Stubborn Swellings: A Rare Case of Kimura's Disease Presenting as Parotid Swellings.

Kimura disease (KD) is a rare chronic inflammatory disorder characterized by the development of painless subcutaneous nodules, predominantly in the head and neck region. Diagnosis relies on a high index of clinical suspicion and clinicopathological correlation, with core biopsy serving as the gold standard for a definitive diagnosis. While the disease itself is benign, it can cause significant morbidity if left untreated. This case report describes a 48-year-old male who presented with bilateral infraauricular swellings, pruritus, and elevated serum IgE levels along with eosinophilia. Imaging and histopathological correlation confirmed the diagnosis of KD. Combination therapy of corticosteroids and cyclosporine resulted in significant clinical improvement, highlighting the efficacy of the approach while avoiding surgical resection. This case emphasizes the importance of radiologic-pathologic correlation along with the use of serology to effectively diagnose KD, even in atypical presentations.

Orbital Kimura disease: maintenance therapy using mycophenolate mofetil.

Kimura disease (KD) is a rare, chronic, inflammatory condition, predominantly found in male patients of Asian ethnicity. It typically presents between 50-60 years of age and usually with bilateral disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) remains the main differential diagnosis, although histological analysis is essential in differentiating from other similarly presenting pathologies. In this case, we present an atypical case of unilateral orbital KD in a middle-aged, Caucasian, male gentleman and no evidence of regional lymphadenopathy along with a literature review of orbital KD and the differential diagnoses, histological features and management modalities available, adding to the sparse literature on the topic. At present, no recognised diagnostic criteria for KD are available, with histopathological analysis through incisional or excisional biopsy being the primary diagnostic method. Complete surgical excision with or without corticosteroid management remains the most common treatment modality although management is shifting to steroid-sparing immunomodulatory therapy. To the best of our knowledge, this is the first case to describe maintenance therapy of KD using mycophenolate mofetil.

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.

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 2-3 cm 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 eosinophilia and Kimura disease: A case report and literature review.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferative condition, typically presenting as subcutaneous nodules in the head and neck region of middle-aged women. Kimura disease (KD) is a benign condition that presents with subcutaneous nodules in a similar distribution with lymphadenopathy and eosinophilia, typically in Asian adult males. These diseases are often discussed together, including whether they exist on a spectrum or if they represent separate disease entities. Both are very rare in the pediatric population; in this report we highlight the case of a 10-year-old Caucasian male presenting with ALHE and KD.

Post-Traumatic Extensive Scalp Ulceration: A Diagnostic and Management Challenge!

Dissecting cellulitis of scalp (DCS) is an uncommon inflammatory dermatosis, which can be difficult to diagnose in the absence of concomitant cutaneous markers. We present an interesting case of DCS in a 60-year-old male, with an onset after blunt trauma to head. The progression was fast and diagnosis could be achieved on multiple biopsies only. The response to therapy with a combination of oral isotretinoin and intralesional triamcinolone acetonide injections is highlighted in this difficult to treat dermatoses.

Recurrent Angiolymphoid Hyperplasia with Eosinophilia of the Parotid.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasculoproliferative disorder. It usually affects young adults presenting as papules or nodules involving the skin of head and neck region and rarely involves extracutaneous sites. ALHE involving parotid is rare and can be a diagnostic dilemma as it mimics a parotid neoplasm. This is a case of a 23 year old male presenting with a recurrent swelling over the left parotid region post surgery. Ultrasonography revealed a vascular soft tissue lesion in the preauricular region suggestive of a benign lesion. Contrast enhanced magnetic resonance imaging showed a hyperintense lesion involving the superficial lobe of the left parotid gland. Patient underwent superficial parotidectomy and histopathologically was diagnosed to have ALHE. Very few cases have been reported and this case is highlighted as the facial nerve was enmeshed by the intraparotid lesion which was a surgical challenge.

Case Report: Successful Treatment of Recurrent Candida Albicans Meningitis with Kimura's Disease Using Amphotericin B Colloidal Dispersion Combined with Fluconazole.

Background: Candida albicans meningitis is a fungal infectious disease of the central nervous system that most often occurs in immunodeficient populations. Kimura's disease is an IgE-mediated inflammatory reactive disease that is a chronic immune disorder with predominantly lymph node, soft tissue, and salivary gland damage, the treatment of which is hormone-based. The combination of Kimura's disease with C. albicans meningitis is relatively uncommon. Herein, we report a case of C. albicans meningitis in combination with Kimura's disease. Case Presentation: The case is a 26-year-old male with a medical history of Kimura, who presented with symptoms of dizziness, headache, and double vision. Lumbar puncture and cerebrospinal fluid examination revealed an increased white blood cell count. Further analysis through cerebrospinal fluid culture and metagenomic second-generation sequencing (mNGS) led to the final diagnosis of C. albicans meningitis. The patient was treated with fluconazole after the onset of C. albicans meningitis and had a good response. During the treatment, changes in the pathogen genome sequences were monitored dynamically using metagenomic next-generation sequencing. After 1 year, the patient had a recurrence of Candida meningitis. Treatment with fluconazole alone was ineffective, while antifungal treatment with amphotericin B colloidal dispersion was effective with no detectable renal injury. Conclusion: Candida meningitis can occur in the context of Kimura disease. In patients with mild disease, the possibility of recurrence exists with fluconazole treatment alone, and the efficacy of amphotericin B colloidal dispersion combined with fluconazole is better than fluconazole alone in patients with a recurrence. No nephrotoxicity was observed during amphotericin B colloidal dispersion treatment. The mNGS allows dynamic monitoring of pathogen sequencing reads, and for Candida meningitis, there may be a mismatch between peak sequencing reads and disease during treatment, the basis for which is unclear.

An 8-year follow-up of IgG4-related skin disease presented with generalized ALHE-like eruptions: successful treatment of glucocorticoid and cyclophosphamide.

Immunoglobulin gamma (IgG) type 4-related disease (IgG4-RD) is a chronic immunologic systemic disorder that could affect multiple organs, which may cause irreversible organ damage or even death. Skin involvement is rare and associated especially with systemic disease. The dermatologist must be equipped to recognize IgG4-RD to prevent delayed identification and treatment. This case reports a very rare case of IgG4-related skin disease (IgG4-RSD) presenting with a generalized angiolymphoid hyperplasia with eosinophilia (ALHE)-like lesions in a middle-aged male patient with no other organ involvement. He was treated with oral glucocorticoid and cyclophosphamide, which resulted in complete remission. No relapse and disease progression were seen with a follow-up for 8 years.

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.

Rare angiolymphoid hyperplasia with eosinophilia examined through fine needle aspiration cytology, histopathology and immunophenotypic characterization: A case report.

Angiolymphoid hyperplasia with eosinophilia (ALHE), a rare benign proliferative tumor, mainly occurs in several countries in Asia and it is characterized by true vascular branching hyperplasia with infiltration of a large number of lymphocytes and eosinophils in the stroma. The present case report analyzed the clinical symptoms and fine-needle aspiration cytology, histopathological and immunohistochemical results of a patient with ALHE, and summarized the clinicopathological diagnostic features of the disease. To the best of our knowledge, this was the first study to comprehensively report the cytological, histopathological and immunophenotypic characteristics of ALHE, which could help clinicians fully understand this rare type of proliferative tumor.

[Multiple, eruptive epithelioid hemangiomas of the scalp]. / Multiple, eruptive epitheloide Hämangiome der Kopfhaut.

Epithelioid hemangioma is a benign vascular neoplasm with a characteristic histological and immunohistochemical pattern, characterized by a lymphocytic inflammatory infiltrate with admixed eosinophils and FOS­B expression. The correct diagnosis is of particular relevance, since malignant vascular tumors with differentiated epithelioid cells can also be considered in the differential diagnosis. We present a patient with multiple epithelioid hemangiomas of the scalp accompanied by severe pain and itching. The long history of multiple therapeutic attempts illustrates the limited success of currently available treatment options.

A Rare Case of Angiolymphoid Hyperplasia With Eosinophilia With a New Effective Treatment.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign locally proliferating lesion of unknown etiology, composed of vascular channels lined by endothelial cells, surrounded by lymphocytes and eosinophils. It presents clinically as a cluster of skin to violaceous-colored nodules on the head and neck, particularly in and around the ear. We present the case of a 50-year-old, Pakistani woman with unilateral multiple nodular lesions for eight years in the left ear concha and postauricular area causing complete obliteration of the external auditory meatus with conductive hearing loss of the left ear for seven years. Biopsy showed lymphoid follicles and dilated blood vessels with mixed infiltrate predominantly eosinophils corresponding to the diagnosis of angiolymphoid hyperplasia with eosinophilia. Surgical excision was not feasible, and there was no response to topical steroids. The patient was started on beta blockers. After three months, postauricular lesions completely resolved, and the size of the rest of the nodules decreased markedly; then hearing loss also recovered. Our objective in this study is to emphasize the importance of considering beta blockers for the treatment of ALHE.

Angiolymphoid Hyperplasia of the Orbit: A Rare Case Report and Review of the Literature.

Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular tumour of the skin which mainly involves the head and neck regions. Case Report: A 41 year old male presented to the ENT outpatient department with a swelling in the anterosuperomedial aspect of right orbital rim since two year. Following a contrast enhanced CT of the Head, complete surgical excision was done and diagnosis of ALHE was confirmed by histopathology. Methodology: We performed a systematic review of the literature following the preferred reporting items for Systematic reviews. Literature searches were conducted in web based search engines using MeSH terms and key words. We found seven publications that fit the inclusion criteria which included case reports and case series. Conclusion: ALHE involving the orbit has been very rarely reported and is a challenging diagnosis. Complete surgical excision is the treatment of choice and recurrence can occur with incomplete removal.

A common presentation of an uncommon pathology: Kimura disease.

Kimura's disease is a chronic, benign inflammatory condition of the subcutaneous tissue. It presents as painless, subcutaneous nodules of the head and neck, which are firm, painless, and may be single or multiple. It is most commonly seen in young adult Asian men. The nodes remain stable or may slowly enlarge over time. Elevated serum immunoglobulin E (IgE) levels, peripheral blood eosinophilia and lymphoid proliferation with eosinophilic infiltration on histopathological examination are the characteristic features. Spontaneous regression is usually seen. The diagnosis of Kimura's disease can be difficult and misleading, and it is important not to ignore histopathological features.

Angiolymphoid Hyperplasia of the External Auditory Canal; Reconstruction of a Complex Defect.

Angiolymphoid hyperplasia (AH) was first described by Wells and Whimster in 1969 as a benign vasoproliferative pathology with a varied infiltrate of eosinophils, lymphocytes, and plasmatic cells. Clinical presentation has been described in the literature as small red-bluish nodules, less than 3 cm in diameter that can bleed in 25% of the cases and be pruritic and painful in 37% and 20% of the cases, respectively. Particularly, AH can appear in the ear; nevertheless, other regions have been affected, including the scalp, lips, tongue, orbit, muscle, and bone. Most of these cases have occurred in adults with an unknown etiology; however, an inflammatory process has been associated in approximately 20% with eosinophilia. No malignancy has been reported.

Kimura's disease: A short study of cytomorphologic features with its differential diagnosis and review of literature.

Kimura's disease (KD) is a chronic inflammatory disorder of unknown etiology, endemic in Asia. The typical clinical manifestations include a triad of painless unilateral cervical lymphadenopathy or subcutaneous masses predominantly in the head-and-neck region, blood and tissue eosinophilia, and elevated serum immunoglobulin (Ig) E levels. Many conditions including benign and malignant may mimic KD clinically. This study reports cytologic features of seven cases of KD which were studied and correlated with histology, Ig profile, and peripheral blood examination. KD shows a good response to medical treatment; hence, fine-needle aspiration cytology in conjunction with other laboratory findings lowers the need for additional biopsy procedures for early diagnosis as well as diagnosis of recurrent lesions.

[Kimura's disease mimicking primary neoplasm of the parotid gland: A case report]. / Enfermedad de Kimura simulando neoplasia primaria de glándula parótida: Reporte de caso.

Background: Kimura's disease is an infrequent inflammatory disorder, of unknown etiology, with few reports outside of Asia. It presents as a nodule or tumor predominantly in the postauricular region, neck and parotid gland. It is histologically characterized by follicular hyperplasia with wellformed mantle zones, preservation of nodal architecture, prominent eosinophilic infiltrate in the germinal centers and interfollicular areas; and associated with elevated levels of IgE and peripheral eosinophilia. Clinical case: We present a case of a 23-year-old man from Mexico, he presented with a recurrent tumor in the right parotid gland, previously treated with surgical resection. Imaging studies were performed and a primary neoplasm of the salivary gland was suspected, he was treated with surgical resection. The histological diagnosis was Kimura's disease. Conclusions: Communication and divulgation of this rare inflammatory disorder expans the knowledge for the differential diagnosis of tumors of the head and neck, and salivary glands, mainly in men with peripheral eosinophilia and elevated IgE; it can sometimes simulate malignant neoplasms, leads to inadequate diagnostic and therapeutic approaches.

Introducción: la enfermedad de Kimura es un desorden inflamatorio poco frecuente, de etiología desconocida y raramente reportado fuera del continente asiático. Se presenta como un nódulo o tumor predominantemente en la región retroauricular, cervical o glándula parótida. Se caracteriza histológicamente por hiperplasia folicular con zonas del manto bien formadas, preservación de la arquitectura ganglionar, infiltrado eosinofílico prominente en los centros germinales y áreas interfoliculares; generalmente asociada a niveles elevados de IgE y eosinofilia periférica. Caso clínico: presentamos el caso de un hombre de 23 años, de origen mexicano que se presentó con un tumor recidivante a dos años de resección quirúrgica previa en glándula parótida derecha, se realizaron estudios de imagen y se sospechó de neoplasia primaria de glándula salival, fue tratado con resección quirúrgica. El diagnóstico histológico fue de enfermedad de Kimura. Conclusiones: la comunicación y difusión de este raro desorden inflamatorio amplía la base del conocimiento para el diagnóstico diferencial de tumores de cabeza y cuello, y glándulas salivales, predominantemente en hombres con eosinofilia periférica y elevación de IgE; que en ocasiones puede simular neoplasias malignas, llevando a abordajes diagnósticos y terapéuticos inadecuados.