Literature DB >> 31848525

[Dermatomyositis combined with IgA vasculitis: A case report].

J Xu2, J Xu2, H Li3, J Tang4, J L Shu4, J Zhang1, L J Shi1, S G Li1.   

Abstract

Dermatomyositis (DM) is an autoimmune disease characterized by muscle involvement of the proximal extremities and specific skin involvement, like Gottron sign and heliotrope rash. HenochSchonlein purpura (IgA vasculitis) nephritis is characterized by hematuria and/or proteinuria clinically, with histologic evidence of IgA nephropathy, and also can be clinically characterized by non-thrombocytopenic purpura, presenting with petechiae and ecchymosis on the skin and mucous membranes, often involving multiple organs and systems, accompanied by abdominal pain, joint swelling and pain, and renal lesions. We reported here a patient with symmetric muscle weakness in her proximal limbs and typical Gottron sign, whose laboratory examination showed elevated creatine kinase (CK) level and myogenic damage electromyographically, which were concomitant with dermatomyositis. We applied prednisone combined with cyclophosphamide, and the patient's muscle strength, interstitial lung disease and all improved gradually. The patient gradually developed severe hepatic damage [significantly increased glutamic-pyruvic transaminase (ALT), glutamic oxalacetic transaminase (AST) and bilirubin], high fever (body temperature fluctuated between 38.0-39.2 °C), thrombocytopenia (limb distal purplish rash, some slightly protruded from the skin surface, some fused into a piece, which did not fade with pressure) and intractable diarrhea (waterlike stool, antidiarrheal drug treatment was not good), with new onset of the skin lesions on multiple areas of her body, as well as abrupt occurrence of massive proteinuria, which resulted in huge challenges in the following diagnosis and treatment. After extensive differential diagnosis from various directions, including pathological biopsies, it finally came out to be dermatomyositis combined with IgA vasculitis, which had been rarely reported. Both cellmediated immunity to muscle antigens and immune-complex disease might participate in the pathogenesis. There was evidence that they were immune complex diseases. Several immune mechanisms played an important role in the pathogenesis of both DM and IgA vasculitis. We conducted a substantial literature review of the above diseases. The purpose of our study is to strengthen the clinical understanding of such complicated diseases, and to highlight the importance of pathological biopsy in the diagnosis (renal biopsy pathology gave us a definite diagnosis). And what is more important is that seizing the opportunity to initiate treatment can control the disease and improve the prognosis.

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Year:  2019        PMID: 31848525      PMCID: PMC7433583     

Source DB:  PubMed          Journal:  Beijing Da Xue Xue Bao Yi Xue Ban        ISSN: 1671-167X


  18 in total

1.  Cholestasis in juvenile dermatomyositis: report of three cases.

Authors:  R A Russo; M M Katsicas; M Dávila; M Ciocca; M Zelazko
Journal:  Arthritis Rheum       Date:  2001-05

2.  Perforation of the bowel in Henoch-Schonlein purpura.

Authors:  R BASU
Journal:  Arch Dis Child       Date:  1959-08       Impact factor: 3.791

3.  Children with Henoch-Schönlein purpura with low complement levels: follow-up for >6 years.

Authors:  Qiang Lin; Xiaozhong Li
Journal:  Pediatr Nephrol       Date:  2017-03-29       Impact factor: 3.714

4.  Histologic Features of Gastrointestinal Tract Biopsies in IgA Vasculitis (Henoch-Schönlein Purpura).

Authors:  Christine Y Louie; Adam J Gomez; Richard K Sibley; Dorsey Bass; Teri A Longacre
Journal:  Am J Surg Pathol       Date:  2018-04       Impact factor: 6.394

5.  Serum complement components in Henoch-Schönlein purpura.

Authors:  M Garcia-Fuentes; A Martin; C Chantler; D G Williams
Journal:  Arch Dis Child       Date:  1978-05       Impact factor: 3.791

6.  Henoch-Schonlein purpura with hypocomplementemia in children.

Authors:  Osamu Motoyama; Kikuo Iitaka
Journal:  Pediatr Int       Date:  2005-02       Impact factor: 1.524

7.  The Clinicopathological Characteristics of Henoch-Schönlein Purpura Nephritis with Presentation of Nephrotic Syndrome.

Authors:  Jiaxing Tan; Yi Tang; Yicong Xu; Siyu Yan; Yuanyuan Xu; Li Tan; Zhengxia Zhong; Padamata Tarun; Wei Qin
Journal:  Kidney Blood Press Res       Date:  2019-08-06       Impact factor: 2.687

8.  Abrupt and durable remission of Henoch-Schönlein purpura nephritis with cyclosporine A.

Authors:  Eleni Georgaki-Angelaki; Stavroula Kostaridou; Athanasia Lourida; C Petraki; Evagelia Lagona
Journal:  NDT Plus       Date:  2008-08-01

9.  Endoscopic and microscopic findings of gastrointestinal tract in Henoch-Schönlein purpura: Single institute experience with review of literature.

Authors:  Yeeun Han; So-Young Jin; Dong Won Kim; Yoon Mi Jeen; Yon Hee Kim; In Ho Choi
Journal:  Medicine (Baltimore)       Date:  2019-05       Impact factor: 1.817

10.  Clinical and Pathological Characteristics of Elderly Japanese Patients with IgA Vasculitis with Nephritis: A Case Series.

Authors:  Hiroyuki Ueda; Yoichi Miyazaki; Nobuo Tsuboi; Keita Hirano; Shinya Yokote; Emi Kobayashi; Makoto Ogura; Tetsuya Kawamura; Munekazu Ryuzaki; Takashi Yokoo
Journal:  Intern Med       Date:  2018-08-10       Impact factor: 1.271

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