DOOR syndrome.

DOOR syndrome is a rare multisystem genetic disorder, consisting of deafness (sensorineural), onychodystrophy, osteodystrophy, and mental retardation. Seizures reported frequently in this condition are often refractory to treatment.

Elephantine but not elephantiasis: Subcutaneous zygomycosis.

Subcutaneous zygomycosis is an unusual disorder caused by a rare fungus, Basidiobolus ranarum. We report this entity in a 4- yr- old boy. Biopsy showed the Splendore Hoeppli phenomenon and the culture yielded Basidiobolus ranarum. The child responded to saturated solution of potassium iodide within 1 month of starting treatment.

A modified surgical technique for steatocystoma multiplex.

BACKGROUND: Steatocystoma multiplex (SM) is a disorder of the pilosebaceous unit characterized by multiple sebum-containing dermal cysts. Different surgical modalities like cryosurgery, aspiration, surgical excision, incision with a surgical blade or sharp-tipped cautery followed by expression of cyst contents and forceps-assisted removal of the cyst wall and carbon dioxide laser have been used in the past. AIMS: To study the efficacy of a modified surgical technique in the treatment of steatocystoma multiplex. MATERIALS AND METHODS: We have used a simple modified surgical technique using a radiofrequency instrument as the incision tool for the treatment of SM in two patients. RESULTS: The results were cosmetically excellent with no complications developing during or after the procedure. No recurrences were observed after five and half months of follow-up. CONCLUSIONS: This is a simple, easy, fast office-based procedure that is associated with minimal blood loss and post inflammatory hypo or hyperpigmentation and scarring are practically absent.

Tophi as first manifestation of gout.

Chronic tophaceous gout classically occurs after 10 years or more of recurrent polyarticular gout. However, tophi can also occur as first sign of the disorder. Here we report a 20-year-old male presenting with multiple subcutaneous nodules on bilateral feet and toes, left palm, right elbow, helix of left ear since last one and half year prior to any other manifestation of gout. He was having mild intermittent arthritis since last six months. Fine Needle Aspiration Cytology of one tophus showed monosodium urate crystals, which are pathognomonic for gout. His serum uric acid was normal and ultrasound revealed bilateral nephrocalcinosis. So far as we know, this is the first case report from India, demonstrating tophi as the initial clinical presentation of gout.

Unusual formation of keloids after each episode of recurrent herpes zoster in an HIV positive patient.

Herpes Zoster (HZ) lesions are well known to heal with keloids. As immunity plays an important role in the development of abnormal scars and keloids, the latter is unusual in HIV where immunity is low. We report a rare case of recurrent HZ in an HIV-positive male where the lesions have healed with formation of keloids in both episodes. Within 50 days of last episode, he had an attack of herpes progenitalis.

Herpes labials and genitalis in a hiv positive patient.

A 22-year-old man who was HIV positive, presented with recurrent oral and genital losions simultaneously.

Effectiveness of Bacillus Calmette-Guerin (BCG) vaccination in the prevention of leprosy; a case-finding control study in Nagpur, India

A hospital-based, pair-matched, casecontrol study was carried out at Government Medical College Hospital in Nagpur in central India to estimate the effectiveness of BCG vaccination in the prevention of leprosy. The study included 314 incidence cases of leprosy [diagnosed by World Health Organization (WHO) criteria] below the age of 32 years. Each case was pair matched with one control for age, sex and socioeconomic status. Controls were selected from subjects attending this hospital for conditions other than tuberculosis and leprosy. A significant protective association between BCG and leprosy was observed (OR 0.29, 95% CI 0.21-0.41). The vaccine effectiveness (VE) was estimated to be 71% (95% CI 59-79). The BCG effectiveness against multibacillary and paucibacillary leprosy was 79% (95% CI 60-89) and 67% (95% CI 45-78), respectively. It was more effective during the first decade of life (VE 74%; 95% CI 38-90), among females (VE 82%; 95% CI 64-90), and in the lower socioeconomic strata (VE 75%; 95% CI 32-92). The prevented fraction was calculated to be 51% (95% CI 38-62). In conclusion, this study has identified a beneficial role of BCG vaccination in the prevention of leprosy in central India.