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CASE REPORTS

Angiolymphoid Hyperplasia with Eosinophilia

Sangeeta B Kulkarni (Bhide), Ila M Vora

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Angiolymphoid hyperplasia with eosinophilia is a benign lesion in skin and subcutaneous tissue with a marked predilection for head and neck - particularly periauricular region. It shows well formed hypertrophic vascular structures lined by plump epithelioid appearing cells with abundant eosinophilic cytoplasm and variable lymphoid infiltrate with eosinophils. Despite their benign nature, considerable controversy still exists as to the basic nature of this lesion. Although rare, there are increasing number of reports of this conditions and should be considered as a separate entity from Kimura’s disease. A case of angiolymphoid hyperplasia with eosinophilia is reported and highlighted the differences from Kumura’s disease.

INTRODUCTION

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an unusual but distinctive vascular lesion that was first described by Wells and Whimster in 1969.1-4 Subsequently it was described by others by different terminologies, as inflammatory angiomatous nodule, atypical or pseudopyogenic granuloma and histiocytoid or epithelioid haemangioma. Angiolymphoid hyperplasia with eosinophilia is benign member of vascular proliferation having indolent nature.5 Although great progress has been made in understanding ALHE, controversy still exists regarding its reactive or neoplastic nature.2,5 Some authors considered ALHE as a vascular tumour of benign nature.2,6,7 Whereas, some considered it as just inflammatory reactive process.3,5 A case of ALHE is reported from glabellar region highlighting the differences from Kimura’s disease.8

CASE REPORT

A 30 year old male presented with a complaint of swelling over right side of glabella since 6 months. Clinical examination did not reveal any other abnormality. Peripheral blood count was normal with eosinophils within normal limits. Clinical diagnosis was sebaceous cyst. The swelling was excised and sent for histopathological examination. Gross examination revealed well-encapsulated globular mass measuring 2 cm in diameter which was firm in consistency and cut surface was homogeneous grey-white. Histopathology showed a circumscribed lesion in the dermis composed of proliferated vessels with a vague lobular arrangement. There were clustering of small capillary vessels around a medium sized parent vessel. These vessels were lined by swollen endothelial cells resembling histiocytoid or epithelioid appearance with scalloped borders and protruding deeply into the lumen giving “Tombstone” or “Hobnail” appearance (Fig. 1). These epithelioid endothelial cells had rounded to lobated nuclei and abundant eosinophilic cytoplasm containing occasional vacuoles. Stroma showed variable amount of infiammatory cell infiltration predominantly of lymphocytes and eosinophils. At places lymphoid aggregates were seen. A diagnosis of ALHE was made.

DISCUSSION

ALHE represents the benign member of a family of vascular proliferations, having as a common denominator the presence of epithelioid endothelial cells, which include epithelioid haemangioendothelioma and epithelioid angiosarcoma.2,3,5 A distinction among these entities is possible in majority of cases on the basis of location, cellularity, atypia and some architectural features.3,5 Differentiation of ALHE from Kimura’s disease is difficult, as both have proliferated capillaries and an extensive cellular infiltrate of lymphocytes, histiocytes and eosinophils. For longer time these two conditions were thought to represent one and the same pathology but the current consensus is, these two conditions should be considered as separate entities.1,2 ALHE is believed to be a disease of Western world and Kimura’s disease of far East and Asia.2,9-11 ALHE occurs during early to middle adult life as in this case. Age group for Kimura’s disease is 15-57 years with mean age of 40 year.1,6,11 Females are commonly affected in ALHE whereas males in Kimura’s disease.1,2,9,11 Bombay Hospital Journal, Vol. 45, No. 4, 2003 639 Commonest location for ALHE is head and face.1,2,5,6 In this case site was glabella. Kimura’s disease presents as subcutaneous soft tissue mass in head and neck region especially in periauricular area. Parotid gland is also involved.1,5,9,11 The duration of lesion in ALHE is shorter as compared to longer duration upto 23 years in Kimura’s disease.1 The ALHE patients usually present with pruritic red patch or solitary to multiple nodular lesions which are less than 2 cm in diameter.1-3,6 While the patients of Kimura’s disease present with insidious onset of tumour like nodules which may sometime attain a very large size.1,11 In ALHE lymphadenopathy and peripheral eosinophilia are rarely seen.1,2,6 Our patient neither had lymphadenopathy nor peripheral eosinophilia. Peripheral eosinophilia and regional lymphadenopathy is nearly always present in Kimura’s disease.1,2,5,11 In ALHE histologically there is vascular and cellular component.1 Vascular component shows vague lobular arrangement as a result of clustering of small capillary sized vessels around a medium sized parent vessel.1,2 There are irregular hypertrophied vascular structures lined by greatly swollen pleomorphic endothelial cells.1-3,11 They protrude into the lumen giving “Tomstone” or “Hobnail” appearance.2,3,6,11 They may show cytoplasmic vacuoles.1,2,6,10 The cellular component consists of an extensive infiltrate of lymphocytes, histiocytes and eosinophils.1-3,5 Although eosinophils are prominent in the infiltrate, they may be few in number or even absent.1,3,5

Our case also showed similar features. The important histopathological differences between ALHE and Kimura’s disease are shown in Table 1. Treatment of choice in ALHE and localised Kimura’s disease in surgical excision although some authors recommend conservative management in Kimura’s disease in the form of intralesional steroids or radiation therapy.2,12 In summary, a case of ALHE is reported from glabellar region and the differences are highlighted from Kimura’s disease.

REFERENCES

1. Urabe A, Tsuneyoshi M, Enjoji M. Epithelioid Hemangioma versus Kimura’s disease : A comparative clinicopathologic study. Am J Surg Pathol 1987; 11 : 758-66. 2. Enzinger FM, Weiss SW. Benign tumours and tumorlike lesions of blood vessels. In Enzinger FM, Weiss SW editors. Soft tissue tumours. 3rd edn., St. Louis: Mosby - yearbook, Inc., 1995 : 579-622. 3. Lever WF, Lever SG. Tumours of vascular tissue. In: Lever WF, Lever SG editors. Histopathology of the skin 6th edn, Philadelphia : JB Lippincott Company, 1983 : 623-47. 4. Reed JR, Terazakis N. Subcutaneous angioblastic lymphoid hyperplasia with eosinophilia (Kimura’s disease). Cancer 1972; 29 : 489-97. 5. Rosai J. Tumour and tumour like conditions. In: Rosai J, editor. Ackerman’s surgical pathology. 8th edn., St. Louis : Mosby-year book, Inc., 1996; 1: 63-197. 6. Fletcher CD, McKnee PH. Vascular tumours. In: McGee J, Isaacson PG, Wright NA, editors Oxford Textbook of Pathology. 1st edn, New York : Oxford University Press, 1992; 2a : 931-9. 7. Miettinen M, Weiss SW. Soft tissue tumours. In: Damjanov L, Linder J, editors Anderson’s Pathology. 10th edn. St. Louis : Mosby-year book, Inc., 1996; 2 : 2480-2520. 8. Kimura’s T, Yoshimura S, Ishikawa E. Abnormal granuloma with proliferation of lymphoid tissue. Trans Soc Pathol Jpn 1948; 37 : 179-80. (In Japanese) Quoted from reference no. 1. 9. Jambhekar NA, Bores AM, Saxena R, Parikh D, Soman C. Angiolymphoid hyperplasia with eosinophilia (Kimura’s disease) : Report of large sized lesion. J Surg Oncol 1991; 47 : 206-8. 10. Santacruz DJ, Hurt MA. Nonmelanocytic cutaneous tumours. In: Sternberg SS, Antonioli DA, Corter D, Mills SE, Oberman AH editors. Diagnostic Surgical Pathology 3rd edn., Philadelphia : Lippincott, Williams and Wilkins, 1991; 1 : 49-87. 11. Hui PK, Chan JK, Ng CS, Kung IT, Gwi E. Lymphadenopathy of Kimura’s disease. Am J Surg Pathol 1989; 13 : 177-86.

WHO NEEDS A DEFIBRILLATOR AFTER MYOCARDIAL INFARCTIONfi Defibrillator implantation for every heart-attack survivor with an impaired left ventricle may soon be recommended. The MADIT-II study (Multicenter Automated Defibrillator Implantation Trial II) recruited patients who had had myocardial infarction more than a month previously and who had left-ventricular ejection fraction of 30% or less, for randomisation to an implantable defibrillator or no device. MADIT-II found a dramatic 34% reduction in hazard of all-cause mortality overall. The follow-up averaged only 20 months, so only a modest reduction in absolute mortality of 5.6% (from 19.8% to 14.2%) was recorded at study end. The Lancet, 2003; 362 : 91.

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