Abstract
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, reactive vaso-proliferative condition in the dermal and subcutaneous tissues of the head and neck. A 28-year-old female presented with slow-growing painless swelling behind her left ear. FNAC revealed benign soft tissue neoplasm and histopathological examination after surgical excision revealed angiolymphoid hyperplasia with eosinophilia. ALHE origin has been variously attributed to prior trauma, hyperestrogenemia, infectious agents, atopy, reactive hyperplasia, and benign neoplasia. Retroauricular ALHE has been rarely reported. However, on the basis of our case report, it should be a viable differential diagnosis when large subcutaneous tumors of the head and neck are encountered. When big subcutaneous tumors of the head and neck are present, especially in females, a valid differential diagnosis for angiolymphoid hyperplasia with eosinophilia, a rare condition marked by dermal or subcutaneous endothelial cell proliferation, should also be considered.
Keywords: Angiolymphoid hyperplasia with eosinophilia, Epithelioid hemangioma, Kimura Disease, Retroauricular
Introduction
Angiolymphoid hyperplasia with eosinophilia (ALHE) also known as epithelioid hemangioma is a rare, benign, reactive vaso-proliferative condition that manifests as painless, vascular nodules in the dermal and subcutaneous tissues of the head and neck, especially around the ear [1]. It is distinguished by intradermal or subcutaneous reddish brown papules or nodules, which mostly appear in the head and neck region. It concerns mostly young female adults [2]. Histologically, it consists of a proliferation of vascular channels with lymphocyte, macrophage, eosinophil infiltration, and abnormally proliferating, plump endothelial cells often near muscular arteries [3]. Additionally, ALHE has been noted in the conjunctiva, lips, tongue, orbits, and scalp [4]. The most effective treatment for ALHE appears to be surgical excision. There may be effective therapy alternatives, including pulsed dye and other lasers. To improve ALHE treatment, additional research is required [5].
This report aims to present a case of an unusual condition with unusual presentation and to show how the histopathological findings change over time.
Case Report
A 28-year-old female presented with slow-growing painless swelling behind her left ear for one year. Her medical history was unremarkable. On clinical examination, an oval swelling of 3.5 cm×2 cm with a smooth surface and well-defined margin was present over the left postauricular region [Fig. 1]. It was firm in consistency, non-tender, and had normal overlying skin. No regional lymphadenopathy was observed. Routine blood counts were normal. Fine needle aspiration cytology (FNAC) revealed benign soft tissue neoplasm. Dissection and complete surgical excision under local anesthesia were done [Figs. 2, 3 and 4]. The gross appearance on the cut section shows a whitish area with hemorrhage inside it [Fig. 5]. The specimen was sent for histopathological examination. Postoperative recovery was excellent.
Fig. 1.
Clinical photograph of the left postauricular swelling
Fig. 2.
Intra-operative dissection of the left postauricular swelling
Fig. 3.
Intra-operative removal of the left postauricular swelling
Fig. 4.
Complete excision of the left postauricular swelling
Fig. 5.
Gross appearance of the left postauricular swelling
Histopathological examination after hematoxylin and eosin staining revealed an encapsulated mass and stroma with numerous small and large blood vessels with plump endothelial cells, lymphoid aggregates, eosinophils, and plasma cells consistent with angiolymphoid hyperplasia with eosinophilia [Fig. 6].
Fig. 6.
Microphotograph showing stroma with numerous small and large blood vessels with endothelial cells, lymphoid aggregates, and eosinophils
Discussion
ALHE origin has been variously attributed to prior trauma, hyperestrogenemia (e.g., pregnancy or oral contraceptive use), infectious agents, atopy, reactive hyperplasia, and benign neoplasia [5].
A group of individuals with subcutaneous lesions characterized by “unusual granulations combined with a hyperplastic change of the lymphatic tissue,“ which they called “eosinophilic lymphoid granuloma,“ were documented by Kimura et al. in 1948 [6]. Asian young men are susceptible to Kimura’s disease. With deeper localization, it can show as a single huge subcutaneous mass or several smaller ones. Usually, the lesions affect the salivary glands in the periauricular or submandibular area. Peripheral blood eosinophilia and increased IgE are frequently detected during laboratory workup. More so than ALHE, Kimura’s disease is linked to nephrotic syndrome. On histology, it is distinguished by hyperplastic lymphoid follicles with noticeable germinal centers and eosinophilic infiltrate. Vascular proliferative changes are seen, although they are not as prominent as in ALHE and do not involve the recognizable plump epithelioid endothelial cells [7, 8]. Therefore, a mesenchymal tumor of the muscles, nerves, or veins in the head and neck, such as neurilemmoma, leiomyoma, hemangioma, and fibrosarcoma, was the primary differential diagnosis [9].
The most effective treatment for ALHE is reportedly surgical excision. Higher rates of recurrence following excision were found in cases with ALHE with symptoms, multiple lesions, longer duration, earlier age of onset, and earlier age of onset. Although the lowest recorded recurrence rate for excision is 40.8%, this rate is still unsatisfactory and should encourage further research into surgical method advancements. The high likelihood of recurrence after routine excision may be related to the challenge of defining the margins of this highly vascular disease, despite the fact that ALHE tends to recur locally [5]. Other treatment options include pulsed-dye laser, carbon dioxide laser, argon laser, cryosurgery, curettage, electrodesiccation, and irradiation have been reported with some success [5]. Therefore due to the high recurrence rate long-term follow-up is necessary.
Retroauricular ALHE has been rarely reported. However, on the basis of our case report, it should be a viable differential diagnosis when large subcutaneous tumors of the head and neck are encountered.
Conclusion
When big subcutaneous tumors of the head and neck are present, especially in females a valid differential diagnosis for angiolymphoid hyperplasia with eosinophilia, a rare condition marked by dermal or subcutaneous endothelial cell proliferation, should also be considered.
Acknowledgement
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