Scleromyxedema
End-stage disease of native kidneys in a patient with biopsy-proven Arndt-Gottron scleromyxoedema and recurrence in the transplanted kidney. (1/10)
End-stage renal disease-stage 5 chronic kidney disease (CKD)-of the native kidneys, related to biopsy-proven Arndt-Gottron scleromyxoedema, developed in a male patient. From 1998 until 2001, the patient was treated by haemodialysis. In June 2001, cadaveric kidney transplantation was performed. In January 2004, a kidney biopsy was performed because of deteriorating renal function revealing relapse of scleromyxoedema with typical concentric narrowing of the arterioles due to accumulation of mucopolysaccharides with severe glomerular ischaemia. Arndt-Gottron scleromyxoedema is an as yet unsuspected cause of stage 5 CKD of the native kidneys. Moreover, the disease can relapse in the transplanted kidney, again leading to intractable transplant stage 5 CKD. (+info)Scleroderma-like fibrosing disorders. (2/10)
(+info)Suspected cardiac toxicity to intravenous immunoglobulin used for treatment of scleromyxedema. (3/10)
Scleromyxedema is a rare, generalized form of lichen myxedematosus, which may be associated with systemic involvement and can be fatal. The therapeutic options available provide partial or inconsistent response and are associated with significant adverse effects. We report a case of scleromyxedema with cardiac involvement, treated with low-dose intravenous immunoglobulin, with almost complete clearing of the skin lesions. The patient died after three cycles of treatment, possibly due to myocardial infarction. (+info)Folliculitis, follicular mucinosis, and papular mucinosis as a presentation of chronic myelomonocytic leukemia. (4/10)
Leukemias and Lymphomas can present in indolent and surprisingly unusual manners. Although uncommon, follicular lesions such as eosinophilic folliculitis have been reported in association with leukemia. However, follicular and papular mucinosis are novel associations for chronic myelomonocytic leukemia. (+info)Vincristine, idarubicin, dexamethasone and thalidomide in scleromyxoedema. (5/10)
(+info)Scleromyxedema-like syndrome with systemic involvement in a cat. (6/10)
(+info)Generalized papular and sclerodermoid eruption: scleromyxedema. (7/10)
(+info)Managing scleromyxedema with intravenous immunoglobulin: acute worsening of scleromyxedema with biclonal gammopathy. (8/10)
Scleromyxedema is a rare chronic cutaneous mucinosis usually associated with a monoclonal gammopathy and underlying systemic disease. The etiology of the disease is not known. There are no standard treatments and response to various therapeutic modalities varies. We report a case of refractory scleromyxedema in a 63-year-old man with a biclonal IgG and IgM lambda-gammopathy. The patient was successfully managed with intravenous immunoglobulin. (+info)Scleromyxedema is a rare, progressive skin disorder characterized by the thickening and hardening of the skin due to excessive deposits of proteins called mucin and abnormal fibrous tissue in the dermis. It is also associated with monoclonal gammopathy, which means there is an overproduction of a specific type of antibody in the blood. The condition can affect various organs and systems in the body, potentially leading to systemic involvement.
The primary clinical features of scleromyxedema include:
- Widespread symmetric papules (small bumps) and plaques (thickened patches) on the skin
- Progressive thickening and hardening of the skin, particularly on the face, neck, hands, and feet
- Fine, waxy, "paradoxical" wrinkling of the skin when stretched or manipulated
- Occasionally, involvement of mucous membranes (e.g., mouth, eyes)
Scleromyxedema is differentiated from another similar condition called lichen myxedematosus by its association with monoclonal gammopathy and systemic symptoms. The exact cause of scleromyxedema remains unclear, but it is believed to involve abnormal immune system activity.
Treatment for scleromyxedema can be challenging due to the rarity and complexity of the condition. Various therapies have been used with varying degrees of success, including intravenous immunoglobulin (IVIG), glucocorticoids, chemotherapy agents, and biologics. The prognosis for scleromyxedema is variable, with some patients experiencing significant improvement or stabilization, while others may have progressive disease that can lead to severe complications and even death.