Calciphylaxis is a rare disease histopathologically characterized by calcification of the medial layer of small cutaneous vessels, resulting in painful and intractable skin ulcerations in the legs. The disease is potentially lifethreatening, with more than 50% of patients dying within 1 year of diagnosis 1). Secondary infections and organ failure are also possible complications. Patients with end-stage renal disease are most susceptible to calciphylaxis; however, calciphylaxis may also affect patients with hypoparathyroidism requiring calcium salts or vitamin D, those with obesity, and those using warfarin. Ever since Cicone et al. 2) reported the successful treatment of calciphylaxis with intravenous sodium thiosulfate (STS), STS therapy has been commonly used as the first-line treatment for this disease, showing beneficial effects in most patients 3). However, systemic STS may have various side effects, such as nausea, vomiting, hypocalcemia, metabolic acidosis, and QTinterval prolongation. According to several recent reports, intralesional STS may prevent systemic side effects, but it may cause pain during injection. Although the use of STS has been described in case reports, case series, and in a single retrospective review, the effects and side effects of STS on calciphylaxis have not been confirmed by blinded randomized clinical studies. Therefore, a standardized treatment protocol for STS has not been
Systemic sclerosis (SSc) or scleroderma is an autoimmune disorder characterized by tissue fibrosis of the skin and internal organs. The etiology of the skin fibrosis is thought to be thickened dermis due to uncontrolled excessive deposition of various extracellular matrix, mainly type I collagen.Systemic treatments with anti-inflammatory and cytotoxic immunosuppressive properties, such as corticosteroids and immunosuppressants, are usually considered for skin sclerosis of patients with SSc. However, their approach must be initiated at the early stage, before the fibrosis is completed, and the effects of the corticosteroids and immunosuppressants are known to be reduced in the late stages of the sclerosis. Furthermore, various significant adverse effects of these treatments must be considered.This paper discusses the present day understanding of therapeutic options using disease-modifying drugs against skin sclerosis of SSc patients and the possible mechanisms.
A 74-year-old woman noticed an edematous erythema on the right upper eyelid two months before her first medical examination, and slight fever, arthralgia and edematous erythema on the forearms and palms around the time of the first medical examination. She presented with typical skin lesions of dermatomyositis including Gottron's signs and a heliotrope rash without any abnormal muscle symptoms. An examination by gastrointestinal endoscopy and a computed tomography scan of the chest revealed that she was complicated with stomach cancer (Stage I A), colon cancer (Stage I) and interstitial pneumonia (IP). She was diagnosed with anti-MDA5 antibody-positive clinically amyopathic dermatomyositis (CADM) complicated with two cancers. Because the IP became aggravated, she was treated with corticosteroids at an initial dose of 1 mg/kg/day and immunosuppressive therapies. Tacrolimus was discontinued due to thrombocytopenia, and she also had an allergic reaction to cyclophosphamide. The administration of azathioprine at a dose of 75 mg/day prevented the exacerbation of IP. We were able to taper the dose of corticosteroids, and endoscopic stomach surgery and abdominal rectal surgery was performed. The anti-MDA5 antibody is a characteristic myositis-specific autoantibody associated with CADM and IP, which may cause a poor prognosis. CADM complicated with malignancy occurs less frequently than similarly complicated dermatomyositis and, to the best of our knowledge, this is the first case of CADM complicated with two cancers.
Systemic sclerosis (SSc) or scleroderma is an autoimmune disorder characterized by tissue fibrosis of the skin and internal organs. The etiology of the skin fibrosis is thought to be thickened dermis due to uncontrolled excessive deposition of various extracellular matrix, mainly type I collagen.Systemic treatments with anti-inflammatory and cytotoxic immunosuppressive properties, such as corticosteroids and immunosuppressants, are usually considered for skin sclerosis of patients with SSc. However, their approach must be initiated at the early stage, before the fibrosis is completed, and the effects of the corticosteroids and immunosuppressants are known to be reduced in the late stages of the sclerosis. Furthermore, various significant adverse effects of these treatments must be considered.This paper discusses the present day understanding of therapeutic options using disease-modifying drugs against skin sclerosis of SSc patients and the possible mechanisms.
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