Clinical features of 24 cases of scleroderma-like cutaneous graft-versus-host disease / 中华皮肤科杂志

Objective:To investigate clinical features of and risk factors for scleroderma-like cutaneous graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation.Methods:Clinical data were collected from 24 patients with scleroderma-like cutaneous GVHD in Department of Dermatology, Peking University People′s Hospital from 2014 to 2019. Clinical features, treatment, prognosis, and possible factors influencing the development of scleroderma-like cutaneous GVHD were analyzed retrospectively.Results:Among the 24 patients, 11 were males, and 13 were females, aged 33 ± 12 years; 20 were human leukocyte antigen (HLA) -identical recipients, 4 were HLA-haploidentical recipients; GVHD occurred 18.5 (8.0, 30.9) months after transplantation. Nineteen patients had discontinued anti-rejection therapy or received low-dose anti-rejection drugs before the onset of GVHD. Fifteen patients presented with generalized scleroderma-like lesions, 1 with linear scleroderma-like lesions, 5 with morphea-like lesions, and 3 with fasciitis-like lesions. None of the 15 patients with generalized scleroderma-like GVHD had Raynaud syndrome. Thirteen patients were accompanied by graft rejection in other systems, 8 had joint mobility limitations, and 1 developed cutaneous squamous cell carcinoma secondary to chronic skin ulcers. All patients were treated with systemic glucocorticoids and immunosuppressive agents, and 11 also with topical glucocorticoids. An intensive follow-up was carried out in 11 patients, of whom 3 achieved marked improvement, 4 achieved improvement, 2 experienced exacerbation, and 2 died. A total of 223 patients with non-sclerodermatous cutaneous GVHD admitting during the same period served as controls, and the proportion of HLA-identical patients was significantly higher in the scleroderma-like cutaneous GVHD group (20/24, 83.3%) than in the non-sclerodermatous cutaneous GVHD group (47/223, 21.1%; P < 0.001) . Conclusions:Scleroderma-like cutaneous GVHD commonly occurs late, and can mimic clinical manifestations of all 4 types of spontaneous scleroderma. HLA-identical transplants, premature discontinuation or excessive dose reduction of anti-rejection drugs may be risk factors for scleroderma-like cutaneous GVHD.

A Case of Scleroderma-like Cutaneous Lesions Induced by Docetaxel in a Patient with Breast Cancer

Docetaxel, an anti-microtubule agent, has been reported to show cytotoxic effects in solid tumors. Its toxicities also include neutropenia, alopecia, skin reaction, and fluid retention. In this study, we report on a case of a 57-year-old Korean female who presented with rapidly progressive scleroderma-like cutaneous changes in the upper and lower extremities after administration of docetaxel. Results of the following tests were normal or negative: full blood count, serum urea, creatinine, electrolytes, liver function test, thyroid function test, rheumatoid factor, anti-nuclear antibody, and anti-topoisomerase antibody. No structural abnormalities were noted on esophagogastroduodenoscopy, chest computed tomography, and Doppler ultrasonography. A biopsy of skin from the left calf showed dermal sclerosis. There was no other explanation for the lesion, except a scleroderma-like cutaneous change induced by docetaxel in this Korean female undergoing treatment for breast cancer.

Esclerodermia localizada: Diagnósticos diferenciales / Localized scleroderma: Differential diagnosis

La esclerodermia localizada constituye un desorden autoinmune órgano específico, que compromete sobre todo la piel. Se caracteriza por inflamación seguida de esclerosis e incluye distintas formas clínicas. La etiología de la esclerodermia localizada no ha sido establecida. El diagnóstico diferencial incluye cuadros esclerodermiformes, desencadenados por factores intrínsecos y extrínsecos que están siendo estudiados. Entre ellos se incluyen: exposición a radiaciones o tóxicos, consumo de medicamentos, infecciones y enfermedades de origen endocrino-metabólico, genético e inflamatorio. En primer lugar, se debe descartar la esclerodermia sistémica. En segundo lugar, se clasifican las entidades con predominio de esclerosis o atrofia. Por último, se incluyen algunas enfermedades en un cuadro comparativo.

Localized scleroderma is an autoimmune organ specific disorder with an important skin compromise. It is an inflammatory process with several distinct clinical characteristics. The etiology of localized scleroderma has not been established yet. Differential diagnosis includes sclerodermiform onset unchained by intrinsic and extrinsic factors that are presently studied. Among them must be taking into account: exposure to radiation or toxic agents, therapeutic drugs, infections and diseases of endocrine, metabolic, genetic or inflammatory etiology. Firstly, it must be point out that systemic scleroderma must be ruled out. Secondly, disorders predominantly with sclerosis and atrophy must be classified and lastly, some other diseases are included in a comparative table.

Eosinophilic fasciitis: A case report and literature review.

Eosinophilic fasciitis (EF) is a rare scleroderma-like syndrome. There has been no previous published report of EF in a Thai patient. We described a 41 year-old Thai man who presented with symmetric induration of the skin of forearms, arms, hands, fingers, lower aspects of the legs, and feet. Physical examination revealed bilateral symmetrical woody induration of the skin with peau d’orange appearance. A groove sign was positive on the flexural surface of both arms. Laboratory testing revealed a peripheral eosinophil count of 54%. The skin and superficial fascia biopsy specimen from the inner aspect of the left forearm was consistent with EF. He was treated with prednisolone, methotrexate, and colchicine. He experienced a gradual improvement within 4 months. A history of acute onset of scleroderma-like syndrome and careful physical examination can lead us to the diagnosis of EF.

Localized Scleroderma-like Reaction Induced by Doxifluridine / 대한피부과학회지

Exogenous factors, including environmental substances and drugs, are known to induce scleroderma-like reactions. Various scleroderma-like reactions induced by anti-cancer drugs have recently been reported. This is the first report that doxifluridine (Didox), an oral prodrug of the antineoplastic agent 5-fluorouracil (5-FU), induced a localized sclerderma-like reaction. A 51-year-old woman was referred to our clinic with multiple pearly, shiny patches on both her breasts, her pelvis and her back. After surgical excision and radiation therapy due to her right breast cancer, she took Didox for 7 months. A skin biopsy specimen revealed that the dermal collagen thickening extended even to the subcutaneous tissue. The routine laboratory tests were within the normal ranges and the tests for antinuclear antibody (ANA), anti SS-A antibody, anti SS-B antibody and anti U1-RNP antibody were all negative. After discontinuation of Didox, the lesions gradually improved. Based on these finding, we diagnosed this case as a localized scleroderma-like reaction induced by doxifluridine and we should pay attention to detect this adverse effect of the long term use of doxifluridine.

A Case of Localized Scleroderma-like Lesion Following Extravasation of Oxaliplatin / 대한피부과학회지

While extravasation from intravenous lines is common and usually benign, leakage of certain cytotoxic drugs can cause severe tissue injury varying from irritation to necrosis. The severity of tissue injury is dependent on the type and concentration of the chemotherapeutic agent and the quantity injected. Oxaliplatin is a novel class of platinum chemotherapeutic agent used in refractory adenocarcinoma. With increased use of oxaliplatin as a potent anticancer drug, there has been increasing numbers of irreversible local tissue reactions at venous administration sites. This report describes a case of the rapid onset of scleroderma-like lesion following a single episode of oxaliplatin extravasation from an implanted chemo-port in a 72-year-old patient with metastatic gastric cancer.