Angiolymphoid hyperplasia with eosinophilia is a rare, benign vascular tumor affecting principally the head and neck region of young adult females. Microscopic analysis reveals hyperplastic blood vessels lined by a hypertrophic endothelium. An inflammatory infiltrate rich in eosinophils is also present. Etiology of the lesion is unknown. Various treatment modalities have been described. We present a case successfully treated by excision and local steroid infiltration.
Angiolymphoid hyperplasia with eosinophilia (ALHE, epithelioid haemangioma, inflammatory angiomatous nodule, atypical granuloma, pseudopyogenic granuloma, and histiocytoid hemangioma) is an uncommon, benign, reactive vaso-proliferative disease, presenting with painless, vascular nodules in the dermal and subcutaneous tissues of the head and neck, particularly around the ear [
ALHE is characterized clinically by single to multiple red brown dome shaped papules or subcutaneous nodules [1-4]. About 1/5 of patients have blood eosinophilia and Lymphadenopathy [
An eighteen-year-old Hindu female attended our outpatient department with complaint of a gradually increasing painless swelling in the left frontal region extending to the eyebrow of one year’s duration (
Routine blood and urine investigations were within normal limit. Chest screening showed no abnormality. Complete surgical excision of the mass was performed under general anaesthesia by maintaining the aesthetic aspect of the surgery (
The size of the tumour was 2.5 cm × 2 cm × 1 cm. The external surface was smooth. On cut surface, the tumour showed fine whitish capsule surrounding pale pink areas. Microscopy revealed lobular proliferation of thick and thin walled blood vessels lined by plump endothelial cells and separated by an inflamed fibrocollagenous stroma. The inflammation comprises of lymphocytes, neutrophils and many eosinophils. Few lymphoid follicles are also seen (Figures 3 and 4).
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare condition affecting muscular arteries, typically of the head and neck [
Histologically the lesions are characterized by a reactive proliferation of small blood vessels, often surrounding a muscular artery, with peripheral inflammatory infiltrates consisting of mononuclear cells and eosinophils. The reactive blood vessels are often epithelioid, leading to the terms “histiocytoid” or, more recently “epithelioid” haemangioma [
The pathogenesis of ALHE remains unclear. Some authors consider ALHE as a neoplasm developing from
endothelial cells; others suggest that it is secondary to an inflammatory vascular reaction secondary to complex immunologic mechanisms. Many other hypotheses have been reported implicating environmental factors such as insect bite, trauma, and infections. Some authors consider that arterio-venous shunt is the main etiopathogenetic mechanism observed in 42 percent of the cases [7,16]. The predominance of T lymphocytes and a rearrangement of TCR receptor in some cases made some authors suppose that ALHE is a low-grade neoplastic disease secondary to various stimuli [7,16].
Serum hypereosinophilia is inconstant (21%) and is not required to make the diagnosis. Given that some patients with ALHE have also been found to have renal disease, urinalysis could be considered. Radiologic examinations such as MRI or angiography may be required to determine the extension of the lesions [7,16]. Positive diagnosis is based upon histological findings. Other differential diagnosis based on clinical and/or histopathologic findings, includes insect bites, angiomatous neoplasias such as capillary hemangioma, granuloma pyogenicum with satellite lesions, angiosarcoma of the face and scalp, and Kaposi sarcoma. Furthermore, epidermal cysts, lymphadenosis cutis benigna, and granuloma faciale might mimic angiolymphoid hyperplasia with eosinophilia.
In the absence of treatment, lesions may either increase progressively or decrease spontaneously [
Interleukin-5 based treatment represents an interesting approach. This cytokine interferes with the production and activation of eosinophils, which are supposed to play a key role in the pathogenesis of ALHE. Imiquimod, which inhibits the production of interleukin 5, and mepolizumab, which inhibits the reaction of IL5 with its receptor, were reported to be effective [
Angiolymphoid hyperplasia with eosinophilia is a rare condition with a challenging diagnosis and treatment. In spite of the benignity of this disease, it causes a therapeutic dilemma because of the cosmetic defects and frequent resistance to treatment.