Cyclophosphamide and congenital erythropoietic porphyria.
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Biomedical subjects
Publications and source records attributed to T R Bedi.
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A unilateral verrucous lesion with clinical characteristics of nevus unius lateralis (NUL) in an 18-year-old boy, showed histopathological features of intraepidermal basaloid cell formation simulating superficial basal cell epitheliomas. Biological significance of this phenomenon is discussed. The lesion is considered a most peculiar variant of NUL.
2 patients with primary localized cutaneous amyloidosis were seen to have widespread macular and lichenoid types of lesions. Diffuse areas of pigmentation appeared to transform gradually over years into lichen amyloidosus as a result of chronic irritation of the skin from scratching. There was no evidence of systemic amyloidosis.
Skin biopsies from clinically normal skin of the scalp, axillary and groin regions in 20 lepromatous leprosy patients revealed significant histopathological findings in upto 25 percent of the patients. The positive findings could, perhaps, be enhanced by studying larger skin materials from these body areas. Indeed, no skin area appears to be immune from invasion by Myco. leprae.
A case of red grain mycetoma on the scalp is described. The disease, caused by Actinomadura pelletieri, is extremely rare in this part of the world, and from India this is perhaps the first case with scalp involvement. The lesion showed satisfactory response to partial excision followed by chemotherapy with streptomycin.
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The mean values of serum immunoglobulins A, G, M and D levels in 42 psoriasis patients showed no significant difference from those of control subjects. Analysis of the same sera for the presence of antiglobulin antibodies also yielded negative results. Antinuclear factor could be demonstrated in only 4.8% of the cases. The possibility that serum antiglobulins get drained into the lesions making their detection in the serum difficult, is supported by our preliminary findings of focal deposition in the stratum corneum of immune complexes of IgG, IgM and complement in tissue sections of psoriatic lesions from these patients.
A distinct summertime skin disorder seen in 25 Indian patients is described under a tentative designation of summertime actinic lichenoid eruption (SALE). The eruption predominantly affects females in their 2nd to 4th decade. The initial lesions are in the form of closely aggregated pruritic lichenoid papules starting during summer months on the exposed parts of the body. The lesions respond favourably to sun protection and corticosteroid cream but the recurrences are common during the subsequent summers when the lesions tend to become more persistent, pruritic and lichenified. Histopathologic features include early basal cell degeneration, spongiotic vesiculation with or without focal parakeratosis and an intense lymphocytic infiltrate in the dermis. In spite of strong clinical evidence of relation to sunlight, the lesions are not easily reproducible by artificial light.
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A case of Buschke-Löwenstein's tumour presenting with urinary fistula is described. The large lesion in the subpreputial sac occluded the preputial opening and infiltrated beneath the skin of the shaft of the penis resulting in a fungating growth encasing the fistulous tract. The lesion responded well to 25% podophyllin, which is reportedly unusual.
Intradermal injections of triamcinolone acetonide have used in the treatment of psoriatic onychodystrophies in seven patients. The therapeutic response initially is good but relapses appear inevitable. One patient developed rather persistent periungual hypopigmentation, thereby limiting the routine recommendation of this form of treatment.
Analysis of 162 psoriasis patients revealed a distinct male preponderance with a sex ratio of 2.5:1. The mean age of males (31.44) was 6 years higher than that of females (25.73). More than half the patients were in their 3rd to 4th decade and the mean age of onset was lower among the females than the males by approximately 5 years. Two-thirds had onset before the age of 30 years. Familial occurrence was recorded in 13.6% of the cases. Itching appeared to be a prominent feature and only a fourth of the total patients showed traditionally quoted worsening in colder months. The scalp and the extremities were the commonest sites of involvement and a large majority (74%) showed nail changes of one type or another. Psoriatic arthropathy and especially mutilating arthritis were rare.
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A patient of lepromatous leprosy with recurrent erythema nodosum leprosum developing acute renal failure proving fatal within 8 weeks is reported. The renal lesion demonstrated acute proliferative glomerulonephritis. Its pathogenesis in relation to ENL is discussed.
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