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Journals and Conferences
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… (More)
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe adverse drug reaction that can affect any age group. We carried out a prospective study of the clinicoepidemiologic aspects of DRESS in children. We prospectively studied all patients ages 12 years and younger admitted to the departments of pediatrics and dermatology at a tertiary… (More)
Hereditary sensory and autonomic neuropathy (HSAN) is a group of rare hereditary diseases. A young female with Type I HSAN with indeterminate Hansen's disease (HD) is reported which is a rare association.
Toxic epidermal necrolysis (TEN) is an acute life-threatening blistering disease characterized by involvement of the skin, multiple mucous membranes and internal organs. It is most commonly precipitated by the administration of medications like anticonvulsants. Neuroleptic malignant syndrome (NMS) is a rare complication of neuroleptic therapy characterized… (More)
OBJECTIVES 1. To study and compare the clinical and histological features of Type 1 and Type 2 lepra reactions. 2. To document the histological patterns of Type 1 and Type 2 lepra reactions observed in the study population. DESIGN Two year cross sectional study. Patients attending the outpatient department of our tertiary care hospital, during the 2 year… (More)
Hyper IgE syndrome with recurrent infection (Job's syndrome) is a rare idiopathic primary immunodeficiency disease characterized by the triad of elevated serum IgE (>2000 IU/ml), recurrent cutaneous abscesses and recurrent sinopulmonary infections. The bacteria which commonly infect these patients are Staphylococcus aureus and Haemophilus influenzae.… (More)
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… (More)
Tuberous sclerosis complex (TSC) and hypomelanosis of Ito (HI) are two uncommon neurocutaneous syndromes and their coexistence is extremely rare. An epileptic child presented with progressively increasing multiple hypopigmented macules arranged in a linear and whorled pattern along the lines of Blaschko over the trunk and limbs, characteristic of HI. He… (More)
A 30-year-old male who presented with indolent leg and foot ulcers, on investigation was found to have Klinefelter's syndrome associated with straight back syndrome.