tacrolimus and Angiolymphoid-Hyperplasia-with-Eosinophilia

The association between membranous nephropathy (MN) and Kimura's disease (KD) has been reported in recent years. The treatment, effect, and prognosis of KD are still unclear.. A 47-year-old KD patient developed a left axillary mass for 3 years and received surgical resection because of the lager mass in August 2016. Then he developed nephrotic syndrome 3 months later. Laboratory index revealed increased eosinophil count, decreased albumin and heavy proteinuria. Lymph node biopsy suggested KD, and renal biopsy suggested MN. He relapsed after a treatment with methylprednisolone (52 mg/d) alone and then tacrolimus (1.5 mg/12h) was added. The patient had no symptoms of relapse in the next 2 years.. The combination therapy of surgery, methylprednisolone, and immunosuppressive agents may be effective in KD with MN.

Topics: Angiolymphoid Hyperplasia with Eosinophilia; Glomerulonephritis, Membranous; Humans; Kimura Disease; Male; Middle Aged; Nephrotic Syndrome; Tacrolimus

Angiolymphoid hyperplasia with eosinophilia is a benign neoplasm that includes blood vessel proliferation and a dense eosinophilic inflammatory infiltrate. Mostly, it affects middle-aged adults manifesting as flesh/plum-colored pruritic nodules and papules, most commonly affecting the ear and the periauricular area.. In this case, we report a 13-year-old Caucasian girl with bilateral, huge, protruding, and yellowish nostril masses which were peculiar in location and of gross appearance. At first, the disease proved to be a diagnostic dilemma. After making a diagnosis of angiolymphoid hyperplasia with eosinophilia, the disease also proved to be a therapeutic dilemma. It did not respond to oral prednisolone or to oral indomethacin, and it proved to be resistant to topical steroids. Although surgery is the standard therapeutic approach, it recurred despite multiple surgical attempts. However, the only regimen that seemed to partially control the lesion was intralesional steroids combined with topical tacrolimus ointment.. Angiolymphoid hyperplasia with eosinophilia proves a therapeutic dilemma, because there is a large variety of proposed treatments, yet there is not enough data on most of them. Although the disease is not deadly by itself, it usually presents with disfiguring lesions that grimly affect the patient's quality of life. This warrants further research and efforts to find an effective cure and a unified therapeutic approach.

Topics: Administration, Topical; Adolescent; Angiolymphoid Hyperplasia with Eosinophilia; Cost of Illness; Female; Humans; Immunosuppressive Agents; Injections, Intralesional; Nose Deformities, Acquired; Nose Diseases; Ointments; Prednisolone; Quality of Life; Tacrolimus; Treatment Outcome

Topics: Angiolymphoid Hyperplasia with Eosinophilia; Humans; Immunosuppressive Agents; Male; Middle Aged; Remission Induction; Tacrolimus

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, benign condition characterized by multiple benign angiomatous nodules or plaques. Cutaneous lesions can be painful, pruritic, pulsatile, or potentially disfiguring resulting in significant morbidity. ALHE is a pathologic diagnosis featuring proliferations of capillary-sized vessels with epithelioid endothelial cells surrounded by larger, thick-walled vessels and accompanying eosinophils and lymphocytes. Surgery is generally required, however the skin lesions often recur after excision. ALHE is notoriously difficult to treat and many physicians would prefer a non-invasive treatment of choice. We report a case of ALHE that was successfully treated with the novel use of topical tacrolimus in a split-face trial with topical timolol solution.

Topics: Administration, Cutaneous; Adrenergic beta-Antagonists; Aged, 80 and over; Angiolymphoid Hyperplasia with Eosinophilia; Follow-Up Studies; Humans; Immunosuppressive Agents; Male; Pharmaceutical Solutions; Tacrolimus; Timolol; Treatment Outcome

Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment.. Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively.. The patients consisted of 27 males and 2 females, with an average age of 35.5 ± 15.1 (13 - 61) years. 27 exhibited proteinuria ranging from 0.730 to 14.1 g/24 h (5.98 ± 3.40 g/24 h). Hypertension, renal insufficiency (serum creatinine (Scr) > 1.24 mg/dL), and microhematuria occurred in 4 (13.8%), 11 (37.9%), and 13 (44.8%) cases, respectively. Light microscopy (LM) identified mesangium proliferation, minimal change, focal and segmental glomerulosclerosis (FSGS), membranous glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), and acute tubular necrosis in 14, 8, 3, 2, 1, and 1 cases, respectively. All were treated with Tripterygium wilfordii (TW), prednisone, leflunomide (LEF), tacrolimus (FK506), myophenolate mofetil (MMF), or renin-angiotensin system blockers (RASI). 26 patients were followed up for 1.60 - 108.7 months (39.6 ± 28.7). After treatments, urinary red blood cells (RBC) decreased in all. The amount of 24-hour urinary protein (24-hUPE) decreased in 24 patients. 22 reached complete remission (CR), 4 partial remissions (PR). The patients who did not relapse were younger than those who relapsed.. KD with renal involvement occurs predominantly among 35 - 50 year old Chinese patients with male predilection. The most common features are proteinuria, hypertension, micro hematuria with minimal change, and mesangial proliferative glomerulonephritis. Most were responsive to treatment, but could relapse. Gender, age, and hypertension are associated with KD recurrence. The prognosis is good mostly.

Topics: Adolescent; Adult; Angiolymphoid Hyperplasia with Eosinophilia; Anti-Inflammatory Agents; China; Female; Glomerulonephritis; Glomerulonephritis, Membranoproliferative; Glomerulonephritis, Membranous; Glomerulosclerosis, Focal Segmental; Hematuria; Humans; Hypertension; Immunosuppressive Agents; Isoxazoles; Leflunomide; Male; Middle Aged; Mycophenolic Acid; Phytotherapy; Plant Preparations; Prednisone; Prognosis; Proteinuria; Recurrence; Renal Insufficiency; Retrospective Studies; Tacrolimus; Tripterygium; Young Adult

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare disease, currently considered a benign vascular proliferation of unknown etiology, and whose treatment is still unclear and challenging.. Two women in their thirties consulted for itchy lesions of the right ear. Both presented with a reddish bleeding papulonodular infiltration of the auricle, with a nodule at the entrance to the external auditory canal in the first patient. Laboratory tests showed no abnormalities and in particular no hypereosinophilia or elevated serum immunoglobulin E. In both cases, histology of lesional skin showed vascular proliferation with thick-walled vessels lined by plump endothelial cells, protruding into the lumen, together with a mixed dermal inflammatory infiltrate consisting primarily of eosinophils and lymphocytes. A diagnosis of ALHE was made in both patients based on clinical and histological features. MRA revealed no underlying vascular malformation in both cases. Patients started treatment with 0.1% tacrolimus ointment twice daily. The pruritic sensation and bleeding had completely subsided within two weeks and the reddish infiltration and nodules had practically disappeared after two months of topical tacrolimus. Continuous application resulted in no recurrence at 6 months of follow-up.. Treatment of ALHE is still poorly standardized due to doubts concerning the pathophysiology of this rare condition and the small number of available studies. Topical tacrolimus was originally developed for the treatment of moderate to severe atopic dermatitis because of its anti-inflammatory and immunomodulatory properties. Recent studies suggest that this drug may be effective in treating other forms of inflammatory dermatosis. Our two observations suggest that tacrolimus ointment also represents potentially valuable treatment in AHLE.

Topics: Administration, Topical; Adult; Angiolymphoid Hyperplasia with Eosinophilia; Female; Humans; Immunosuppressive Agents; Pruritus; Tacrolimus

We report preliminary results of an ongoing study that assesses the efficacy of tacrolimus on Kimura's disease (KD).. A patient with refractory KD after surgery and treatment with prednisone was treated with tacrolimus. Tacrolimus (FK-506) was administered at an initial dosage of 1 mg every 12 hours, and FK-506 concentration in the blood was monitored monthly. FK-506 blood concentration was controlled within 5 to 15 μg/L. After 6 months, the dosage of tacrolimus was reduced to 0.5 mg daily for another 2 months and then treatment was stopped.. Swelling of the bilateral salivary glands disappeared within the first week. No serious side effects were noted and the disease has not recurred in the 2 years of follow-up.. Tacrolimus may be an effective treatment for patients with KD, but more research is needed to determine its long-term efficacy and safety as well as its mechanism of action.

Topics: Adult; Angiolymphoid Hyperplasia with Eosinophilia; Combined Modality Therapy; Glucocorticoids; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Prednisone; Tacrolimus

Topics: Adult; Angiolymphoid Hyperplasia with Eosinophilia; Humans; Immunosuppressive Agents; Male; Ointments; Tacrolimus; Treatment Outcome

Topics: Adult; Angiolymphoid Hyperplasia with Eosinophilia; Ear Diseases; Ear, External; Female; Humans; Immunosuppressive Agents; Ointments; Tacrolimus