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.

Kimura\'s disease (KD) is a chronic inflammatory disease characterized by painless subcutaneous head and neck swelling, eosinophilia, and elevated serum immunoglobulin (Ig) E levels. There are various therapies, including surgery, radiation, systemic steroids, and immune suppressants, but their efficacy remains moderate due to the high recurrence rate. Biologics, like monoclonal antibodies, have shown tremendous effectiveness for chronic inflammatory diseases. Omalizumab is a monoclonal antibody against IgE and has not been approved for KD so far. We describe two refractory KD cases that responded to a small dose of steroids plus omalizumab. Additionally, we reviewed another 13 KD cases that were treated with biologics, including omalizumab, rituximab, dupilumab, and mepolizumab. The results indicate that biologics provide an alternative treatment strategy for KD.

UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

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.

Kimura\'s disease (KD) is a rare chronic inflammatory disorder that commonly occurs in Asian males. It mainly presents as painless subcutaneous masses or lymphadenopathy in the head and neck region. The incidence of KD in the oral cavity is quite rare. We reported a rare case of a 53-year-old male who had KD in his soft palate, hard palate and bilateral tonsils associated with severe sleep apnea. This patient underwent radiotherapy and exhibited a good response to the treatment. Throughout the 12-month follow-up period, the patient\'s condition remained satisfactory. Of the other 14 reviewed cases of KD in the oral cavity, the lesions can occur in the buccal mucosa, hard and soft palate, and mouth floor with specific clinical features. We further summarized their manifestations and treatments in order to guide the future identification and management of KD with lesions in the oral cavity.

UNASSIGNED: Kimura\'s disease (KD) is an eosinophilic proliferative lymphoid disease with rare incidence rate and nonspecific clinical symptoms.
UNASSIGNED: To investigate the clinical characteristics, diagnosis, treatment, and prognosis of KD.
UNASSIGNED: The clinical manifestations, laboratory examination, imaging examination, proposed treatment, and prognosis of 20 patients with KD treated in our hospital were retrospectively analyzed.
UNASSIGNED: All cases showed painless masses. The proportion of peripheral blood eosinophils rose in 18 cases (90%), the eosinophil count increased in 14 cases (70%) and the serum immunoglobulin E (IgE) levels were significantly increased in 5 patients (100%). Pathological examination revealed follicular hyperplasia, eosinophils infiltration, and tiny vessels proliferation in KD samples.
UNASSIGNED: KD should be considered when the patients suffered head and neck subcutaneous painless mass, increased peripheral blood eosinophils and raised serum IgE level. Pathological examination is the golden standard for diagnosing KD. Surgical intervention, medical treatments, and radiotherapy may be effective for KD treatment.
UNASSIGNED: This study might shed a light on improving the diagnosis and treatment for KD.

BACKGROUND: Kimura\'s disease is a rare, benign, chronic inflammatory disease that presents as painless, solid masses mainly affecting the deep subcutaneous areas of the head and neck, especially the salivary glands, parotid glands and nearby lymph nodes. It is characterized by elevated peripheral blood eosinophil and Immunoglobulin E (IgE) levels.
METHODS: A 31-year-old Asian male presented with an orbital space-occupying lesion lasting for 1.5 years. Ten years prior, surgical excision of bilateral fossa cubitalis and groin masses was performed, and the pathological examination showed \"lymphoproliferative disease\". One year later, masses reappeared near the surgical sites; they grew slowly and shrank after glucocorticoid treatment. At this point, admission examinations showed in the peripheral blood an eosinophil proportion of 13.4%, a total IgE level of 26,900.00 IU/mL, prurigo present on the whole body, and multiple palpable masses near the bilateral fossa cubitalis and groin. The left eyeball was exophthalmic. The left elbow mass was excised, and the pathological examination confirmed Kimura\'s disease. Oral glucocorticoid therapy is taken and tapering regularly. The eosinophil count returned to normal, the IgE level gradually decreased, the orbital space-occupying lesion and elbow and groin masses shrank significantly, and the whole-body skin prurigo disappeared. Currently, the patient has been in a stable condition for eighteen months.
CONCLUSIONS: Our case provides a novel insight that Kimura\'s disease should be involved in the differential diagnosis of inflammatory lesion mass of orbit and also supports systemic regular glucocorticoid as a valuable therapy of such condition, but close follow-up and long-term observation are crucial.

Kimura\'s disease (KD) is a rare chronic inflammatory or allergic disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm. Their relationship has always been debated. This article reports two rare cases, one of each disease. One patient was a 48-year-old female that presented with a mass on her right mandible. She also had oedema erythema and wheals on her lower limbs. She was diagnosed with Kimura\'s disease complicated with chronic urticaria. The second patient was a 23-year-old female that presented with multiple nodules of unequal size on the scalp. She was diagnosed with angiolymphoid hyperplasia with eosinophilia. The first patient recovered after being treated with surgical resection, glucocorticosteroids, cyclophosphamide and radiotherapy. The second patient underwent the first stage of surgical excision and is currently being followed-up. Comparison of the clinical and histopathological features of these two cases supports the theory that KD and ALHE are two separate disease entities.

BACKGROUND: Ankylosing spondylitis (AS) and Kimura\'s disease (KD) which is quite rare are both chronic inflammatory diseases. Recently we encountered a patient who suffered from KD and AS, and some of his family members also suffer from AS. We, therefore, investigated this unique case and conducted the family-based whole exome sequencing to explore the possible genetic alterations.
UNASSIGNED: Here, we reported a case of a 44-year-old Chinese man with multiple painless masses all over his body and a back pain for 32 years. His uncle and sister were diagnosed with AS.
METHODS: The diagnosis of KD was based on the patient\'s clinical features and the biopsy of the neck masses. The diagnosis of AS was based on the patient\'s clinical features, HLA-B27(+) and the radiologic changes of sacroiliac joints. The genetic test showed that ARPC1B gene which was associated with recurrent infections, auto-inflammatory changes and elevated IgE levels was mutated in this patient.
METHODS: Neck masses were removed by surgery. Systemic glucocorticoid, nonsteroidal anti-inflammatory agents, combined with cyclosporine were orally administered, and Etanercept was injected subcutaneously.
RESULTS: The masses disappeared rapidly after surgery combined with systemic glucocorticoid, but relapsed shortly after the therapy was discontinued. Low dose glucocorticoid, cyclosporine and Etanercept could keep both KD and AS remained long-term remission.
CONCLUSIONS: Our experience suggests that low dose glucocorticoid, cyclosporine and Etanercept could be beneficial for the patient with KD and AS. The mutation of ARPC1B gene in this case, which is associated with immunologic disturbance, may increase the susceptibility of KD.

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