Cases reported "Mucinoses"

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1/4. abdominal wall ulceration and mucinosis secondary to recombinant human interferon-beta-1b.

    46-year-old woman developed painful ulcers over her lower abdomen in the form of reticulate erythema after injecting interferon beta-1b subcutaneously for multiple sclerosis. skin biopsy revealed multiple superficial thrombosed vessels with focal epidermal necrosis as well as prominent interstitial mucinosis. Treatment with low-molecular-weight heparin followed by a heparinoid resulted in slow healing of the ulcers but also allowed the subcutaneous interferon injections to be continued.
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2/4. Chronic obesity lymphoedematous mucinosis: three cases of pretibial mucinosis in obese patients with pitting oedema.

    Pretibial mucin deposition on the shins is known as pretibial myxoedema. We report three patients with pretibial mucinosis without thyroid disease. The patients were characterized clinically by morbid obesity and bilateral lower extremity pitting oedema with gradual and painless onset, and that did not involve the feet and ankles. Vesicles, semitranslucent papules or a woody plaque were found on the shins. Histologically, patients showed characteristic features of epidermal atrophy with effacement of the rete ridge pattern, separation of collagen bundles associated with oedema with stellate to linear fibroblasts, upward-running increased capillary and small vessels with haemosiderin deposition, and mucin deposition at the superficial papillary dermis and around the vessels. We propose that the present cases of 'chronic obesity lymphoedematous mucinosis' belong to the clinical entity of pretibial mucinosis.
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3/4. Nodular cutaneous lupus mucinosis associated with atrophie blanche-like lesions in a patient with systemic lupus erythematosus.

    BACKGROUND: Multiple, pale, atrophic depressions surrounded by erythema developed on nodular cutaneous mucinosis lesions in a patient with systemic lupus erythematosus. OBSERVATIONS: A 22-year-old man developed multiple nodular erythematous lesions on the upper arms, back, and chest during the course of systemic lupus erythematosus. Histologically, these nodular lesions were diagnosed as lupus mucinosis. Three years later, the patient began to have atrophic depressed lesions on several nodular mucinosis lesions. These lesions did not develop on normal appearing skin. A biopsy specimen from one of these lesions showed occlusion of blood vessels with thickening of their walls and perivascular dense lymphocytic infiltration. No leukocytoclastic vasculitis was found. Hyaline degeneration was present in some parts of the fatty tissue. CONCLUSION: The atrophic depressed lesions were atrophie blanche-like and induced by the vascular occlusion. Since these lesions developed only on the lupus mucinosis lesions, mucin deposition and vascular changes may be closely related.
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4/4. Fatal scleromyxedema: report of a case and review of the literature.

    scleromyxedema is a rare fibromucinous connective tissue that can be associated with systemic changes, such as myopathy, neurologic defects, esophageal dysmotility, paraproteinemia, and restrictive lung disease. We describe a fatal case of scleromyxedema in which neurologic, cardiac, gastrointestinal, and muscle changes were present. At autopsy, mucin was found in the papillary dermis of skin and in coronary and pulmonary vessels, but was absent from the brain, kidneys, heart, gastrointestinal tract, esophagus, liver, thyroid, lymph nodes, bone marrow, and pancreas. Because the pathogenesis of scleromyxedema may not always be attributable to mucin deposition, the role of circulating factors in the development of systemic manifestations warrants further investigation.
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ranking = 1
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