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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
CASE REPORT Table of Contents   
Year : 2005  |  Volume : 9  |  Issue : 1  |  Page : 24-26
 

Angiolymphoid hyperplasia with eosinophilia (ALHE)


1 Department of Oral Pathlogy and Microbiology, Govt. Dental College, Trivandrum, India
2 Department of Pathology, Govt. Medical College, Trivandrum, India

Correspondence Address:
R Rajendran
Department of Oral Pathlogy and Microbiology, Govt. Dental College, Trivandrum
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-029X.39056

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   Abstract 

A 16-year old girl reported with a dome shaped, smooth-surfaced subcutaneous nodule in the left submandibular area of 8 months duration. Absence of relevant positive findings lead to the provisional diagnosis of benign submandibular gland neoplasm or reactive lymphadenitis of neck. As excision biopsy was performed of the lesion and the wound primarily closed. The surgical section revealed a mixed inflammatory dermal infiltrate with lymphocytes and numerous eosinophils and proliferation of blood vessels with plump endothelial cells protruding into the lumen. The overlying epidermis was hyperplastic. A histologic diagnosis of ALHE was made and the patient was managed surgically. She underwent an uneventful recovery. A follow up of 6 months post-operatively failed to notice any recurrence.



How to cite this article:
Rajendran R, Padmakumar S K, Kothawar S, Balaraman NM. Angiolymphoid hyperplasia with eosinophilia (ALHE). J Oral Maxillofac Pathol 2005;9:24-6

How to cite this URL:
Rajendran R, Padmakumar S K, Kothawar S, Balaraman NM. Angiolymphoid hyperplasia with eosinophilia (ALHE). J Oral Maxillofac Pathol [serial online] 2005 [cited 2019 Jul 23];9:24-6. Available from: http://www.jomfp.in/text.asp?2005/9/1/24/39056



   Introduction Top


Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncomrnon idiopathic condition that presents with isolated or grouped plaques or nodules in the skin of the head and neck. Most patients present with lesions in the periauricular region, forehead or scalp, and on the neck. Young to middle-age females are predominantly affected. Pruritus is the most common symptom. Spontaneous bleeding and pulsation over the lesion have been reported. The nodules are intradermal but can occasionally be subcutaneous. They usually measure less than 1 cm in diameter but may be as large as 10 cm. Peripheral eosinophilia and elevated levels of scrum IgE occasionally occur [1] .

ALHE typically appears as dome shaped, smooth - surfaced papules or nodules. Approximately 85% of the lesions occur in the skin of the head and the neck; most of them are on or near the ear or on the forehead or scalp. The lesions range from erythematous to brown and may be eroded or crusted. The cause of ALHE is idiopathic, whether the condition is neoplastic or reactive state is uncertain; a reactive cause is favoured. An arterio-venous shunt has been demonstrated deep to the dermal lesions in some specimens [2] .

Owing to the associated eosinophilia and the elevated lgE levels, the pathogenesis of ALHE is presumed by some authors to be hypersensitive response; however, the stimulus is unknown. A prior traumatic insult was reported in only 9% of 116 patients with ALHE [3] .

The differential diagnosis of ALHE includes both benign and malignant vascular neoplasms of the skin, as well as various reactive states dominated by lymphocytes and eosinophils, including angiosarcoma, pyogenic granuloma, venous lakes, angiomatous lymphoid hamartoma, eosinophilic granuloma, granuloma faciale, and pseudolymphoma. However, the clinical picture, combined with the characteristic microscopic findings of vascular proliferation with hobnailed endothelial appearance and lymphocytic infiltration with eosinophils, should help to exclude the other conditions.

A case of ALHE with involvement of the left submandibular region in a young female of 16 years is presented here.


   Case Report Top


A 16-year-old female was reported to the Department of Oral Pathology and Microbiology for evaluation of a neck mass of 8 months duration, painless and slowly increased in size to the present 1 x 1 sq. cm. It was a firm swelling in the left submandibular region anterior to the masseteric insertion. It appeared as a dome-shaped, smooth surfaced, subcutaneous nodule without surface erosion and / or encrustation. The lesion was an isolated one and non pulsatile. The teeth in the neighbouring quadrant tested positive for vitality and no clinical caries and/ or periodontal decay noticed. Symptoms for Wharton's duct obstruction were negative and gave either evidence for submandibular sialadenitis. The patient showed a negative tuberculin test and the chest radiographs were nom-contributory. The hemogram values were all within normal limits except mild eosinophilia (count 17 cells/ cu.mm).

A provisional clinical diagnosis of benign submandibular gland neoplasm or reactive submandibular lymphadenitis was made. The patient underwent an excision biopsy of the lesion and the wound closed primarily. She had an uneventful recovery.

Macroscopy

The surgical Specimen consisted of a firm mass. 1 x 1 sq. cm, grayish brown with evidence of encapsulation by condensation of peripheral connective tissue which was deficient in entirety. The cut surface was moderately compact with areas of cavitation and hemorrhage. No calcified masses were noticed in gross section.

Histologic findings

The section showed characteristic histologic features, including a proliferation of small blood vessels, many of which are lined by enlarged endothelial cells with uniform ovoid nuclei and intracytoplasmic vacuoles. The endothelial cells were distinctive and had a cobblestone appearance. In addition, a perivascular and interstitial infiltrate composed primarily of lymphocytes and cosinophils was present. Eosinophils typically comprised 5%- 15% of the infiltrate. Lymphoid aggregates with and without follicle formation were noticed at different fields.

The histologic impression was compatible with Angiolymphoid hyperplasia with tissue eosinophilia (ALHE).


   Discussion Top


A definite pathologic entity ALHE is marked by proliferation of blood vessels with distinctive large endothelial cells. These blood vessels are accompanied by a characteristic inflammatory infiltrate that includes eosinophils. The lesion is benign but may be persistent and difficult to eradicate. Whether ALHE represents a benign neoplasm or an unusual reaction to varied stimuli, including trauma, remains unclear.

While ALHE shows some similarity to Kimura's disease, it is a distinct condition. In ALHE, there is abundant angiomatoid proliferation, with uncanalized masses of endothelial cells, in contrast to the capillary proliferation seen in kimura's disease [4] . The features that ALHE and kimura's disease seem to have in common are lymphoid follicles with eosinophils, occasional elevation in serum IgE levels, and peripheral eosinophilia.

Pathophysiology

Although ALHE may be a benign tumour, numerous factors suggest that it is an unusual reactive process. The condition may be multifocal. ALHE has occurred following various forms of trauma or infection. Histologically, most cases of ALHE show damaged and or tortuous arteries and veins at the base of the lesion suggesting that arterio-venous shunting may play a role in the pathogenesis. Hyperestrogenemic states (e.g. pregnancy, oral contraceptive use) may foster lesion growth. Additionally, the distinctive inflammatory infiltrate in ALHE appears to be an intrinsic (not secondary) component of the lesion. Approximately 20% of patients have blood eosinophilia.

Frequency

Internationally, although frequency is unknown, cases have been reported worldwide. ALHE is uncommon but not rare: it may he more common in Japan than in other countries. It is seen most commonly in Asians, followed by Caucasians. Although less common, blacks can develop ALHE. It is somewhat more common in females; however, a male predominance has been noted in selected Asian studies. ALHE present most commonly in patients aged 20 - 50 years, with mean onset of 30 - 33 years. This condition is rare in elderly patients and in the non-Asian pediatric population.

Lab studies

A complete blood count (CSC) revel as eosinophilia in approximately 20% of patients.

Procedures

The clinical presentation of papules around the ears may suggest ALHE, but a biopsy is required to establish the diagnosis.

Medical care

Treatment is not mandatory. Intralesional corticosteroids and irradiation have been employed but are not very effective.

Surgical care

Surgical removal of the lesions has demonstrated the best results. Excision that include the arterial and venous segments at the base of the lesion prove most efficacious. A 33% recurrence rate has been reported [2] .

Superficial irradiation, cryotheraphy and intralesional steroid therapy have been used, with limited success. Spontaneous resolution can occur [4] .

Complications

No significant complications have been reported.

Prognosis

Lesions tend to remit over the course of months or years; however, they may recur.

 
   References Top

1.Leiferman KM, Gleich GJ. Cutaneous eosinophilic diseases. In: Fitzpatric TB, Eisen AZ, Wolffk, Freedberg IM, Austen KF, eds. Dermatology in General Medicine, 4 th edition, New York, NY: McGraw-Hill Book Co, 1993: 1156-1164.  Back to cited text no. 1    
2.Lever WE, Schaumburg - Lever G. Histopathology of the skin, 7th edition, Philadelphia, PA: JB Lippincott; 1990.  Back to cited text no. 2    
3.Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia: a clinicopathologic study of 116 patients.  Back to cited text no. 3    
4.Kung IT, Gibson JB, Bannatyne PM. Kimura's disease: a Clinico-pathological study of 21 cases and its distinction front angiolymphoid hyperplasia with eosinophilia. Pathology 1984; 16:39-44.  Back to cited text no. 4  [PUBMED]  


    Figures

  [Figure - 1], [Figure - 2]


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