Scleromyxedema, a positive effect with thalidomide

Scleromyxedema is a rare dermatological disorder marked by widespread symmetric 2- to 3-mm, firm, waxy, closely spaced papules involving the hands, forearms, face, neck, upper trunk and thighs. The most common extracutaneous manifestation of scleromyxedema is a benign plasma cell dyscrasia. Treatment of scleromyxedema is limited by the lack of long-term results, toxicity and significant adverse side effects. We report a severe case of scleromyxedema who had marked improvement when treated with thalidomide.     

A case of severe actinic prurigo successfully treated with thalidomide

Actinic prurigo is an uncommon and usually persistent idiopathic photodermatosis with typical human leukocyte antigen (HLA) associations (HLA-DR4, particularly subtypes DRB1*0407 and DRB1*0401). Although its mechanism of action is not clearly understood, thalidomide has been shown to be particularly efficacious in treating actinic prurigo, among other conditions. A 31-year-old Australian woman who had suffered actinic prurigo for most of her life was treated with two courses of thalidomide (50-100 mg nocte) over consecutive summers. Remission was observed after cessation of the second course of thalidomide and had continued 4 years later. Abnormalities in the cutaneous response to ultraviolet radiation at the time of diagnosis, detected by monochromator phototesting, reverted to normal following treatment.     

Scleromyxedema: successful treatment with thalidomide in two patients

BACKGROUND: Scleromyxedema is an unusual skin disease characterized by mucinous infiltration of the skin. There is no satisfactory treatment for this disorder. CASE REPORT: We report 2 cases of scleromyxedema, both associated with an IgG-lambda paraprotein. Treatment with thalidomide was started and great improvement of the lesion was noted as shown by a quality of life questionnaire similar to those used in rheumatoid arthritis. TNF-alpha value was elevated in both cases during this treatment. DISCUSSION: We hypothesize that the efficiency of thalidomide could be mediated by its costimulatory effect on T cytotoxic lymphocytes.     

沙利度胺成功治疗皮肤Rosai-Dorfman病一例

患者,女,53岁。面部暗红色和淡黄色丘疹、结节1年。组织病理示：表皮正常,真皮全层可见致密的组织细胞浸润,伴散在淋巴细胞、浆细胞及中性粒细胞浸润,可见伸入运动。免疫组化染色：S-100(++)、CD68(-)、CD1a(-)。综上诊断为皮肤Rosai-Dorfman病。予沙利度胺治疗,6个月后皮疹基本消退。     

Langerhans cell histiocytosis: an uncommon presentation, successfully treated by thalidomide

Langerhans cell histiocytosis (LCH) is a rare disease and generally affects children under 15 years of age. Adult onset form and cutaneous features at presentation are uncommon. There are some options for treatment of the skin lesions of LCH such as topical and intralesional corticosteroid, nitrogen mustard, etc., which are not completely curative. Herein, we report a case of perianal LCH in a 20-year-old man with one-year history of recalcitrant well-demarcated, erythematous, and ulcerated plaque surrounding the anal orifice, with pain and difficulty in defecation that was successfully treated with thalidomide.     

Rosai‐Dorfman disease successfully treated with thalidomide: A case report

Rosai‐Dorfman disease (RDD) is a rare disease which characterized by proliferation and overproduction of histiocytes in the lymph nodes appearing as lymphadenopathy, however, it may also occur in extranodal sites. The occurrence of unusual manifestations of the disease such as the appearance of the mass in an unusual area may increase the probability of misdiagnosis. Herein, we describe a case of RDD in an old woman with an unusual appearance of RDD in the leg that was successfully treated by thalidomide.     

沙利度胺口服联合糖皮质激素局部注射治疗皮肤型Rosai-Dorfman病一例

根据文献报道皮肤型Rosai-Dorfman病(CRDD)的治疗方法包括抗生素、维A酸类、雷公藤、甲泼尼龙等药物治疗及手术治疗,目前尚无统一的治疗策略,本文报道一例全身多发结节的CRDD患者,其皮损通过沙利度胺口服联合局部注射曲安奈德缓解,随访4个月,患者皮损明显减少。     

Acne treated successfully with azithromycin

AIM: To study the efficacy, safety, and compliance of 500 mg azithromycin thrice weekly for 12 weeks in acne vulgaris. METHODS: An open-label, noncomparative study was carried out for 12 weeks at the outpatient clinics of Aga Khan University Hospital, Abassi Shaheed Hospital, and Burhani Community Hospital in Karachi, Pakistan. Thirty-five adolescent and postadolescent patients with moderate to severe papulopustular acne vulgaris were enrolled. All patients completed the study. Azithromycin, 500 mg orally thrice weekly for 12 weeks, was used. After the baseline visit, patients were scheduled to return at four-weekly intervals for 12 weeks. Efficacy was gauged by the percentage clearance of papulopustular acne lesions. Safety assessments included the monitoring of adverse events, and compliance was checked at the four-weekly regular visits up to 12 weeks. RESULTS: Twenty-nine patients (82.9%) showed remarkable improvement in the first 4 weeks with 60% reduction of their inflammatory papulopustular lesions. Maximum clearance (80%) was observed at 12 weeks. Residual postinflammatory pigmentation and pitted and linear scarring represented the aftermath of the relapsing pattern of acne. Six patients (17.1%) showed slow clearance with eruptions of new lesions. Adverse events, such as heartburn and nausea, were reported by four patients (11.4%). All patients completed the 12-week study period. CONCLUSION: Azithromycin, 500 mg thrice weekly for 12 weeks, is a safe and effective treatment of acne vulgaris with excellent patient compliance.     

Urticaria successfully treated by desensitization with grass pollen extract

We report a case of severe incapacitating seasonal urticaria and angioedema in an 11-year-old boy due to grass pollen sensitivity, who responded well to desensitization therapy with Timothy grass pollen extract.     

Propylthiouracil-induced ANCA-positive erythema nodosum treated with thalidomide

Background Propylthiouracil (PTU), one of the mainstays of antithyroid therapy drugs, can lead to antineutrophil cytoplasmic antibody (ANCA) positivity and skin lesions. PTU‐induced ANCA‐positive vasculitis is rare and even more rare is erythema nodosum. Objective To report a case of a 57‐year‐old woman with hyperthyroidism who developed myeloperoxidase (MPO)‐ANCA erythema nodosum after PTU treatment for 11 months. Methods Skin biopsy demonstrated septal panniculitis without vasculitis. PTU‐induced ANCA‐positive erythema nodosum was made. Results With discontinuation of PTU and initiation of thalidomide, skin lesions resolved completely in three weeks, and after three months, the titers of MPO‐ANCA and perinuclear‐ANCA (p‐ANCA) had decreased remarkably. At 14‐month follow‐up, the patient was asymptomatic, but low levels of ANCA titers persisted. Conclusions This report indicated that ANCA positive erythema nodosum could develop following PTU treatment. Thalidomide has been proven to be helpful and averted the adverse effects from systemic corticosteroids and other immunosuppressive drugs in this patient.