Angiolymphoid Hyperplasia Research Articles

076. Surgical Management Challenges in Recurrent Kimura Disease: A Case Study of a Rare Disease

Background: Kimura disease (KD) is a rare autoimmune disease characterized by angiolymphoid hyperplasia with eosinophilia, primarily affecting young males of Asian descent. First described in 1948, approximately 200 cases have been reported globally. Despite surgical excision, recurrence is common, posing significant challenges for management. This report details the management challenges of recurrent KD. Case: A 30-years-old male presented with recurrent KD of the right buccal. Histologic examination revealed chronic lymphadenitis and eosinophilia. MRI showed a solid heterogeneous mass in the right buccal region and superficial to deep lobe of the right parotid gland, with multiple reactive lymphadenopathies. The patient underwent superficial instead of total parotidectomy due to dissection complexities, revealing a large tumor (13 cm in diameter) with poorly defined borders and extensive fibrotic tissue. Postoperatively, the patient received an immunomodulator and systemic corticosteroid without any follow-up surgery. Surgical excision remains the gold standard for localized KD, but likelihood of recurrence increases when tumors exceed 3 cm. Adjunctive therapies such as systemic corticosteroid, chemotherapy, and radiotherapy are considered for recurrence prevention. Systemic corticosteroids are used to reduce inflammation and lesion size but may lead to relapse upon withdrawal. Postoperative radiotherapy may help control residual disease. The use of targeted therapies such as anti-IL-5 monoclonal antibody showing promise as an immunomodulator. Conclusion: The treatment of KD requires a comprehensive approach that considers the extent of the disease and the potential recurrence. Surgical excision is the primary treatment modality, supplemented by immunosuppressive agents, radiotherapy, and chemotherapy.

Angiolymphoid hyperplasia with eosinophilia of the ear.

Angiolymphoid hyperplasia with eosinophilia of the ear.

Characterization and Management of Epithelioid Hemangioma on the Lip Mucosa: A Case Report

Epithelioid Hemangioma (EH) is a rare benign vascular tumor characterized by well-formed blood vessels and epithelioid endothelial cells. Known as Angiolymphoid Hyperplasia with Eosinophilia (ALHE), the World Health Organization does not recommend the use of this term since 2020. Despite its common occurrence in subcutaneous soft tissues of head and neck, cases involving oral mucosa and other extra cutaneous areas are infrequent. This article presents a case of EH in an unusual location and discusses the differential diagnosis with other entities, including Kimura´s Disease (KD). We report the clinical case of a 26 year old man with no significant medical history who presented with a painless increase in volume on the right upper lip, subsequently diagnosed as EH through biopsy and histopathology. The lesion exhibited typical clinical features and a complex histological pattern including epithelioid endothelial cells and an inflammatory infiltrate rich in eosinophils. Diagnosis of EH is challenging due to morphological variability and the need to differentiate it from similar conditions such as KD. Differential diagnosis is crucial, especially in atypical locations. This case shows the importance of histopathology and complementary examinations in diagnosis and the need to consider KD in the differential diagnosis due to its therapeutic and prognostic implications. Therefore, it is concluded that EH, although benign, requires a rigorous diagnostic approach to differentiate it from similar diseases and manage it appropriately. Continuous control is essential due to the potential for recurrence. This case shows the importance of careful differential diagnosis, especially in atypical locations, to optimize management and improve patient outcomes.

JAAD Game Changers: “Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia: A systematic review”

JAAD Game Changers: “Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia: A systematic review”

Ear lobule reduction using a sub-antitragal groove technique in patients with angiolymphoid hyperplasia with eosinophilia on the earlobe: a case report and literature review.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare condition characterized by nodular lesions predominantly in the head and neck region, often causing discomfort or pain. Treatment remains challenging because of its rarity and the lack of established guidelines. This report presents a case of ALHE affecting the earlobes that was successfully managed using ear lobule reduction surgery and subsequent intralesional steroid injections. A 31-year-old woman with a history of recurrent earlobe masses underwent a partial excision to avoid the loss of the earlobe. Histopathological examination confirmed Kimura disease, a variant of ALHE. Subsequent local methylprednisolone injections effectively controlled the remaining lesions, resulting in significant size reduction without notching. Various treatment modalities have been attempted for this condition; however, recurrence rates remain high. Surgical resection combined with intralesional corticosteroid injections is the preferred approach. In this case, a sub-antitragal groove technique for earlobe reduction was employed to preserve the lateral edge of the ear lobule, minimize the risk of deformity, and achieve a predictable outcome. The sub-antitragal groove technique offers an approach to reduce earlobe size without compromising aesthetics. Further research is required to elucidate the pathogenesis of ALHE and establish standardized treatment protocols for this rare condition.

Unusual Presentation of Marginal Zone Lymphoma

Primary cutaneous B-cell lymphomas (pcBCLs) are non-Hodgkin lymphomas that present with exclusive cutaneous disease at the time of diagnosis. The lesions may be indolent, subtle, or waxing and waning, making the diagnosis challenging. The ear is an uncommon location, and its frequency of involvement remains unknown. A retrospective analysis of a single patient with primary cutaneous marginal zone lymphoma of the ear. The patient was a 44-year-old male who presented with erythematous tender nodules on the left superior posterior helix. Shave biopsy revealed mixed dermal lymphoid infiltrate with eosinophils and telangiectasias. The patient was diagnosed with angiolymphoid hyperplasia with eosinophilia. Multiple treatment regiments were trialed but failed due to lack of therapeutic response, relapse, and clinical progression. The patient ultimately underwent Mohs surgery with biopsy findings that demonstrated CD-20 positive cells. Evaluation with B-cell histopathology and gene rearrangement studies confirmed the diagnosis of marginal zone lymphoma. This case report shows a unique presentation of marginal zone lymphoma of the ear, initially diagnosed as angiolymphoid hyperplasia with eosinophilia. Our findings demonstrate a relatively rare presentation for this disease and highlight the challenges in diagnosing primary cutaneous marginal zone lymphoma.

Epithelioid Hemangioma: Report of a Case Series of 4 Patients

Epithelioid hemangioma or Angiolymphoid Hyperplasia with Eosinophilia (ALHE) is a rare benign vascular disease, characterized by solitary or multiple angiomatous papules and nodules, which are usually located on the skin of scalp and face (mainly in the preauricular zone); these have characteristic histological and immunohistochemical patterns, in particular characterized by an important lymphocytic inflammatory infiltrated with many eosinophils and vascular proliferation of capillaries with endothelial cellular changes. In this paper, four cases of this disease are reported, in the first series of Ecuadorian cases described in the literature.

The "great imitator": IgG4-related disease of the oral cavity. Two case reports and scoping review.

This study aimed to review the lesser-known intraoral manifestations of immunoglobulin G4-related disease (IgG4-RD). In this paper we report an unprecedented case of oral IgG4-RD mimicking angiolymphoid hyperplasia with eosinophilia (ALHE), and another case presenting as plasma cell gingivitis. We then performed a scoping review of published cases of IgG4-RD involving the oral cavity. The following data were collected for each case: age, sex, intraoral site(s) involved, clinical appearance, imaging features, serum IgG4 values, histopathology, treatment, and follow-up duration. Fifty-one cases of oral IgG4-RD were published in literature. The hard palate and jaw bones were the two main locations reported, while the histological identification of a IgG4/IgG plasma cells ratio ≥40% was fundamental for diagnosis. Conversely, the pathological features of storiform fibrosis and obliterative phlebitis were not common. Future reports regarding oral IgG4-RD should report clear adherence to the recognized international diagnostic criteria of the disease.

Hiperplasia Angiolinfoide: consideraciones clínico- patológicas

Two cases of angiolymphoid hyperplasia with eosinophilia or Kimuras disease are presented. A nodular and papuoid pattem of presentation was observed. The first one is the histologic expression of the vascular and cellular components which were found n the subcutaneous fat whereas the latter was in the dermis.Clinical asid pathological considerations sere made to differentiate both illnesses. However, due to the overlapping of certain criteria it is considered that ve are dealing with the same disease at different phases.

Epithelioid Hemangioma Masquerading as Traumatic Lesion: A Rare Case Report with Diagnostic Workup

Abstract Wells and Whemser first identified epithelioid hemangioma (EH) as angiolymphoid hyperplasia in 1969. It is a rare type of vascular tumor that primarily affects men in their middle ages. Typically, it appears as a single or few reddish-to-brown nodules. EHs of the oral cavity have only seldom been reported up to this point. Histologically, it is distinguished by lobular vessel configuration, tombstone-like epithelioid cells lining the vessel wall, and a persistent inflammatory infiltration predominately made up of eosinophils. Although the terms EH and epithelioid hemangioendothelioma are sometimes confused, the two are distinct based on histopathology and a number of immunohistochemistry (IHC) markers, including high Ki67 index and ERG positivity observed later. Local trauma to a nearby vessel may be one of the etiopathogeneses for this entity. This is a rare case report of EH that was identified with the use of a panel of IHC markers and hematoxylin and eosin histology.

Angiolymphoid hyperplasia with eosinophilia in the eyelid: A case series and literature review

Abstract: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular disorder characterized by the proliferation of blood vessels and lymphoid tissue. While it can occur in various regions of the body, ALHE presenting in the eyelid is relatively uncommon, leading to diagnostic and management challenges. In this case series from South India, we present three unique cases of ALHE specifically affecting the eyelids. Each case illustrates different clinical manifestations, diagnostic workups, and treatment approaches. Tissue biopsies typically reveal nodules comprising immature and mature vascular structures, including capillaries, arterioles, and venules, which are lined by plump endothelial cells with an epithelioid appearance. The surrounding stroma contains a dense infiltrate of inflammatory cells, predominantly eosinophils, histiocytes, lymphocytes, and mast cells. Increased awareness of ALHE in the eyelid will aid ophthalmologists in early recognition, accurate diagnosis, and appropriate management, resulting in improved patient outcomes.

Treatment of Vulvar Angiolymphoid Hyperplasia with Eosinophilia with Topical Corticosteroids

Abstract is missing (Short communication)

A Case of Angiolymphoid Hyperplasia with Eosinophilia Treated with Combination Therapy of Topical and Intralesional Corticosteroids

Angiolymphoid hyperplasia with eosinophilia is an uncommon, benign vasoproliferative disorder that can present with single or multiple pink to red-brown dome shaped papules, predominantly on the head and neck. The most common reported symptoms include, but are not limited to, pruritus, bleeding and pain. Currently, there is no definitive treatment for this condition, with numerous therapeutic modalities described in the literature. The most effective and commonly used treatment is excision but can be more invasive and still has a high failure rate (40.3%). Despite the high failure rates associated with topical, systemic and Intralesional corticosteroids, we were able to show significant improvement with the combination of topical and Intralesional corticosteroids. This form of therapy is considerably cheaper and non-invasive compared to alternative therapies. Given the high recurrence and lack of definitive treatments, more research is needed for a better understanding of pathophysiology and identification of effective therapies.

214 Angiolymphoid hyperplasia with eosinophilia and kimura disease: a case of clinical and histopathological discrepancy and response to dupilumab

214 Angiolymphoid hyperplasia with eosinophilia and kimura disease: a case of clinical and histopathological discrepancy and response to dupilumab

Angiolymphoid Hyperplasia with Eosinophilia- Think Twice with Scalp Papules! A Case Report

Angiolymphoid hyperplasia is an uncommon condition characterized by multiple erythematous papules and nodules which are vascular and present over the head and neck area, predominantly around the ears. It is a benign condition but treatment is often sought for the appearance of lesions. We report a case that had a recurrence of erythematous lesions after incomplete electrocoagulation and the diagnosis of angiolymphoid hyperplasia with eosinophilia was confirmed on histopathological evaluation after complete excision of the lesion. In conclusion, as angiolymphoid hyperplasia can be clinically misdiagnosed, histopathological evaluation is warranted in erythematous nodules presenting over the sites of predilection.

Angiolymphoid hyperplasia with eosinophilia of the tongue – A case report

Angiolymphoid lymphoid hyperplasia with eosinophilia (ALHE) represents a heterogenous group of benign vascular lesions that is characterized by proliferation of small to medium sized vascular structures lined by plump epitheloid (histiocytoid)endothelial cells surrounded by mixed inflammatory infiltrate predominantly eosinophils. Intraoral lesions of ALHE are uncommon and tongue involvement is extremely rare. Here we report a case of ALHE of tongue in a 60 yr old female diagnosed on histopathology and confirmed by IHC.

An Unusual Presentation of Angiolymphoid Hyperplasia with Eosinophilia as Postauricular Mass: A case Report.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, reactive vaso-proliferative condition in the dermal and subcutaneous tissues of the head and neck. A 28-year-old female presented with slow-growing painless swelling behind her left ear. FNAC revealed benign soft tissue neoplasm and histopathological examination after surgical excision revealed angiolymphoid hyperplasia with eosinophilia. ALHE origin has been variously attributed to prior trauma, hyperestrogenemia, infectious agents, atopy, reactive hyperplasia, and benign neoplasia. Retroauricular ALHE has been rarely reported. However, on the basis of our case report, it should be a viable differential diagnosis when large subcutaneous tumors of the head and neck are encountered. When big subcutaneous tumors of the head and neck are present, especially in females, a valid differential diagnosis for angiolymphoid hyperplasia with eosinophilia, a rare condition marked by dermal or subcutaneous endothelial cell proliferation, should also be considered.

Angiolymphoid Hyperplasia with Eosinophilia of the External Auditory Canal.

Angiolymphoid hyperplasia with eosinophilia is a rare, benign, vaso-proliferative disease with a dense eosinophilic inflammatory infiltrate seen in the intradermal or subcutaneous region. It is also termed an epitheloid hemangioma, and it mostly affects females rather than males. It is seen as reddish to brown, fleshy, proliferative, papular, or nodular lesion affecting the head and neck region, most commonly the auricular and periauricular regions. The aetiology is unknown, but the most likely causes are trauma, infection, and hyperestrogenic condition like pregnancy or the use of oral contraceptive agents. It usually measures about 2-3cm in size. It should be differentiated from Kimura disease, which is characterized by chronic inflammation and large subcutaneous nodules in the head and neck region. Here is a rare case report of angiolymphoid hyperplasia in a middle-aged male patient involving the external auditory canal of the left ear.

Angiolymphoid Hyperplasia With Temporal Artery Eosinophilia: A Case Report.

Angiolymphoid Hyperplasia with Eosinophilia (ALHE) is a benign vascular proliferative disorder with uncertain etiology and pathogenesis. The aim of this paper is to report a case of ALHE in the temporal artery and discuss the general aspects of this pathology. A 29-year-old female black patient sought the Vascular Surgery Outpatient Service, complaining of bulging in the right temporal region, associated with pain and local discomfort. Physical examination revealed pulsatile bulging in the right temporal region measuring approximately 2.5 × 1.5cm. Nuclear Magnetic Resonance showed an expansive fusiform lesion in the superficial soft parts of the right temporal region, measuring 2.9cm in the longest longitudinal axis. Surgical excision proved to be the best therapeutic option for the patient in this case. Histopathological sections showed the proliferation of vessels of different sizes, covered by swollen endothelium, prominent inflammatory infiltrate composed of lymphocytes, plasma cells, eosinophils, and scarce histiocytes. Immunohistochemical analysis of the lesion showed positivity for CD31, corroborating the diagnosis of ALHE.

Epithelioid hemangioma (ALHE) on the tongue of an infant treated with oral corticosteroids: A case report and review of the literature.

Epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular lesion presenting as multiple nodules on the head and neck. Surgery had been considered to be the best treatment modality for ALHE. We report the case of a 6-month-old boy with ALHE on his tongue that was treated successfully with oral prednisolone.