The Demographic Attributes, Clinical Features, and Optimal Management of 143 Patients with Pemphigus: A Retrospective Observational Study from a Tertiary Care Center of India.

Background: This retrospective study was to understand the clinico-epidemiologic and therapeutic aspects of pemphigus patients attending our clinic. Methods: We analyzed charts of 143 (M: F; 51:92) pemphigus patients having variable severity recorded between 2009 and 2019. Therapies were customized based on patient's age, disease severity, comorbidities, compliance prospects, and affordability. The patients were monitored monthly and as needed for therapeutic outcome in terms of disease control, reduced hospitalization, remission/relapse, and drug toxicity. Results: These patients were aged 15 to 86 years, the majority, 68 (47.5%), was 41 to 60 years of age. The pemphigus vulgaris in 83.9% patients was the commonest variant. Treatment regimens were; dexamethasone-cyclophosphamide-pulse (DCP) therapy in 51.2%, dexamethasone-azathioprine-pulse (DAP) therapy in 11%, dexamethasone-pulse (DP) therapy in 5.5%, rituximab in 24.4%, IVIg in 5.5% patients, and oral corticosteroids with or without adjuvant. Remission occurred after 2-17 (mean 5.8) DCP doses; 14 and 7 patients achieved remission for ≥2 y and ≥5 y, respectively. Rituximab was effective to treat both new and relapsed cases (n = 31). Additional treatment with another adjuvant prolonged remission in seven patients relapsed 12-16 months after treatment with rituximab alone. Overall, oral corticosteroids alone and DAP therapy showed unsatisfactory response. Adverse effects seen in 41.9% of patients were mainly corticosteroids related. Conclusion: The overall clinico-epidemiologic spectrum of pemphigus and therapeutic efficacy of DCP, DAP, or corticosteroids in this study was in sync with the literature. Combining rituximab and corticosteroids plus an immunomodulator initially (phase-1), followed by immunomodulator alone for one year (phase-2) will improve long-term (phase-3) therapeutic outcome. IVIg was effectively useful in patients with concurrent infections.

The Clinical Characteristics, Putative Drugs, and Optimal Management of 62 Patients With Stevens-Johnson Syndrome and/or Toxic Epidermal Necrolysis: A Retrospective Observational Study.

BACKGROUND: Case reviews of severe cutaneous adverse drug reactions (ADRs) such as SJS/TEN provide useful insights for clinical characteristics, putative drugs, and management protocols. PATIENTS AND METHODS: Medical charts of 62 (m:f- 20:42) patients with SJS/TEN hospitalized between 2010 and 2019 were analyzed retrospectively for clinical attributes, putative drugs and their indications, extracutaneous complications, and therapeutic outcome. The diagnosis was clinical based on established criteria. WHO-UMC scale for reporting ADR and ALDEN algorithm score were used for causality assessment. Therapies were customized based on in-house resources and affordability. RESULTS: Cases included were SJS (41.9%), SJS/TEN overlap (33.9%), and TEN (24.2%) aged 4-85 years. Complications included transaminitis (69.4%), lymphadenopathy (15.5%), septicemia (11.3%), and wound infections (4.8%). Aromatic anticonvulsants (37.1%), disease-modifying antirheumatic drugs (25.8%), antiretroviral drugs (12.9%), non-steroidal anti-inflammatory drugs (8.1%), antimicrobials (4.8%), and trihexyphenidyl (3.2%) were major putative drugs. The mean latent period was 16.6 days. The observed 8% mortality was because of primary comorbidities or multiorgan failure. Addition of fresh blood transfusion (BT, n = 11) or IVIg (n = 7) to systemic corticosteroids showed early relief in skin tenderness, improvement in general condition, and re-epithelialization. Only 16% of patients developed sequelae. CONCLUSION: Aromatic anticonvulsants, allopurinol, nevirapine, cotrimoxazole, paracetamol, and diclofenac remain the most implicated drugs. Sulfasalazine, leflunomide, ethambutol, and trihexyphenidyl were uncommon additions. A short course of high-dose dexamethasone in the early stage was useful. Addition of BT or IVIg provided rapid relief. Preexisting HIV disease, kidney disease, and sepsis remain important for in-hospital deaths. Retrospective study design and small number of cases remain major limitations.

Therapeutic efficacy and safety of oral tranexamic acid 250 mg once a day versus 500 mg twice a day: a comparative study.

Oral tranexamic acid (TXA) 250 mg twice daily has been used effectively for 4 weeks to 6 months to treat melasma. As relapses are frequent on discontinuation, a minimum effective dose of TXA that can be used safely for long time remains unknown. We compared the efficacy of oral TXA 250 mg once daily and 500 mg twice daily given for 16 weeks in 132 (m:f 23:109) adults with melasma. 42 patients in Group-A (TXA 250 mg/d) and 46 patients in Group-B (TXA 500 mg twice/d) completed the study. They were followed up at 4-week interval for percentage reduction in baseline Melasma Area Severity Index (MASI) and at 24 and 28 weeks for relapse. Therapeutic response, for both as per-protocol and intention-to-treat analysis, was scored as very good (> 75% reduction), good (51-75% reduction), moderate (25-50% reduction), mild (< 25% reduction) or no improvement. Reduction in mean MASI score at 4 weeks was not statistically significant in Group-A but it decreased significantly 8 weeks onwards and was comparable with that in Group-B. The relapse rate was higher in Group-B (10.8%) than Group-A (4.7%) at the end of 28 weeks. Oligomenorrhoea and abdominal discomfort in few patients did not necessitate treatment discontinuation. TXA 500 mg twice daily showed early reduction in mean MASI score compared to 250 mg given once daily with comparable safety and therapeutic efficacy at 16 weeks. Open-label cross-sectional design, no control arm, small number of patients in each group, MASI score being subjective assessment tool, short duration of treatment and follow-up are study limitations.

Association of Psoriasis with Autoimmune Disorders: Results of a Pilot Study.

BACKGROUND: Association of psoriasis with other autoimmune diseases remains an ongoing research subject. OBJECTIVES: To investigate the association of psoriasis with other autoimmune disorders. MATERIALS AND METHODS: We studied 80 (M: F 57:23) psoriasis patients aged 13-75 years for concurrent autoimmune disorders. After clinical examination, hemogram, fasting blood sugar, HbA1c, thyroid function tests, anti-TPO antibody, rheumatoid factor, anti-tTG antibody, anti-CCP antibody, ANA, anti-dsDNA antibody, anti-Ro antibody, and fecal calprotectin were estimated. RESULTS: Mild-to-moderate and severe psoriasis was present in 86.3% and 13.8% patients, respectively. Psoriatic arthritis was present in 3.8% patients, all of whom also had severe psoriasis. Only 37 (46.3%) patients had clinical and/or sero-abnormality suggestive of autoimmune disorders; vitiligo in 3.8%, type-1 diabetes mellitus (DM) in 1.3%, and type-2 DM in 6.3% patients. Sero-positivity reflecting subclinical autoimmunity was noted for anti-CCP antibodies (in 2.5%), rheumatoid factor (in 2.5%), hypo- or hyper-thyroidism (in 8.8%), anti-TPO antibodies (in 5.0%), anti-tTG antibody (in 1.3%), ANA (in 5.0%), anti-dsDNA antibody (in 2.5%), and anti-Ro antibody in 11.3% patients. Elevated fecal calprotectin levels suggestive of inflammatory bowel disease (IBD) occurred in 11.2% of 27 patients. Multiple abnormalities happened in 2.5% patients. CONCLUSION: Apparently psoriasis patients seem to have a predilection for other autoimmune disorders particularly for vitiligo, diabetes mellitus, autoimmune thyroiditis, rheumatoid arthritis, and IBD. However, association between psoriasis and other autoimmune disorders at best remains tenuous for want of strong evidence. Nevertheless, screening for them will improve overall management of these patients. Cross-sectional study design and small number of study subjects remain important limitations.

Transient Symptomatic Zinc Deficiency in a Breastfed Infant Associated with Low Zinc Levels in Maternal Serum and Breast Milk Improving after Zinc Supplementation: An Uncommon Phenotype?

Acrodermatitis enteropathica (AE) is a rare, autosomal-recessive disorder of neonatal zinc deficiency due to SLC39A4 (intestinal zinc transporter, Zip4) gene mutation with onset after weaning while breastfeeding during this period will be protective. Transient symptomatic zinc deficiency is also acquired rarely in breastfed infants with increased zinc requirements and/or inadequate concentration of zinc in breast milk. The nursing mothers of transient symptomatic zinc deficiency infants show SLC30A2 (mammary epithelial zinc transporter, ZnT-2) gene mutation and abnormally low zinc levels in the breast milk despite normal serum zinc levels, which do not improve after zinc supplementation. A 2-month-old breastfed male infant had AE-like clinical features of zinc deficiency for two weeks. His symptoms and low serum zinc levels improved rapidly after zinc supplementation. The mother also had low serum and breast milk zinc concentration and both improved after oral zinc therapy indicating a non-heritable phenotype. The relevant literature is reviewed and significance of dietary zinc supplementation during pregnancy/lactation is emphasized.

Darier's Disease - Response to Oral Vitamin A: Report of a Case and Brief Review.

Darier's disease is an uncommon chronic dermatosis of autosomal dominant inheritance with significant psychosocial morbidity and shows unsatisfactory response to several topical and systemic therapies or various resurfacing or surgical procedure. A 24-year-old woman having characteristic asymptomatic and progressive, hyperkeratotic papular lesions involving the scalp, face, upper trunk, and dorsal hands and feet was diagnosed clinicopathologically as Darier's disease. She was treated successfully with oral Vitamin A 25000 IU given twice daily. The response was apparent within 4 weeks and most lesions cleared in 2 months without any adverse effects. Oral Vitamin A provides an effective and safe low-cost therapeutic alternative in Darier's disease, especially when systemic retinoids, the only effective treatment, remain contraindicated or is not tolerated and for maintenance therapy in the long-term.

Assessment of liver and renal functions in human immunodeficiency virus-infected persons on highly active antiretroviral therapy: A mixed cohort study.

BACKGROUND: Indian data on potential hepatorenal toxic effects of highly active antiretroviral therapy (HAART) in HIV/AIDS-affected persons is lacking. OBJECTIVES: To assess hepatorenal abnormalities in HIV-infected persons on HAART in a hospital-based mixed cohort study using concurrent and nonconcurrent data analysis. METHODS: Hepatorenal function tests, urinalysis and ultrasonogaphy for liver/kidneys (when applicable) were assessed in 400 (men 185; women 215) persons aged 2-84 (mean 47.8) years on HAART. Acute liver toxicity, acute kidney injury and chronic kidney disease were defined depending upon abnormal serum alanine aminotransferase, urea and creatinine levels/clearance as per standard guidelines. RESULTS: The duration of HAART was 1 month to 9 years (mean 3.7 years) with 284 (71%) individuals being on treatment for ≤5years. The major HAART regimens included zidovudine + lamivudine + nevirapine in 175 (43.8%), tenofovir + lamivudine + efavirenz in 174 (43.5%) and zidovudine + lamivudine + efavirenz in 20 (5%) individuals and were associated with grade-1 hepatic dysfunction in 57 (14.3%) individuals, with men aged between 31 and 45 years on antiretroviral therapy for >5 years being mainly affected. Forty two (17.1%) of 246 individuals with anemia and 15 (9.7%) of 154 individuals without anemia showed hepatic dysfunction. None had acute kidney injury, chronic kidney disease or abnormal urinalysis or ultrasonography. In contrast, the pretreatment elevated serum alanine amiotranerase in 99 (22.3%) and blood urea and/or creatinine levels in 16 (4%) individuals decreased significantly post highly active antiretroviral therapy. CONCLUSIONS: The study reflects the low frequency of regimen based highly active antiretroviral therapy-associated hepatic or nephrotoxicity despite prolonged use, especially in the absence of other risk factors. Preexisting anemia appears an important risk factor for highly active antiretroviral therapy-induced hepatotoxicity (OR 1.90, Cl 95% CI 1.02-3.57, P = 0.04). Highly active antiretroviral therapy-associated nephrotoxicity was not a significant problem. Study of viral load or other risk factors and potential of each drug for hepatorenal toxicity/dysfunction in HIV affected were not part of the study. A small number of subjects and retrospective analysis of biochemical parameters were other important limitations.

Clinico-Epidemiological Profile of Patients with Vitiligo: A Retrospective Study from a Tertiary Care Center of North India.

BACKGROUND: We studied clinico-epidemiological features of 945 patients with vitiligo with an objective to delineate epidemiological and clinical aspects of vitiligo from this part of the country. MATERIALS AND METHODS: The medical records of patients with vitiligo attending outpatient clinic over a 5-year period from January 2013 to December 2017 were analyzed retrospectively for this descriptive, observational study. RESULTS: There were 449 men and 496 women (m:f 1:1.1) aged between 2 and 83 years (mean 24.4 years) and having vitiligo for 1 week to 64 years (mean 5.1 years). The majority, 478 (50.6%) patients were aged ≤20 years and 248 (26.2%) were children aged ≤12 years. The age at the onset was between 6 months and 82 years (mean 20.5 years), and the majority 674 (71.3%) patients had it before 25 years of age. The consultation time was within 5 years in 692 (73.2%) patients. A family history of vitiligo was present in 150 (15.9%) patients. The majority 871 (92.2%) patients had involvement of up to 10% body surface area and vitiligo vulgaris in 562 (59.5%) and focal vitiligo in 117 (18.7%) patients were the most common clinical types. An association with other systemic disorders was in 124 (13.1%) patients and predominately included thyroid abnormalities and diabetes mellitus. CONCLUSIONS: Our observations are essentially consistent with the literature. There was no difference in clinico-epidemiological features of vitiligo. Patients with an affected first-degree family member had early onset, but difference was not statistically significant. Screening for concurrent thyroid disorders appears important. However, our inferences remain limited by single center, retrospective, observational, and cross-sectional nature of the study.