Blue Rubber Bleb Nevus Syndrome with Musculo-skeletal Involvement and Pulmonary Stenosis.

Background: Blue rubber bleb nevus syndrome is a rare clinical entity. Case characteristics: A 13-year-old Indian boy presented with characteristic cutaneous lesions, gastrointestinal malformations, skeletal involvement and pulmonary stenosis. Observation: Diagnosis was confirmed on skin biopsy, radiographic evaluation, colonoscopy and echocardiography. Echocardiography revealed pulmonary stenosis, an association hitherto undescribed. Message: Detailed evaluation in a patient of blue rubber bleb nerves syndrome is mandatory.

Adult onset, hypopigmented solitary mastocytoma: report of two cases.

Solitary mastocytoma is known to occur predominantly in children below 2 years of age and onset in adulthood is rare. Lesions are hyperpigmented in the majority of cases owing to the stimulation of melanin synthesis by mast cell growth factor. We hereby report two patients with adult onset solitary mastocytoma presenting as hypopigmented plaque. The first case was a 24-year-old man who presented with a plaque on the back of the neck of 5 years duration. The second case was a 30-year-old man who had a well-defined solitary, oval 3 x 2.5 cm plaque on the nape of the neck. Stroking of lesion resulted in a wheal with flare (Darier's sign) in both cases. Systemic examination was within normal limits in both cases. Histopathology revealed a dense toluidine blue-positive infiltrate of mast cells in the upper dermis in both cases.

Symptomatic hepatic hemangioendothelioma in a newborn.

A case of hepatic hemangioendothelioma presenting as congestive cardiac failure in a neonate is reported. There was also evidence of platelet consumption. The case was managed successfully with oral prednisolone, resulting in improvement of symptoms and tumor regression over 3 months.

Eyelid involvement in disseminated chronic cutaneous lupus erythematosus.

Eye involvement in chronic cutaneous lupus erythematosus (CCLE) has been reported infrequently. We describe two cases of disseminated CCLE, a 50-year-old woman and a 45-year-old man, with eyelid involvement and disease duration of 4 and 20 years respectively. The first patient showed an excellent response to chloroquine while there was lack of response in the second patient.

Lepromatous leprosy with bilateral facial nerve palsy and hyperthyroidism.

Bilateral lagophthalmos secondary to facial nerve is extremely uncommon. Further, the aetiology in most of these cases is of central origin unlike the peripheral involvement in leprosy. A patient of lepromatous leprosy (LL) may be euthyroid or hypothyroid on account of leprous involvement of the thyroid gland. A case of LL with bilateral lagophthalmos and hyperthyroidism is reported.

Intra-abdominal extralobar pulmonary sequestration presenting antenatally as a suprarenal mass.

Extralobar pulmonary sequestration is a rare pulmonary parenchymal anomaly which rarely may be present in an intra-abdominal location. The authors report a case of intra-abdominal extralobar pulmonary sequestration which presented to us as an antenatally diagnosed suprarenal mass and was worked up as such. The diagnosis was revealed only at laparotomy. Intra-abdominal extralobar pulmonary sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location, especially on the left side.

Factors associated with sight threatening lesions of leprosy in patients on multidrug therapy.

One hundred and six consecutive patients (old and newly diagnosed) attending the leprosy clinic at Guru Teg Bahadur Hospital, Delhi, underwent a detailed ophthalmic examination to identify factors associated with the prevalence of sight threatening lesions of leprosy so as to delineate a sub-group of patients who should continue under active ophthalmic surveillance to prevent blindness. All of them were receiving multidrug therapy as recommended by WHO. Demographic data, systemic parameters and ophthalmic lesions attributable to the disease were endorsed on to a proforma. The data were analysed by chi-square test, discriminant analysis and multivariate regression analysis to isolate factors that could be associated with sight threatening lesions of leprosy. In 106 leprosy patients 212 eyes were examined; sight threatening lesions were found in 37 eyes (17.4%). The comea was involved most commonly (78.3%). The lesions were still present in 15.8% of patients who had completed multidrug therapy. Leprosy related visual impairment was seen in 2.3% eyes and blindness in 0.9% eyes. Factors significantly associated with sight threatening lesions included female gender, multibacillary leprosy, increasing duration of leprosy, thickened corneal nerves, subepithelial punctate corneal opacities and presence of any academic lesion of leprosy. On multivariate and discriminant analysis, female gender, thickened corneal nerves and duration of leprosy > 7 years were significantly associated with sight threatening lesions.

Involvement of genitofemoral nerve with genital lesions in lepromatous leprosy.

The case of a male patient diagnosed to have lepromatous leprosy with type 2 reaction on multibacillary multidrug therapy, with unusual, widespread involvement of genitalia in the form of plaque and nodules of leprosy over scrotum and perimeatal region of glans, necrotic lesions of erythema nodosum leprosum over scrotum, neuritis of genital branch of genitofemoral nerve bilaterally, and azoospermia, is reported.

Diffuse intra-abdominal fibromatosis-report of a new entity with review of literature.

Fibromatosis, arare non-neoplastic spindle cell proliferation of unknown aetiology, can occur anywhere in the body. Though extra-abdominal sites are commonly involved, intra-abdominal fibromatosis has also been described. Described herein is an unusual case of diffuse intra-abdominal fibromatosis in a 9-year-old boy, who could not be salvaged despite extensive medical management.

Treatment of Schamberg's disease with pentoxifylline--therapeutic trial.

Twenty patients with Schamberg's disease were started on pentoxifylline (400 mg once daily) for a period of 8 weeks. Improvement was assessed at 2 weekly intervals by two observers independently and graded as mild (< 25%, moderate (25-50%) and marked (>50%). Marked improvement was observed in 10/20(50%) patients. We conclude that pentoxifylline should be considered as first line therapy in all patients with Schamberg's disease.

Hypopigmented pityriasis versicolor developing on a pre-existing Becker's Naevus.

A 24-year-old male presenting with pityriasis versicolor developing on a pre-existing lesion of Becker's naevus is being reported for its rare occurrence.

Splenoportovenography: safety, success rate and hemodynamic changes, and incidence and natural history of splenic hematoma.

Forty-five non-cirrhotic patients undergoing splenoportovenography (SPV) were studied. SPV was performed successfully in 43 (95.5%) patients. Local pain was encountered in 12 (26.6%) and lasted for a mean (+/- SD) of 7 +/- 3.8 hours. Local tenderness was noted in 17 (37.7%) and lasted for 6.8 +/- 3.7 hours. The mean rise in the pulse rate after the procedure was 6.5 +/- 4.7 beats per minute and the fall in the systolic and diastolic blood pressures was 3.4 +/- 2.5 and 0.8 +/- 1.7 mmHg respectively. Post-SPV splenic hematoma was noted in 3 (6.6%) patients. All were subcapsular, at the site of puncture, and disappeared by the seventh day. There was no difference in the age, change in the pulse rate, and fall in systolic or diastolic blood pressures between those who developed and those who did not develop hematoma. One patient required one unit of blood transfusion. There were no deaths. SPV was noted to be a simple and safe procedure with a high success rate with minimal morbidity and no mortality.