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CASE REPORT
Year : 2022  |  Volume : 11  |  Issue : 3  |  Page : 246-250

Angiolymphoid hyperplasia with eosinophilia of the palate: A case report with literature review


1 Department of Oral Pathology, Vishnu Dental College, Bhimavaram, Andhra Pradesh, India
2 Department of Oral and Maxillofacial Surgery, Vishnu Dental College, Bhimavaram, Andhra Pradesh, India
3 Department of Oral Medicine and Radiology, Vishnu Dental College, Bhimavaram, Andhra Pradesh, India

Date of Submission27-Apr-2021
Date of Acceptance19-May-2021
Date of Web Publication26-Dec-2022

Correspondence Address:
Dr. Satya Tejaswi Akula
Department of Oral Pathology, Vishnu Dental College, Bhimavaram - 534 202, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdrntruhs.jdrntruhs_48_21

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  Abstract 


Angiolymphoid hyperplasia with tissue eosinophilia (ALHE) is a rarely occurring benign vascular tumor of unknown etiology and most commonly affecting the head and neck region. It usually presents clinically as brownish or purplish papules or nodules with major prevalence in females. As the name defines, histologically it shows proliferating endothelial cells and lymphoid aggregates with stromal eosinophilia. It is very rarely identified clinically and the diagnosis is purely based on histopathology. This is a unique case report of intraoral ALHE with an unusual clinical appearance as ulceroproliferative lesion that showed quick remission after incisional biopsy.

Keywords: ALHE, endothelial cells, eosinophilia, Kimura's disease, papulonodules


How to cite this article:
Medikonda P, Akula ST, Manyam R, Swetha P, Moturi K, Tatapudi R. Angiolymphoid hyperplasia with eosinophilia of the palate: A case report with literature review. J NTR Univ Health Sci 2022;11:246-50

How to cite this URL:
Medikonda P, Akula ST, Manyam R, Swetha P, Moturi K, Tatapudi R. Angiolymphoid hyperplasia with eosinophilia of the palate: A case report with literature review. J NTR Univ Health Sci [serial online] 2022 [cited 2023 Feb 7];11:246-50. Available from: https://www.jdrntruhs.org/text.asp?2022/11/3/246/365021




  Introduction Top


Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular tumor of unknown etiology. It is characterized by red to brown papulonodules, typically localized to the head and neck region, particularly around the ear, as single or multiple lesions. Young to middle-aged females are more commonly affected. The histological examination corresponds to a florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate.[1] In this article, we report a unique case of ALHE in a 55-year-old female patient with an ulceroproliferative presentation unlike an ALHE, in an uncommon intraoral location––palate, associated with denture-induced trauma which showed spontaneous healing in 4 weeks after incisional biopsy.


  Case Report Top


A 55-year-old married female presented with a complaint of pain and burning sensation on her palate since 1 month. A remarkable history of denture use for 2 years was identified, which she stopped wearing after developing the symptoms. The patient's medical history was normal, except for the use of antidepressant drugs occasionally from the past 10 years. A solitary ulceroproliferative lesion was witnessed on oral examination involving posterior part of the hard palate and a small portion of the soft palate on the left side [Figure 1]. The lesion measured about 2.5 cm × 1.5 cm, irregular in shape with an everted border and ill-defined, erythematous margins. Palpatory findings showed the lesion was soft, tender, covered by a non-scrapable whitish slough, and with an indurated border. Regional lymphadenopathy was absent, and no abnormality was detected related to other organ systems. All these findings were correlated, and a provisional diagnosis of non-healing ulcer was thought. A complete hematological examination was done prior to the surgical biopsy procedure. All the test reports obtained were within the normal range. An incisional biopsy was performed by taking tissues from two clinically distinct and prominent sites of the lesion.
Figure 1: Clinical image of a 55 year old female patient showing extensions of the palatal lesion which is ulceroproliferative involving the hard palate and small portion of soft palate surrounded by erythematous margins

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Histopathology of the ulceroproliferative lesion revealed hyperplastic stratified squamous parakeratinized epithelium showing acanthosis and mild intraepithelial neutrophilic infiltrate and areas of ulceration. The connective tissue stroma is hypercellular with highly proliferating and enlarged epithelioid endothelial cells containing vacuolated cytoplasm. A few of them were seen protruding deeply into the lumen giving a solid sheet-like appearance. Evidence of damaged arterioles in few regions was noted. Dense lymphocytic aggregates surrounded the endothelial proliferations along with plasma cells and with significant eosinophilic infiltration. Deeper connective tissue composed of mucous acini, their ducts, and adipocytes showing degenerative changes due to this dense and diffuse lymphocytic infiltration were also observed [Figure 2] and [Figure 3]. There was no nuclear atypia, hyperchromasia, or increased mitotic activity. These findings were substantial enough to label our case as ALHE.
Figure 2: Sheets of epithelioid endothelial cells with vesicular nucleus and occasional mitotic figures surrounded by dense lymphoplasmacytic infiltrate

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Figure 3: Connective tissue stroma showing epithelioid endothelial cells intermixed with dense eosinophilia

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After the incisional biopsy, the patient was given with amoxicillin 500 mg, ketorolac 10 mg, and pantoprazole 40 mg for 5 days. Frequent follow-up was done for every week until 2 months. Healing and slight regression of the lesion was observed in the second week and a complete healing along with regression up to 60% was seen in the fourth week of follow-up. By the end of sixth week, the lesion has completely subsided [Figure 4]. A thorough follow-up was done until 6 months where no recurrence was noted.
Figure 4: Postoperative image after 4 weeks showing regression of the lesion more than 60%

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  Discussion Top


ALHE is a rare benign vascular tumor first described in 1969 by Wells and Whimster. They considered ALHE to be a late stage of Kimura's disease, described in the Japanese literature in 1948. It should be considered as a benign reactive inflammatory lesion rather than a tumor. It has been known by multiple names, such as epithelioid hemangioma, pseudopyogenic granuloma, inflammatory angiomatous nodule, papular angioplasia, subcutaneous angioblastic lymphoid hyperplasia with eosinophilia and lymphofolliculosis, intravenous atypical vascular proliferation, and histiocytoid hemangioma. It has been classified recently under cutaneous vascular hyperplasias, along with pyogenic granuloma and bacillary angiomatosis.[2]

It is characterized by one or more brownish to purplish papules and nodules with a predilection for the head and neck region. Most papulonodules are dermal in location, but some are subcutaneous; occasionally, ALHE involves deeper soft tissues or arises from vessels. The actual etiopathogenesis of ALHE is unknown; although two viral causes have been proposed. The first is herpes virus type 8 (HHV-8) which is also known to cause Kaposi's sarcoma. The other virus is the EBV that is a known cause of infectious mononucleosis, Hodgkin's lymphomas, Burkitt's lymphomas, and nasopharyngeal carcinoma.[2] A trauma history can be elicited in some patients. The frequent presence of mural damage or rupture in intralesional large vessels of ALHE has suggested a role for trauma or arteriovenous shunting in its pathogenesis. However, various stimuli inducing reactive vascular hyperplasia is the most accepted hypothesis. Factors like hyperestrogenemia (e.g., pregnancy, oral contraceptive use) may foster lesion growth. T-cell monoclonality has been reported in few cases without any further explanation.

This tumor has a high prevalence for females and with a wide age range of third to fifth decade. Skin lesions most commonly occur in the preauricular area (36%) followed by facial area (28,2%) and scalp (17,3%). Less commonly, lesions have been observed in the mouth and on the trunk, distal extremities, vulva, and penis. Approximately half of the patients have multiple lesions, generally in the clustered pattern. ALHE can be either asymptomatic or painful, pruritic or pulsatile. Some patients have regional lymph node enlargement and an investigatory finding of peripheral eosinophilia. Radiologic ex-aminations such as MRI or angiography may be required to determine the extension of the lesions which are deep-seated or associated with vessels.[3] However, confirmative diagnosis is based upon histological findings.

ALHE is often mistaken for Kimura's disease. In some older reports, ALHE and Kimura's disease were even described as the same disease with a spectral difference [Table 1]. Both lesions present as nodules, preferably in the head and cervical region, but in ALHE they tend to be much more erythematous as opposed to Kimura's disease, where the lesions are normochromic. ALHE lesions are localized in the superficial tissues, while Kimura disease involves deeper tissues such as lymph nodes and salivary glands. On the contrary, there are few reports of coexisting Kimura's disease and ALHE. But unlike Kimura's disease ALHE shows eosinophilia in only 20% of the cases, and lymphadenopathy is uncommon. They also share histopathological similarities.[4]
Table 1: Differentiating features between alhe and Kimura's disease

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The diagnosis of ALHE is solely based on histopathology because it is a histological diagnosis rather than a clinical diagnosis. Different phases of the disease show different histopathological pictures. The early phase shows high cellularity with a more vascular component, whereas in later stages there will be more inflammatory components around mature, smaller endothelial cells. Classically there is a proliferation of small to medium-sized capillaries and blood vessels, often exhibiting a lobular architecture, most of which are lined by prominent endothelial cells with varied appearance. Histologically, the prominent endothelial cells show a “histiocytoid” or “epithelioid” appearance with highly vacuolated cytoplasm. A perivascular inflammatory cell infiltrates composed predominantly of lymphocytes and eosinophils is an important finding. Eosinophils usually account for 5-15% of the infiltrate, though in rare cases eosinophils may account for up to 50%. Immunohistochemical evaluation of the lesions shows endothelial cells positive for CD34, von Willebrand factor, and CD31.[3]

Our case was not classically characterized by all the features of ALHE but showed female preponderance, trauma-induced bleeding, non-healing nature with erythematous appearance and histopathological findings like proliferating epithelioid endothelial cells showing highly vacuolated cytoplasm with incompletely developed lumina. Another contrasting feature is the involvement of minor salivary glands which is unlike ALHE and more likely towards Kimura's disease. But it was ruled out as prominent lymphoid and eosinophilic aggregates were seen without any evidence of germinal centers and lack of peripheral eosinophilia.

Other histological differential diagnoses of ALHE include vascular tumors and tumor-like conditions of blood vessels, such as angiosarcoma, cutaneous epithelioid angiomatous nodule, epithelioid hemangioendothelioma, and bacillary angiomatosis. Although it resembled angiosarcoma because of the highly cellular nature of the lesion in our case, it was ruled out because of the absence of conspicuous cytological atypia, frequent mitotic figures, or piling up of cells.[4]

Treatment options are pulse-dye laser, topical tacrolimus, and surgery. Although instances of spontaneous regression have been reported, excisional surgery is the one with lower recurrence and higher cure rate compared to others. It is reasonable to observe the lesion for 3–6 months and await spontaneous regression. The present case also has shown regression within 4 weeks after performing the incisional biopsy. About one-third of lesions recur after excision which may be associated with incomplete surgical excision by the operators. Several other treatment modalities have been used, including intralesional corticosteroid injections, interferon α2b, cytotoxic agents, and electrodessication. Intralesional corticoid therapy is effective with recurrence rates similar to the surgical excision, however with the esthetic result. Other treatments such as cryotherapy, photodynamic therapy, and application of imiquimod which acts by inducing the production of interferon-α and inhibition of certain cytokines, mainly interleukin-5, implicated in the genesis of ALHE are described in the literature with good results.[5]

Acknowledgments

Authors acknowledge the Department of Maxillofacial Pathology and Microbiology, Department of Oral Medicine and Radiology and Department of Oral Surgery and, Vishnu Dental College, Bhimavaram, Andhra Pradesh, India.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Youssef A, Hasan AR, Youssef Y, Al-Soufi L, Elshimali Y, Alshehabi Z. Angiolymphoid hyperplasia with eosinophilia: A case report. J Med Case Rep 2018;12:44-7.  Back to cited text no. 1
    
2.
Santosa C, Wardhana M, Saputra H. Angiolymphoid hyperplasia with eosinophilia with clinical pictures of keratoacanthoma: A rare case report. Clin Case Reports 2019;7:189-92.  Back to cited text no. 2
    
3.
Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia: A clinicopathologic study of 116 patients. J Am Acad Dermatol 1985;12:781-96.  Back to cited text no. 3
    
4.
De Bastos Jt, E Silva Pmc, Cassia F De F, Da Rocha Crm, De Freitas Bmp, Avelleira JCR. Angiolymphoid hyperplasia with eosinophilia versus Kimura's disease: A case report and a clinical and histopathological comparison. An Bras Dermatol 2017;92:392-4.  Back to cited text no. 4
    
5.
Adler BL, Krausz AE, Minuti A, Silverberg JI, Lev-Tov H. Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia (ALHE): A systematic review. J Am Acad Dermatol 2016;74:506-12.e11.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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