Paracetamol-induced fixed drug eruption presenting as eyelid skin necrosis

Fixed drug eruption (FDE) is a type of drug-induced cutaneous disorder that characteristically presents with recurrence of similar lesion at the same skin or mucosal site as a result of systemic exposure to a drug. Paracetamol is commonly prescribed analgesic-antipyretic agent in all age group of patients. FDE due to paracetamol is not very common but it is well reported in literature for all age groups. We report a case of a 7-year-old male with FDE due to paracetamol involving upper eyelid and presenting as an eyelid skin necrosis.

ANA-negative SLE presenting with nephritis and oculomotor palsy--a case report.

Most large series of patients with systemic lupus erythematosus (SLE) have revealed a very small group of patients who are persistently antinuclear antibody (ANA) negative with prominent dermatologic involvement but infrequent neurological and renal involvement. We present a very unusual case report of a young female with ANA-negative SLE presenting with lupus nephritis and oculomotor palsy without any dermatologic manifestations.

Radionuclide scintigraphy in Caroli's disease.

Congenital cystic dilatation of the intrahepatic bile ducts involving the major intrahepatic radicles was first described by Caroli and hence named as Caroli's disease. We present here a case in which the only symptom was intermittent pain in the abdomen for last one-and-a-half-year and a radionuclide scan done effectively, not only diagnosed the disease but also the associated cholangitis.

Epidemiology and clinical presentation of abdominal tuberculosis--a retrospective study.

The epidemiology and clinical presentation of abdominal tuberculosis were studied retrospectively in 298 adult cases admitted in Safdarjang Hospital, New Delhi over a 3-year period. These constituted 17% of the total number of admissions for tuberculosis. Age at presentation was variable with maximum cases in 21 to 40-year age group (58% of cases) with a mean age of 32.7 years. There was a slight female preponderance (57%). Sixty-three per cent were residing in urban areas. Pain abdomen, ascites and subacute intestinal obstruction were the commonest modes of presentation (34%, 30%, 28% respectively). Other clinical features included fever (21%), altered bowel habits (19%), weight loss (8%) and lump abdomen (6%). Acute intestinal obstruction and lower gastro-intestinal bleeding were uncommon (5% and 4% respectively). Co-existent pulmonary tuberculosis was seen in 16% cases. Histological evidence was available in 41% cases. Majority improved with conservative management with only 21% requiring surgical intervention. Mortality recorded was 11%.

Distribution of HLA antigens in Indian Gurkha population.

Fifty unrelated Indian Gurkha of Nepalese origin were studied to analyse the HLA antigen profile and their relation with other populations. Haplotype B35-Cw4 occurred with highest incidence and significant positive linkage disequilibrium in Gurkhas. Haplotype A10-B8 which occurs with the highest frequency in north Indians was also observed to occur with significant positive linkage in Gurkhas. HLA profile of Gurkhas thus may be the result of long-term isolation and genetic drift.

Restriction fragment length polymorphisms in HLA-DR4-DQ3 haplotypes associated with rheumatoid arthritis.

Restriction fragment length polymorphism (RFLP) patterns were studied in serologically confirmed DR4-DQ3 positive patients with rheumatoid arthritis by Southern blot analysis using full length cDNA probes specific for DRB, DQA and DQB hybridized with genomic DNA digested with informative restriction endonucleases. The RFLP patterns correlated with serology confirming all patients to be DR4+ve. The DQB1*0302 (DQ8) allele identified by 12.0kb BamHI, 3.3kb Hind III and 1.8kb Taql fragments was present in all patients suggesting them to be DR4-DQB1*0302. Hybridization of Taq 1 and PVU II digested genomic DNA with DQA cDNA probe revealed four informative RFLP patterns. While three of them correlated with known DR4 subtypes, one was a new polymorphism observed specifically in Indian patients with rheumatoid arthritis. The study further indicated that two of the several known subtypes of DR4, viz., DRB1*0401-DW4-DQB1*0302 and DRB1*0404-DW14-DQB1*0302 may be implicated in susceptibility to rheumatoid arthritis in the Indian population.

Effect of D-penicillamine on lymphocyte subsets: correlation with clinical response in rheumatoid arthritis.

We studied the effects of D-penicillamine (DP) on the clinical response, immunoinflammatory parameters and the lymphocyte subsets in 46 patients with rheumatoid arthritis (RA). Patients were evaluated before the start of the drug and then at 3 and 9 months during the follow up. 38 of 46 (82.6%) patients could continue DP treatment for over 9 months, while in 8 the drug was withdrawn due to adverse effects. Improvement in the various disease activity indices of more than 50% (responders) was seen in 25 of 38 (65.8%) patients. Responders showed a significant decrease in the serum IgA and IgM at 9 months, and in IgM only at 3 months. The serum levels of C3 and C4 did not show any significant change. Serum levels of C-reactive protein and rheumatoid factor (RF) showed a significant decrease at 3 and 9 months. A significant decrease in CD3+ and CD4+ lymphocytes along with a fall in CD4+/CD8+ lymphocyte ratio was also seen in responders at 3 and 9 months, compared to the baseline. Our results suggest that DP may have immunomodulatory action in RA.

Dermatoglyphic patterns of patients with rheumatoid arthritis.

Finger tip and palmar dermatoglyphics were studied in 31 patients (22 females and 9 males) with rheumatoid arthritis (RA) and 38 matched controls (20 females and 18 males) from North India. While not many differences were observed in palmar patterns, a low ending of line A was found on both hands of two patients. Finger tip patterns were significantly different in patients compared to controls. No association with any dermatoglyphic feature and HLA antigens was observed.

Protective & risk DR phenotypes in Asian Indian patients with rheumatoid arthritis.

This study of 168 north Indian patients with rheumatoid arthritis (RA) confirms the significant association of susceptibility to RA with DR4 specificity (P less than 0.0001). This association was observed equally in familial as well as sporadic patients. The HLA-DR2 and DR5 alleles were identified to be conferring protection in RA, DR5 being reduced significantly in the non-familial patients only. None of the other DR antigens revealed any association with RA in this population, including the DR4 negative group of patients. An analysis of the DR phenotypes in patients and controls revealed that DR4 in combination with DR1 provided the highest relative risk (71.9) followed by DR4, DR4 (RR = 4.1). These results demonstrate that susceptibility to RA is not due to a single HLA specificity but the effect of a group of related epitopes occurring in common among subtypes of DR4 as well as in some DR1 alleles.

Immunologic and immunogenetic studies in rheumatic fever and rheumatic heart disease.

In order to evaluate all the important limbs of the immune system in the same patient population with rheumatic fever (RF) and rheumatic heart disease (RHD) cellular and humoral immune parameters as well as the immunogenetic profile in 265 North Indian patients with RHD were evaluated. They were studied for class in HLA antigens and 165 of them were also evaluated for the class II (DR locus) antigen profile. Data obtained was compared with 400 and 134 healthy controls respectively of the same ethnicity. Humoral immune parameters (Serum immunoglobulins IgG, IgA; Serum complement fractions C3, C4, C3d; circulating immune complexes and B lymphocyte numbers) and cellular immune parameters (total leucocyte and lymphocyte counts; T lymphocyte sub-populations--CD4, CD8 counts; lymphocyte migration inhibition to an extracellular streptococcal antigen, streptolysin 'O') were studied in 23 patients with RF, 21 patients with "inactive" RHD and 20 normal controls. Patients of RHD were noted to have an increased frequency of DR3 (P less than 0.001; Relative risk = 2.3) and a decreased frequency of DR2 (P less than 0.001; Relative risk = 0.3) as compared to the controls. Patients of RF had evidence of an altered regulatory T cell function (increased CD4/CD8 ratio) and decreased cell mediated immunity to streptolysin 'O'. An increased humoral immune response (increased B cell counts, elevated serum IgG, circulating immune complexes and C3d) was noted in patients of RF as well as "inactive" RHD. An integrated pathogenetic model with immune response associated antigens of the DR locus influencing selection of cardiac cross-reactive antigens by the antigen processing macrophages, an altered regulatory T cell function with decreased suppressor T cell activity leading to an abnormal immune response is proposed to explain the pathogenesis of RF.

Effect of maternal iron deficiency on GABA shunt pathway of developing rat brain.

Iron deficiency during pregnancy and lactation (35 mg iron/kg diet) produced a significant reduction in liver nonheme iron in dams as well as in fetus and young ones. The body, liver and brain weights of fetus, new-born, and developing pups remained unaffected. However, the body weight and PCV were reduced only in 21-day-old pups. The enzyme activities of GDH, GAD, GABA-transaminase, and NAD(+)-linked ICDH were reduced in 14 and 21-day-old pups. The enzyme activities of NADP(+)-linked ICDH activities remained unaffected in the fetus and developing pups brain. Maternal rehabilitation on iron sufficient diet for 1 week from day 14 to 21 of lactation period did not reverse these changes. The maternal iron deficiency during lactation period alone did not cause any alteration in all parameters assayed, however, there was a reduction in liver non-heme iron of pups on days 14 and 21.

Evidence to show MHC-linked factors other than HLA-B27 governing susceptibility to spondylo-arthropathies in Asian Indians.

The HLA antigen profile of 129 North Indian patients with ankylosing spondylitis, 66 patients with Reiter's syndrome and 57 patients with 'unclassifiable' arthritis was compared with 380 normal, healthy controls. Besides B27 which appeared with a significantly increased frequency in the three patient groups, other HLA antigens, viz. A2 and B35, showed deviated frequencies. The HLA supratype A2, B27 was found to be at an elevated frequency in patients with ankylosing spondylitis and unclassifiable arthritis whereas the B35, B27 combination showed a decreased frequency in our Reiter's syndrome sample. These data suggest that besides B27, other HLA-linked factors influence susceptibility to spondylitic disorders and might act as 'modifier' genes for the type and severity of spondylo-arthropathy in a B27-positive individual.