHE) has been debated. Given substantial clinical and histological overlap, these entities were once considered to represent a disease spectrum; however, they are now widely considered to be nosologically distinct. A diagnosis of either condition is further complicated by resemblance to various malignancies, which must be carefully excluded. Coexistence of ALHE and KD in a patient is extremely rare, with only four cases reported in the English literature. We report what is to our knowledge the first case of ALHE and KD overlap with evidence of diffuse visceral involvement.

Case Report: Hyper IgE, but Not the Usual Suspects–Kimura Disease in an Adolescent Female

“…ALHE shares sufficient clinical and histopathologic features with KD that investigators in the past wondered if these entities were part of the same disease spectrum, although they are now considered by most to be distinct diseases ( 18 , 25 ). While the lesions of ALHE are also generally located in the head and neck region, they tend to be smaller and are more often found in multiples ( 18 ). ALHE is also more common in females and the lesions are often described as discolored and pruritic ( 18 ).…”

“…While the lesions of ALHE are also generally located in the head and neck region, they tend to be smaller and are more often found in multiples ( 18 ). ALHE is also more common in females and the lesions are often described as discolored and pruritic ( 18 ). Although the peak incidence of ALHE is in the second to fourth decade of life, ALHE has been described in all age groups ( 25 ).…”

“…Although the peak incidence of ALHE is in the second to fourth decade of life, ALHE has been described in all age groups (25). Unlike KD, ALHE is not increased in Asian patients (18). While both ALHE and KD demonstrate vascular proliferation on histology, the vessels in ALHE are abnormal with characteristically plump, histiocytoid endothelial cells with cytoplasmic vacuoles (18,25).…”

“…Disease comparison(18)(19)(20). Kimura Disease; ALHE, angiolymphoid hyperplasia with eosinophilia; IgG4-RD, Immunoglobulin G4-related disease; L-HES, lymphocytic hypereosinophilic syndrome.…”

A fleshy papule on the eyelid

“…It is a friable vascular lesion and may be associated with bleeding on manipulation [7]. Kimura disease, which was previously believed to be a variant of AHLE owing to clinical and histological resemblance, has now been established as a separate entity, although there are a few rare instances of AHLE co-existing with Kimura disease [8]. Clinically, Kimura disease is characterized by a deep seated large swelling often associated with lymphadenopathy and without any surface skin changes.…”