Encephalocraniocutaneous lipomatosis: a rare neurocutaneous syndrome.

Encephalocraniocutaneous lipomatosis is a congenital hamartomatous disorder with unique ocular, cutaneous and neurological features. A 13-year-old boy presented with history of mental retardation and delayed developmental milestones. Bulbar conjunctiva of left eye showed hypertrophy with a soft reddish limbal nodule encroaching on the cornea. Dermatological examination showed multiple patches of alopecia, soft papules in the left perioral and periorbital areas, soft masses over the right axilla, trunk and in the lumbosacral region suggestive of lipomas. The CT scan of the brain revealed well-defined, hypodense lesions in both the cerebellar hemispheres suggestive of lipomas. The constellation of these findings led us to a diagnosis of encephalocraniocutaneous lipomatosis.

Progressive systemic sclerosis in childhood: a report of three cases.

Systemic sclerosis is unusual in childhood. We describe three children who presented with diffuse hidebound skin associated with gastrointestinal and pulmonary abnormalities. Cardiac and renal dysfunctions, which are often encountered in these patients, were notably absent in our cases.

Treatment of pemphigus with intravenous pulse cyclophosphamide.

BACKGROUND: The complications associated with the use of systemic corticosteroid therapy have prompted a search for alternative agents. However, these agents are themselves associated with increased risk of myelosuppression or malignancy. METHODS: The present study included twenty patients with recalcitrant pemphigus or steroid induced side effects who were treated with intravenous cyclophosphamide pulse therapy. RESULTS: Out of 18 patients who completed the study, 14 showed good to excellent response. Thirteen were able to decrease their daily dose of steroid to 5-10 mg prednisolone while one could stop steroids altogether. Most patients did not experience serious side effects. DISCUSSION: Thus intravenous pulse cyclophosphamide is a promising form of therapy in pemphigus either recalcitrant or intolerant to steroid therapy. The decreased total cumulative dose of cyclophosphamide with intravenous therapy as compared to oral therapy may reduce the incidence of secondary malignancy.

Intranasal administration of fusidic acid cream in leprosy.

The effect of local treatment of nostrils with fusidic acid cream was investigated in 30 previously untreated lepromatous leprosy patients. The cream was applied in the nostrils after flushing the nostrils with normal saline, twice a day for a period of four weeks. It was found that 20 mg/gm of sodium fusidate was effective in reducing the morphological index of the nose-blow smear to zero in two weeks in majority of the patients. No untoward side effect was seen in any of the patients. Such nasal treatment along with multidrug therapy may help in reducing the patient's level of infectiousness to their contacts, since the nose is recognized to be an important portal of exit of M. leprae.

A study of pure neuritic leprosy: clinical experience.

Pure neuritic leprosy is a well-recognized clinical entity. Manifestations of leprosy in pure neuritic form accounted for 179 patients out of the total 3853 leprosy patients (4.6%) attending our Poona Urban Leprosy Investigation Centre clinics. Patients with pure neuritic leprosy are prone to develop nerve damage. Eight-seven (48.6%) of our pure neuritic patients presented with deformities. Involvement of upper extremity and right ulnar nerve in particular was the most common clinical feature. Patients presenting with involvement of two nerves of the same extremity was also quite common. None of our patients developed skin lesions while on anti-leprosy treatment. It is important to recognize neuritic symptoms early and suspect leprosy even in the absence of skin lesions.

Study of deformities in children with leprosy: an urban experience.

A study was conducted to assess the deformities in children with leprosy. Eyes, hands and feet were examined for leprosy-related deformities. The influence of age, sex, duration of disease, type of disease, occurrence of leprosy reactions and anti-leprosy treatment on the occurrence of deformities was studied. In our urban leprosy project the percentage of children (0-14 years) suffering from leprosy is 7% of the total number of leprosy patients. The response to multidrug therapy, health education and physiotherapy was good.

Wax therapy for dry feet in leprosy.

A comparative study of the effect of wax therapy and foot soaks on dry plantar skin was conducted in patients with leprosy. Thirty patients with varying grades of fissures and callosities were given wax therapy for feet, and 20 similar patients were given foot soaks. Patients given wax therapy felt subjectively much better than those who had soaking. Healing of cracks and fissures and softening of callosities was observed more frequently in patients with wax therapy. These differences are statistically significant. As an institutional method wax therapy has definite advantages for treating patients with fissures and callosities, whereas soaking of the feet is easy and readily available in patients' homes to restore the dry skin to normal.

Oral zinc therapy in recurrent erythema nodosum leprosum: a clinical study.

Effect of oral zinc as an immunomodulator was studied clinically in patients with recurrent ENL over a period of one year. In this study, 40 leprosy patients with chronic ENL, requiring more than 30-40 mg of prednisolone/day for the control of their reactions, were given oral zinc sulphate for a period of four months, and, marked improvement in the frequency, duration and severity of reactions was observed after zinc therapy. Also evident was marked reduction in the steroid requirement after oral zinc therapy. It appears that zinc may be a good substitute for the present day anti-reaction treatment which is not free from disadvantages. Further investigations to know the precise action of zinc on immune-system may help to understand the role of zinc therapy and its optimum duration.