Suspected cardiac toxicity to intravenous immunoglobulin used for treatment of scleromyxedema.

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.

A study of oxidative stress in paucibacillary and multibacillary leprosy.

BACKGROUND: The study and assessment of oxidative stress plays a significant role in the arena of leprosy treatment.Once the presence of oxidative stress is proved, antioxidant supplements can be provided to reduce tissue injury and deformity. AIM: To study oxidative stress in paucibacillary (PB) and multibacillary (MB) leprosy and to compare it with that in a control group. METHODS: Fifty-eight untreated leprosy patients (23 PB and 35 MB cases) were studied and compared with 58 healthy controls. Superoxide dismutase (SOD) level as a measure of antioxidant status; malondialdehyde (MDA)level, an indicator of lipid peroxidation; and MDA/SOD ratio, an index of oxidative stress were estimated in the serum.RESULTS: The SOD level was decreased in leprosy patients, especially in MB leprosy. The MDA level was increased in PB and MB leprosy. The MDA/SOD ratio was significantly elevated in MB patients. There was a steady increase in this ratio along the spectrum from tuberculoid to lepromatous leprosy (LL). CONCLUSION: There is increased oxidative stress in MB leprosy, especially in LL. This warrants antioxidant supplements to prevent tissue injury.

Moore Federman syndrome: A rare cause of pseudoscleroderma.

The Moore Federman syndrome (MFS) is characterized by short stature, stiffness of the joints, characteristic facies and ocular abnormalities. Herein, we report the case of a 45 year-old lady with short stature, thickening of the skin, stiffness of the joints, typical facies, iridodonesis and cataract since the age of 12 years. She had short digits and no family history of similar illness. To the best of our knowledge, this could be the fourth report of MFS.

Lymphangitic chromoblastomycosis.

Chromoblastomycosis (CM), a chronic subcutaneous mycosis, is caused by several dematiaceous fungi, the most common being Fonsecaea pedrosoi. It usually occurs in the lower extremities following traumatic implantation of the organisms. We are reporting a case of chromoblastomycosis on the right lower limb in a sporotrichoid pattern caused by F. pedrosoi. The pattern was probably due to lymphatic spread that seems to be one of the rare presentations. The histopathology showed typical muriform or medlar bodies both intracellularly and extracellularly within the granuloma. Culture revealed sporulating organisms (Cladosporium and Rhinocladiella type) by a combination method, characteristic of F. pedrosoi. Our case responded well to itraconazole.

Cerebrotendinous xanthomatosis: need for early diagnosis.

Cerebrotendinous xanthomatosis is a rare autosomal recessive lipid storage disease characterized by widespread tissue deposition of two neutral sterols, cholestanol and cholesterol, resulting in tendinous xanthomas, juvenile cataracts, progressive neurological defects and premature death from arteriosclerosis. The primary biochemical defect is deficiency of hepatic mitochondrial enzyme sterol-27-hydroxylase which catalyses the hydroxylation of cholestanol (5-alpha dehydro derivative of cholesterol) and this deficiency decreases bile acid synthesis. Substantial elevation of serum cholestanol and urinary bile alcohols with low to normal plasma cholesterol concentration establishes the diagnosis. Cerebrotendinous xanthomatosis is exceptionally rare in the Indian population. We are reporting a woman with this rare disorder, who was on antiepileptic and antipsychotic drugs for a prolonged period and whose original condition went undiagnosed. She presented with xanthomas on the Achilles tendons and the upper end of tibia. She was mentally subnormal and her serum cholestanol level was raised. Her younger sister too was severely affected by this disorder. Early treatment with chenodeoxycholic acid is known to prevent disease progression.

Primary cutaneous multifocal CD30+ anaplastic large cell lymphoma.

A 45-year-old male presented with asymptomatic tumors all over the body. The tumors showed no signs of ulceration or regression. There were generalized, nontender, firm to hard enlarged lymph nodes without hepatosplenomegaly. Biopsy and immunophenotyping revealed CD 30+ anaplastic primary cutaneous large cell lymphoma. Primary cutaneous anaplastic large cell lymphoma is characterized by single or grouped reddish-brown tumor nodules, which frequently tend to ulcerate. Secondary involvement of lymph nodes is seen in only 25%. The lesions responded dramatically to chemotherapy, but recurred.