Dilemma in diagnosing a post-auricular mass-angiolymphoid hyperplasia with eosinophilia versus Kimura’s disease, an infrequent encounter

INTRODUCTION

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular tumor and Kimura’s disease is a chronic inflammatory disease. Both not only have uncertainty in etiology and pathogenesis, but also there is therapeutic dilemma in management. Fewer than 1000 cases of ALHE and 300 cases of Kimura’s disease has been reported so far.^1,2 In the olden days, both were thought to be two different presentations of the same disease. Although they share overlapping features, with distinctive clinical, epidemiological and histopathological features which were declared currently, both are considered as separate entities. In this article, we describe a typical clinical case of ALHE which posed a riddle in coming to correct diagnosis from its main differential diagnosis, Kimura’s disease.

CASE REPORT

An otherwise normal 39 year old male person presented to our ENT Outpatient department with swelling in the left post-auricular region which appeared 1 and half year before and gradually progressed to its present size. He also had pain over the swelling for past 2 months. There was no history of fever, sore throat, cough, ear discharge, weight loss or trauma. There is no relevant family history.

On examination, a single smooth spherical swelling of size 3×3 cm with well-defined edges and normal skin was noted in upper part of post-auricular region (Figure 1). On palpation, it was firm, mobile, non-tender, non-pulsatile, non-fluctuant with no trans-illumination.

Routine blood investigations were done which revealed rise in absolute eosinophil count (550 cells/cu.mm). Serology for HIV, HBsAg and HCV were non-reactive. We further subjected the patient for FNAC followed by HRCT. FNAC had scantly cellular and occasionally scattered fibroblasts, pinkish stromal collagen fragments with inflammatory cells in background of hemorrhage suggestive of soft tissue neoplasm. HRCT temporal bone showed a well-defined rounded hypo-dense lesion measuring 28×23 cm with plain CT value of 37 HU noted in the post-auricular region of left pinna causing anterior displacement of pinna with no effect of adjacent bony erosion / calcifications/ cystic changes suggestive of benign etiology (Figure 2).

Suspecting it to be schwannoma or dermoid cyst, an excision biopsy was planned. On dissection, a firm smooth, bi-lobed well encapsulated swelling was noted and excised along with post-auricular lymphnode. (Figure 3)

Histopathology showed florid proliferation of blood vessels lined by plump endothelial cells having vesicular nucleus. The blood vessels were surrounded by fibrous tissue with dense infiltration by lymphocytes and eosinophils with scattered lymphoid aggregates (Figure 4a, 4b). These features are suggestive of angiolymphoid hyperplasia with eosinophilia.

In consideration of eosinophilia, a special investigation of serum IgE levels was sent to rule out Kimura’s disease which was found to be within normal limits. Currently, the patient is doing well with no recurrence observed during his 6 months follow up.

DISCUSSION

Both ALHE and Kimura’s are uncommon chronic benign lesions of the skin mainly involving the head and neck regions. ALHE involves vascular and lymphocytic proliferation whereas Kimura’s is of inflammatory type disorder. Dermal involvement is more in ALHE but In Kimura’s disease the lymphnodes and subcutaneous tissues are more commonly involved.

In 1969, Wells and Whimster, first derived the term, Angiolymphoid hyperplasia with eosinophilia who described it as distinguished neoplasm.³ Later, In 1982, Weiss and Enzinger evidently distinguished the lesion from malignant vascular tumour, epithelioid hemangioendothelioma, and put forth the term epithelioid hemangioma (EH).⁴ In 1937, Kimura’s disease was first described and later popularized in 1947 by Kimura and his associates.⁵

ALHE commonly seen in young and middle aged men and women of Asian origin followed by Caucasians.^6,7 It typically involves the peri-auricular region, face and scalp, rarely trunk, extremities, hands, penis, oral mucosa, colon are affected.⁸ On the other hand, Kimuras is more prevalent in young Asian lineage , mostly in their third decade of life.^9,10 Kimura’s disease occurs mostly in infra-auricular and retro-auricular region and rarely, orbit, eyelid, palate, pharynx were also involved.^9,11,12 There is no sex predominance in ALHE where as in Kimura’s disease, male predominance is seen.^8,13

The etiopathogenesis of both diseases are currently unknown. Several theories have been proposed for ALH, including a reactive process, neoplastic process and a infective mechanism with possible association with HIV.¹⁴ The association of monoclonal T-cell process is under debate.¹⁵ Peripheral T-cell lymphoma case has also been reported in a case of ALHE. Associated T-cell receptor (TCR gene) rearrangement and monoclonality has been detected.¹⁴ While in Kimura’s disease, it has been put forward that a common trigger factor such as viral, parasitic infection or toxin could cause alteration of T-cell immunoregulation or induce an IgE mediated hypersensitivity resulting in release of eosiniphiliatrophic cytokines. The role of cytokines, such as interleukin (IL) 4, IL-5, IL-13 has been documented, which may precipitate high IgE levels and marked eosinophilia in this disease.⁹

Clinically, ALHE presents as solitary or multiple red or brown papules or nodules, measuring upto 3 cms, producing symptoms such as pruritis, bleeding and pain. Regional lymphadenopathy is rarely reported. Prolonged duration of lesion tend to produce more symptoms. Systemic manifestations are uncommon.¹⁶ On the flip side, Kimura’s disease is predominantly unilateral and presents as subcutaneous nodules with occasional pruritis, eczema and rashes. It is associated with regional lymphadenopathy and may or may not involve salivary glands.¹⁷ It is characterized by triad of painless subcutaneous mass, blood and tissue eosiniphilia and elevated serum IgE levels. Almost 60% of persons have renal involvement, usually manifesting as membranous glomerulonephritis and nephritic syndrome.^12,18 Our case presented as solitary smooth spherical mass measuring 2.8×2.3 cm with pain over the swelling and elevated eosinophilia which cause dilemma in reaching the diagnosis.

The lesions can also be investigated additionally with USG, CT scan and MRI. But radiological features are not pathognomonic. The diagnosis is made only by histopathological examination of excision biopsy specimen.

Histopathology of ALHE demonstrates abnormal vascular proliferation of varying sizes and types is present, and the vessels are lined by plump histiocytoid or epithelioid endothelial cells. There are perivascular and interstitial inflammatory infiltrates with numerous eosinophils (5-15% of the infiltrate), lymphocytes with occasional lymphoid follicle formation.¹⁹ In Kimura’s disease, there is florid lymphoid follicles with germinal center formation, eosinophilic infiltrates, micro-abcesses and folliculolysis. In contrast to Kimura’s disease, the histiocytoid or epithelioid cells are characteristic feature of the ALHA.¹⁴

Both diseases follow a benign clinical course with no metastases reported so far. But there is high chance of recurrence. Spontaneous remission has also been reported. There is no consensus in the treatment modality of both diseases. Various methods of management have been tried with variable success rate such as intralesional and topical corticosteroids, pulse-dye laser, radiotherapy, cryotherapy, systemic and topical antibiotics, oral isotretinoin, oral pentoxifylline. The treatment of choice in both cases is surgical excision of the lesion. But there is high chance of local recurrence with recurrence rate of 40% in ALHE and 33-50% in Kimura’s disease.^20,21

CONCLUSION

Though the diagnosis is difficult, it is important to distinguish the lesion from malignant neoplasms like epithelioid hemangioendothelioma, kaposis sarcoma. It helps us avoiding unnecessary radical surgery being done. As the disease is of rare phenomenon and of inconclusive etiopathogenesis, there is therapeutic inconsensus in management. Hence, more studies have to be done and a standardized treatment protocol has to be established.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

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