Rituximab in Pemphigus - An Observational Study from a Tertiary Care Center of North India.

Background: Pemphigus is a group of potentially fatal autoimmune mucocutaneous blistering diseases. Rituximab (RTX) is a chimeric anti-CD20 (anti-cluster of differentiate 20) monoclonal antibody being increasingly used and becoming the first-line therapy in the management of pemphigus. Aims and Objectives: This was an observational study to evaluate the efficacy and safety of rituximab in patients of pemphigus vulgaris (PV) who either did not respond or relapsed after conventional therapeutic regimens and in treatment naive pemphigus patients. Materials and Methods: The study included pemphigus patients coming to our immunobullous clinic who did not respond to conventional therapy or relapsed after receiving conventional therapy as well as fresh cases between January 2019 and October 2021. All enrolled patients received two doses of rituximab (1 gram in each) as intravenous infusions two weeks apart as per the rheumatoid arthritis protocol. The efficacy and safety were evaluated by assessing pemphigus area and activity score (PAAS) before and after the therapy, clinical response, and any adverse events during follow-up. Results: Sixteen (ten males and six females) patients were included in the study. The age of these patients ranged from 27 to 60 years, with a mean of 43.8 ± 9.8 years. There were 15 (93.75%) patients with PV (14 mucocutaneous type and 01 mucosal) and one (6.25%) with pemphigus foliaceus. Among these patients, nine (56.25%) were relapse cases, four (25%) were non-responders, and three (18.75%) were fresh cases who received rituximab as first-line therapy. Fourteen (87.5%) patients reached complete remission off therapy over a median time of 6.36 months (ranging from 18 weeks to 35 weeks). Rituximab was well-tolerated by our patients, and no serious adverse events were observed. The main limitation of our study was the small sample size and the lack of a comparison group. Conclusion: Rituximab is a safe and effective treatment for pemphigus.

Drug reaction with eosinophilia and systemic symptoms: manifestations, treatment, and outcome in 17 patients.

BACKGROUND: Drug-induced hypersensitivity syndrome (DIHS) or drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare type of adverse drug reaction with complex clinical features involving multiple systems of the body. OBJECTIVES: This study was designed to evaluate the clinical features, course, response to treatment, and outcome of DRESS. METHODS: The study involved a retrospective analysis of data collected over a period of four years in 17 patients with DRESS. Clinical features, laboratory findings, responses to treatment, and outcomes were investigated. RESULTS: The study population included 17 patients, of whom eight (47.1%) were male and nine (52.9%) were female. The most common (64.7%) culprit drugs were anticonvulsants (phenytoin, phenobarbitone, carbamazepine, oxcarbazepine, and lamotrigine). Other causative drugs included allopurinol, dapsone, vancomycin, leflunomide, and nitrofurantoin. The latency period varied from 11 days to 34 days, with a mean of 22.35 ± 5.83 days. The mean latency period of anticonvulsant drugs was longer than that of other drugs. Multisystem involvement was present in all patients. Systemic corticosteroids, injectable followed by oral, were administered to all patients. Thirteen (76.5%) patients recovered completely, two (11.7%) developed post-inflammatory hyperpigmentation, and one (5.9%) patient developed renal failure. One patient with liver failure had a poor outcome. CONCLUSIONS: A variety of drugs can cause DRESS, the most common being anticonvulsants. Patients show diverse presentations with varied organ involvement. Systemic corticosteroids are an effective management option and are associated with a good clinical outcome if started early.

Two contrasting post-zoster dermatomal phenomena.

A 29-year-old, normotensive, nondiabetic man presented with a 9-day history of a scaly, pruritic eruption involving the right chest, axilla, and arm. He had a history of herpes zoster involving the same areas about 4 weeks ago. The present eruption started after the herpetic lesions had healed. Examination revealed scaly, erythematous plaques and papules involving the right side of the chest, axilla, and arm in a dermatomal pattern (figure 1). Removal of the scales revealed underlying bleeding points (positive Auspitz sign). The rest of the body, including scalp, palms, soles, and nails, were normal. There was no history suggestive of psoriasis in any family member. Systemic examination and routine investigations were noncontributory. A clinical diagnosis of psoriasis was made and confirmed by histopathologic examination of a skin biopsy sample. The patient was prescribed a topical clobetasol cream and oral levocetirizine. The eruption resolved completely after 3 weeks. A 43-year-old normotensive, nondiabetic woman presented with a 2-day history of fever, arthalgias, and generalized erythematous dermatitis. Five days ago, the patient had a toothache for which she was prescribed injectable ampicillin. After receiving ampicillin for 3 days, she developed fever, myalgias, and arthalgias, which was followed several hours later by an erythematous eruption. The dermatitis started on the trunk and, over a period of several hours, progressed to involve the face and limbs. The eruption was slightly pruritic. History revealed herpes zoster 7 months ago involving left thoracic dermatomes, for which the patient was treated with valacyclovir (1 g thrice a day x 7 days) and analgesics. There was no history of post-zoster neuralgia. On examination, the patient was febrile (oral temperature 102 degrees F), her heart rate was 118 beats per minute, and her blood pressure was 110/70 mm Hg. Cutaneous examination revealed an erythematous, maculopapular dermatitis involving the face and limbs in a bilaterally symmetrical pattern; the palms and soles were also bilaterally involved. The whole of the trunk was involved with erythematous and, in places, violaceous, maculopapular eruption except for a small area on the left side corresponding to T8 and T9 thoracic dermatomes (Figure 2). Complete blood cell counts revealed eosinophilia (9%) and liver function tests, kidney function tests, random blood sugar, routine urine examination, and blood and urine cultures were noncontributory. Histopathologic examination of lesional skin biopsy revealed an intense mononuclear cell infiltration with many eosinophils and an interface dermatitis with hydropic degeneration of basal keratinocytes, while in the spared area, only slight lymphocytic infiltration was present in a perivascular distribution. Based on the history and examination, a diagnosis of ampicillin-induced drug dermatitis was made. The ampicillin was stopped and the patient was put on a short course of oral prednisolone, antipyretics, and topical calamine. The patient was afebrile in 2 days and the eruption resolved completely in 8 days.

Split thickness skin grafting in patients with stable vitiligo.

BACKGROUND: Vitiligo is an acquired disorder of depigmentation. Various surgical modalities are recommended for quicker resolution of lesions in stable cases. AIM: To report the efficacy of split thickness skin grafting in a series of 50 patients with stable vitiligo. MATERIALS AND METHODS: Fifty patients with stable vitiligo, not responding to adequate trial of medical line of treatment were taken in this prospective study. After doing complete investigations, including coagulogram, they were subjected to split thickness skin grafting. RESULT: Satisfactory cosmetic results were obtained in all cases. Colour match was good with minimal complications reported. CONCLUSION: Split thickness skin grafting remains a promising option for patients with stable recalcitrant vitiligo. LIMITATIONS: Comparison of efficacy and side effects of various vitiligo surgical modalities was not done.

Bloom syndrome in two siblings.

Bloom syndrome (congenital telangiectatic erythema) is a rare autosomal recessive disorder characterized by telangiectasias and photosensitivity, growth deficiency of prenatal onset, variable degrees of immunodeficiency, and increased susceptibility to neoplasms of many sites and types. We are reporting Bloom syndrome in two brothers from Kashmir (India), 8 and 6 years of age, who presented with erythematous rashes on the face, photosensitivity, and growth retardation.

Antigliadin antibodies in psoriasis.

BACKGROUND/OBJECTIVES: Although antigliadin antibodies (AGA) are markers of coeliac disease, elevated levels of these antibodies are also seen in many other autoimmune, neurological, haematological, collagen vascular and cutaneous disorders, even in the absence of clinically overt gastrointestinal disease. Several studies have reported an association between psoriasis and AGA, with improvement in severity of psoriasis on a gluten-free diet. This study aims to determine whether patients with psoriasis in Kashmir have an increased prevalence of elevated AGA. METHODS: A total of 120 patients (all native Kashmiris) with psoriasis and an equal number of age- and sex-matched controls without any personal or family history of autoimmune disorders were included in the study. Both groups were tested for IgA and IgG AGAs using a standard enzyme-linked immunosorbent assay method. RESULTS: No statistically significant difference in the prevalence of AGA among patients with psoriasis (6.67% for IgA and 4.17% for IgG) and control group individuals (7.5% IgA and 5.0% IgG) was observed. The mean AGA levels in the two groups were not statistically different. Furthermore, no significant association between AGA levels and psoriasis severity, joint involvement, age of onset of psoriasis or arthritis was observed. CONCLUSIONS: The results of our study show that AGA are not elevated in psoriasis patients as compared with a healthy population, and there is no association between AGA and psoriasis, its onset, severity and joint symptoms.