Overlapping Hyaline Fibromatosis Syndrome: A Rare Case of Juvenile Hyaline Fibromatosis and Infantile Systemic Hyalinosis.

Juvenile hyaline fibromatosis (JHF) and infantile systemic hyalinosis (ISF) are rare progressive, fatal autosomal recessive fibromatosis disorders that are characterized by the deposition of hyaline in various tissues. Mutations in capillary morphogenesis gene 2 are responsible for both of these conditions. These disorders usually present with fleshy, papular lesions, joint contractures, gingival hyperplasia, and persistent diarrhoea. An 18-month-old boy presented with multiple scalp abscesses, facial nodules, gingival hypertrophy, hypertrophic verrucous plaques and joint contractures with unique dermoscopic features and a history of recurrent diarrhoea and infections. Histopathological examination following skin biopsy revealed deposition of hyaline in the stroma and subcutaneous tissues. JHF is a differential diagnosis in children who present with multiple scalp nodules. Here, we report the case of overlapping features of JHF and ISH. The evolution of this case provides a special opportunity to further understand the pathogenesis and clinical characterization of hyaline fibromatosis syndrome.

Pattern of sexually transmitted infections in a tertiary care centre at Puducherry.

BACKGROUND: The prevalence of sexually transmitted infections (STI) varies widely from region to region in our country. AIMS: To highlight the pattern of STIs and the profile of patients with HIV infection in STD patients as seen at our hospital. METHODS: A retrospective chart analysis of clients attending STI clinic, JIPMER, Puducherry, from June 2004 to June 2006 was done. RESULTS: A total of 866 clients attended our STI clinic, out of whom 435 (50.2%) had proven STI. STIs were more common in men, with a male (290): female (145) ratio of 2:1. Their age ranged from 1 year to 75 years (mean age = 32.38 years) with the maximum number of patients in the age group of 21-30 years, while children constituted only 2.8%. Herpes genitalis (107 patients, 32.8%) was the most common ulcerative STI, while genital wart was the most common nonulcerative STI (56 patients, 17.1%). Non-gonococcal urethritis (46 patients, 14.1%) was more common than gonococcal urethritis. HIV infection was the most common STI in our study, at an alarmingly high rate of 34.5% (151/435). HIV seropositivity was more common in patients who presented with ulcerative STIs than with nonulcerative STIs. CONCLUSIONS: Herpes genitalis was the most common ulcerative STD, while genital wart was the most common nonulcerative STI in our study. The prevalence of HIV among STI clients in India has been on the rise, but has quite alarmingly become the most common STI in our study.

A case report of venous thrombosis in a leprosy patient treated with corticosteroid and thalidomide.

Thalidomide is the drug of choice in the treatment of severe erythema nodosum leprosum (ENL) in men. It has recently been associated with deep venous thrombosis (DVT) when used in treatment of refractory multiple myeloma along with combination chemotherapy. We report a case of DVT in a patient treated for ENL with corticosteroids and thalidomide, and suggest a possible mechanism for the association.

Cutaneous diphtheria masquerading as a sexually transmitted disease.

A 41 year-old, sexually promiscuous, married male, an agricultural laborer by occupation, presented to our sexually transmitted diseases (STD) clinic with multiple ulcers over the scrotum and genitalia of 20 days' duration. Bacterial culture from swabs taken from the genital ulcer, grew organisms morphologically and biochemically characteristic of Corynebacterium diphtheriae. He made a complete and uneventful recovery after two weeks of therapy with antidiphtheria serum and crystalline penicillin. This case brings into light this hitherto unreported presentation of wound diphtheria mimicking a sexually transmitted genital ulcer disease and thus, underlines the importance of considering diphtheria as differential in atypical, long-standing genital ulcers.