| Abstract|| |
Angiolymphoid hyperplasia with an eosinophilia (ALHE) is a rare benign entity whose etiology and pathogenesis is under debate. Clinically, it is characterised by cutaneous papules or nodules on the head and neck. Literature reveals very few cases of this entity in the oral mucosa. Here, we report a case of ALHE in a 25 year-old woman, who presented with a painless, 5mm x 5mm, sub mucosal erythematous nodule on left angle of mouth at lower lip. Histological examination of lip biopsy specimens revealed an increase in small vessels. The vascular walls consist of prominent endothelial cells with a histiocytoid appearance, which protruded into the lumen. Many eosinophils and lymphocytes were also seen around the vessels. The diagnosis of ALHE was made from the above findings.
Keywords: Angiolymphoid hyperplasia with an eosinophilia, epithelioid hemangioma, oral mucosa
|How to cite this article:|
Aggarwal A, Keluskar V. Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in the oral mucosa. Indian J Dent Res 2012;23:271-4
|How to cite this URL:|
Aggarwal A, Keluskar V. Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) in the oral mucosa. Indian J Dent Res [serial online] 2012 [cited 2016 Jul 24];23:271-4. Available from: http://www.ijdr.in/text.asp?2012/23/2/271/100439
| Introduction|| |
ALHE is an uncommon benign vascular lesion whose etiology and pathogenesis is not yet clarified. It is an uncommon benign vascular lesion and histologically composed of well-formed but often immature vessels lined by epithelioid (histiocytoid) endothelial cells with prominent chronic inflammatory component. 
The term "Angio-lymphoid hyperplasia with eosinophilia" was proposed by Wells and Whimster in 1969.  Today, ALHE, together with some other related vascular lesions, is classified under the general term "epithelioid (histiocytoid) hemangioma". The term epithelioid hemangioma was proposed by Enzinger and Weiss in 1983.
The lesions of ALHE are angioma-like nodules, red to brown, quite small, mainly located intradermally or subcutaneously at the head (face, ears and scalp) and neck region. They
usually occur in young adults, often persist, but may resolve spontaneously.
Histopathologicaly, vascular walls consist of prominent endothelial cells with a histiocytoid appearance, which protruded into the lumen along with eosinophils and lymphocytes which formed around the vessel.
Lesions mostly shows muscular, bone and salivary gland involvement. The cases reported bearing the features of ALHE in an oral mucosa are rare. We describe such a case of this rare association.
| Case Report|| |
Here, we report a rare case of ALHE present on a lip. A 25- year-old female, reported to the department of Oral Medicine and Radiology of KLE VK Institute of Dental Sciences, Belgaum, with a chief complaint of an erythematous nodule on left angle of mouth at lower lip [Figure 1]. The duration of the lesion was 3 years. There was an increase in size of lesion with time. History of occasional discharge of blood from the lesion was also reported. Similar lesion was also found on labial mucosa of upper lip [Figure 2], on labial mucosa of lower lip [Figure 3], and minute similar multiple lesions on the left buccal mucosa.
|Figure 1: Showing Erythematous nodule on left angle of mouth at lower lip|
Click here to view
Lesions were presents on the forehead and left lower one-third of the face at an angle of the mandible. Patient had undergone surgical excision of the skin lesions one month ago. There was no relevant history of an arthropod bite.
The lesions presented as smooth surface nodules, having minute granular appearance with well demarcated border. The lesions were red to brown in color and measured 0.5 x 0.5 cm in size. The lesions were asymptomatic, soft in consistency, non-tender, did not blanch on pressure. There was no regional lymphadenopathy.
Her past dental history was non-contributory and on general physical examination, patient was moderately built and nourished with normal gait .All the vital signs were within the normal range.
Intraoral examination did not reveal any significant dental/mucosal findings in relation to lesions in an oral mucosa. Other intraoral findings include pit and fissure caries with 16 and 17, deep caries with 26, with mild stains and calculus.
Based on history and clinical findings, a provisional diagnosis of 'atypical granuloma' was made.
Salivary gland neoplasm, Hemangioma, Lymphoma, Pyogenic granuloma (lobular capillary hemangioma) was considered as differential diagnosis.
The patient was subjected for excisional biopsy, following a complete blood investigation and the excised tissue was sent for the histopathological examination. Microscopically [Figure 4] at low power, the histology revealed stratified squamous nonkeratinized epithelium along with basilar hyperplasia. The stroma shows flat to plump endothelial cells, showing thickening of blood vessels. Chronic inflammatory cells predominantly lymphocytes and few plasma cells are present. Numerous endothelial lined blood vessels with red blood corpuscles and extravasated red blood corpuscles with minor salivary gland acini predominantly mucous type are also presented.
|Figure 4: A photomicrograph showing flat to plump endothelial cells showing thickening of blood vessels|
Click here to view
The management includes an intralesional corticosteroids, have been employed but are not very effective. Surgical excision of all lesions present on an oral mucosa was done and no recurrence was reported in successive follow up.
Thus on the basis of history, clinical findings, and histopathological evaluation, a final diagnosis of "Angiolymphoid hyperplasia with eosinophilia" was made.
| Discussion|| |
ALHE is benign, locally proliferating lesion composed of the channels of small blood vessel surrounded by lymphocytes and eosinophil. An etiology of ALHE is still unknown, but proposed pathogenesis includes a neoplastic process, a hypersensitivity reaction, an inflammatory vascular reaction or a tissue reaction to a previous trauma as seen in cases of acquired traumatic A-V fistulas. ALHE has been reported to occur following various forms of trauma or infection. Hyperestrogenemia (eg., in pregnancy, with 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. Although frequency is unknown, it has been reported from many parts of the world; and appears to be particularly common in Japan. ALHE is seen most commonly in Asians, followed by whites and less common in blacks. ALHE is slightly more prevalent in females, though a male predominance has been noted in selected Asian studies. It presents most commonly in patients aged 20-50 years, with a mean onset of 30-33 years. 
ALHE typically appears as dome-shaped, smooth-surfaced papules or nodules. Approximately 85% of lesions occur in the skin of the head and neck; most of them are on or near an ear; or on the forehead or scalp. The lesions range from an erythematous to brown and may be eroded or crusted. Approximately 80% of patients present with isolated lesions, while the remaining patients usually demonstrate grouped papules or nodules in a single region. Most lesions are 0.5-2 cm in diameter; with a range of 0.2-8 cm. Larger nodules tend to be deeply centered within the subcutis. 
Kaposi's sarcoma, Salivary Gland Tumor, Squamous Cell Carcinoma, Lymphona, Granuloma Faciale, Insect Bites, Pyogenic Granuloma (Lobular Capillary Hemangioma), Hemangioma can be considered as differential diagnosis with respect to this lesion.
ALHE shows characteristic histologic features, including a proliferation of small blood vessels, many of which are lined by enlarged endothelial cells with uniform ovoid nuclei and intra cytoplasmic vacuoles. These distinctive endothelial cells have been described as having a cobblestone appearance. In addition, a perivascular and interstitial infiltrate composed primarily of lymphocytes and eosinophils is present. Eosinophils typically comprise 5-15% of an infiltrate. Rarely, they can account for as much as 50% of an infiltrate. Occasionally, an infiltrate is devoid of eosinophils. Lymphoid aggregates with or without follicle formation are typical. Pathologically, the EH in an oral mucosa should be distinctive from other vascular tumors showing epithelioid characteristics including epithelioid angiosarcoma, epithelioid hemangioendothelioma, hobnail hemangioma and epithelioid angiomatous nodule. Epitheloid angiosarcoma presents as an infiltrative, destructive growth pattern and composed of markedly pleomorphic cells. Mitoses with atypical forms and necrosis are frequently seen.  Epithelioid hemangioendothelioma microscopically composed of short cords and solid nests of round to slightly spindle-shaped endothelial cells in an infiltrative growth pattern. Well-defined vascular channels are not seen usually. The endothelial cells show mild cellular pleomorphism and intracytoplasmic lumens with a hyalinized or myxoid stroma. Hobnail hemangioma is extremely rare in an oral mucosa and characterized by well-defined, dilated, vascular channels lined by prominent ''hobnailed''endothelial cells.  Cutaneous epithelioid angiomatous nodule was a recent described vascular proliferation with distinct morphologic spectrum. The lesions show considerable overlap with epithelioid hemangioma, in clinical and pathological features. However, epithelioid angiomatous nodules are usually confined to the dermis with only infrequent extension into superficial subcutaneous tissue and rarely in submucosa. At low magnification, the lesions consistently present as an unilobular pattern and no involvement of small muscular vessels were observed. Moreover, the clinical presentation on trunk and extremities should be distinctive from that of the epithelioid hemangioma.
Angiolymphoid hyperplasia with an eosinophilia (ALHE) treatment is often challenging. Intralesional corticosteroids and irradiation have been used but are not very effective. Therapy includes steroids (intralesional, oral), oral retinoids, pentoxifyllin, intralesional chemotherapy (vinblastine, bleomycin, fluouracil), radiotherapy, cryotherapy. The most recommended management remains deep surgical excision.
ALHE and Kimura's disease (KD), which share many common features, must be distinguished because they are unrelated and represent different entities.  ALHE usually appears in Caucasians and is characterised macroscopically by superficial papules or nodules, without lymphadenopathy and accompanied by a mild peripheral eosinophilia. KD, on the other hand, is mostly observed in Orientals and occurs as deep nodules or tumors with lymphadenopathy and a marked peripheral eosinophilia. The main histological features of ALHE is the vascular component, characterised by proliferation and swelling of the endothelial cells, whereas in KD, prominent cellular areas with lymphocytes forming follicles
are observed and are surrounded by an inflammatory infiltrate with an eosinophilia and fibrosis. Histopathologically, ALHE is considered to be of an endothelial cell origin, while KD is a chronic inflammatory process.
In this case, oral ALHE appears as many solitary erythematous nodules on left angle of mouth at lower lip, buccal mucosa, and adult age of 24 years are positive findings, which are the clinical features of an oral ALHE mentioned in the various literature of this disease.
Zhi-Jun Sun et al. examined the clinical records of thirty two patients presenting with an oral ALHE. They found an oral ALHE seems to have a predilection for men and most prominently in young adults (male: female=23:13). The lips are most frequent site (16 out of 36), followed by tongue (10 out of 36), buccal mucosa (6 out of 36), and palate (3 out of 36); while cutaneous ALHE most frequently affects women in the third and fourth decade.
Zhi-Jun Sun et al. also reported that, out of 36 cases of ALHE in oral mucosa, 32 were treated with surgical excision and recurrence rate was very low. This was consistent with this case, wherein all the lesion were subjected to the complete surgical excision and no recurrence were reported.
| Conclusion|| |
Angiolymphoid hyperplasia with an eosinophilia should be considered as one of the differential diagnosis in case of an erythematous nodule present on lip and on an oral mucosa. Complete excisional biopsy is generally used for definitive diagnosis as well as for treatment. Recurrence is generally not reported in cases of an oral mucosa. Treatment is done to restore an esthetic and functional rehabilitation. Other therapeutic approaches used in cutaneous ALHE, such as diathermy, laser cauterisation, cryotherapy, intralesional corticosteroids, oral retinoids and pentoxifylline have not been employed in an oral ALHE.
| References|| |
|1.||Fetsch JF. Epithelioid hemangioma. In: Fletcher CD, Unni KK, editors. World Health Organization classification of tumours: pathology and genetics of tumours of soft tissue and bone. 3 rd ed. Lyon: IARC Press; 2002. p. 159-60. |
|2.||Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol 1969;81:1-14. |
|3.||Hine S. Levy a textbook of oral pathology 5 th ed. India: Elsevier; 2006. p. 33-34 |
|4.||Triantafillidou K, Lazaridis N, Zaramboukas T. Epithelioid angiosarcoma of the maxillary sinus and the maxilla: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:333-7. |
|5.||Mentzel T, Partanen TA, Kutzner H. Hobnail hemangioma (targetoid hemosiderotic hemangioma): Clinicopathologic and immunohistochemical analysis of 62 cases. J Cutan Pathol 1999;26:279-86. |
|6.||Urabe A, Tsuneyoshi M, Enjoji M. Epithelioid hemangioma versus Kimura's disease. A comparative clinicopathologic study. Am J Surg Pathol 1987;11:758-66. |
Department of Oral Medicine & Radiology, KLE’S V K Institute of Dental Sciences, JNMC campus, Nehru nagar, Belgaum, Karnataka
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]