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Author(s): Megha P. Lakum (corresponding author) [1]; Jignaben K. Padhiyar [1]; Nayankumar H. Patel [1]; Jahnvi R. Patel [1] A two-year-old girl, born at full term by vaginal delivery to [...]

The article discusses a rare case of late infantile form of Multiple Sulfatase Deficiency (MSD) in a two-year-old girl. The child presented with dry skin lesions and neurological symptoms, leading to a diagnosis of MSD through genetic analysis. MSD is a lysosomal storage disorder caused by mutations in the SUMF1 gene, resulting in neurologic deterioration, skeletal anomalies, organomegaly, and ichthyosis. The article emphasizes the importance of considering MSD in children with skin problems and neurological issues, highlighting the need for detailed examinations and genetic analysis for accurate diagnosis and counseling. [Extracted from the article]

Byline: Nayankumar. Patel, Jignaben. Padhiyar, Tejas. Patel, Nishi. Trivedi, Vidhi. Chandibhamar, Ranjan. Raval Sir, Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease also known as linear IgA [...]

This article discusses the phenomenon of paradoxical disease flare, skin infection, and hypogammaglobulinemia in patients with pemphigus vulgaris (PV) who are treated with rituximab. The available data on this topic are limited to isolated case reports. Hypogammaglobulinemia is a known adverse event of rituximab therapy and has been extensively studied in rheumatic disorders. In this study, four patients with PV experienced a paradoxical disease flare, hypogammaglobulinemia, and bacterial skin infection after receiving rituximab treatment. The authors suggest that monitoring IgG levels and minimizing hospital stays post-infusion may be important in the treatment of PV with rituximab. [Extracted from the article]

This article discusses the relationship between hypogammaglobulinemia-induced skin infections and post-rituximab paradoxical flare in pemphigus. The study found that certain clinical and immunological factors, such as Pemphigus Disease Area Index (PDAI) score and anti-Dsg1 levels, were significantly associated with disease flare after rituximab infusion. The authors also mention the importance of investigating the role of infections, hypogammaglobulinemia, and other underlying factors in predicting and preventing flare-ups in pemphigus patients treated with rituximab. However, there are some points that require further clarification, such as the reasons for changes in treatment plans and the lack of reported microbial studies from lesional skin. [Extracted from the article]

Byline: Jignaben. Padhiyar, Nayankumar. Patel, Trusha. Gajjar, Bhagirath. Patel, Aseem. Chhibber, Mansi. Buch Infectious diseases can clinically present as vesiculobullous disorders. Direct immunofluorescence (DIF) study of skin biopsy helps distinguish [...]

Introduction: In the present epidemic of chronic, recalcitrant, and widespread dermatophytosis, impact of disease on quality of life (QoL) can be severe. Similarly, financial impact due to treatment cost and loss of wages needs consideration too. Aims and Objectives: Our primary aim was to evaluate the impact of dermatophytosis on QoL and the financial burden on individual and family. Materials and Methods: Adult patients having dermatophytosis were included in the study. Standard Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate the psychosocial impact, and financial burden was evaluated by nine binary questions. Results: A total of completed 299 DLQI surveys and 275 financial burden surveys were taken for final analysis. Mean DLQI was 12.25 (SD = 5.56, n = 299). Significant association between total body surface area (BSA) and DLQI score was observed (rs= 0.251,P <0.001, n = 299). Presence of tinea corporis was found to be significantly affecting the choice of clothing (P = 0.018, χ2 = 5.127, CI 95%). More numbers of male respondents reported loss of work or study hours due to their illness (P = 0.015, χ2 = 5.196, CI 95%), which was significantly associated with the BSA involved (P < 0.05, t = 5.529, CI 95%). Some difficulties in sexual activity were reported by 35.6% patients, which has significant association with tinea cruris (P = 0.001, χ2 = 10.810, CI 95%). Median household income was 10,000 INR and interquartile range (IQR) being 8,000–19,000. Mean financial burden calculated at 3.458 (SD = 1.696, n = 275), whereas mean financial worry reported by patient stood at 3.661 (SD = 1.216, n = 275). Financial burden showed significant correlation with "previous treatment approximate cost," financial worry, and DLQI (P < 0.05, CI 95%). Financial worry (P = 0.016, rs= 0.145, CI 95%) and financial burden (P = 0.002, rs- = 0.145, CI 95%) both showed positive correlation with duration of disease. Conclusion: In present scenario of Dermatophytosis in India, the disease and its treatment causing impact on QOL as well as on personal financial burden and worry need consideration. [ABSTRACT FROM AUTHOR]

Background: Propranolol may be more effective and safer than previously established therapies, and it may be used as a first-line therapy for infantile hemangioma (IH). Propranolol is thought to inhibit the growth of blood vessels by decreasing vascular endothelial growth factor. Aims and Objectives: The aim of this study is to study the efficacy and safety of propranolol in IH and for standardization of dose in tablet form. Materials and Methods: A total of 23 patients with 30 IH s were recruited in the study prospectively, after ruling out any contraindications for oral propranolol and obtaining consent from parents. Patients with <7 kg weight were given oral propranolol 5 mg twice daily and >7 kg weight were given 5 mg thrice daily. Patients were evaluated according to visual analog scale and ultrasonographically on day 0, day 30, day 60, and day 90. Results: Out of a total of 30, 25 (83.33%) were superficial, 3 (10%) were deep, and 2 (6.66%) were mixed hemangioma. All patients with superficial hemangiomas showed a change in the color of the lesion and arrest of growth within the 1st month of therapy and a gradual decrease in size was noticed in 23 (92%, n = 25) patients during the study period except in 2 (8%). Complete clearance was noticed in 68% of patients of superficial hemangioma at the end of the study period. Out of 25 patients, a total of 5 (20%) patients had ulceration at the time of presentation which started to heal within 15 days of therapy. Five (16.66%, n = 30) patients with deep and mixed variety showed arrest of growth but no decrease in size. No side effects were seen except temporary coldness of extremity in 1 (3.33%) patient. Conclusion: Propranolol is safe and effective for treatment of proliferative phase of superficial hemangiomas with very less side effects compared to oral steroids and other recommended therapies, and unavailability of syrup can be overcome by giving tablet in fixed dosage. [ABSTRACT FROM AUTHOR]

Secondary cutaneous diffuse large B-cell lymphoma has a higher international prognostic index score and worse prognosis than diffuse large B-cell lymphoma, leg type. Secondary cutaneous DLBCL demonstrates higher international prognostic index (IPI) when compared to DLBCL leg type.[[5]] According to revised IPI score, our patient had poor prognosis with probable overall survival of 55% and progression-free survival of 53%. Secondary cutaneous lymphoma: Comparative clinical features and survival outcome analysis of 106 cases according to lymphoma cell lineage. [Extracted from the article]

Direct immunofluorescence (DIF) is widely used in dermatopathology for the diagnosis of autoimmune blistering diseases (AIBDs), cutaneous vasculitis, and connective tissue disorders. Although it is easy and useful to perform, it needs technical expertise and experience for proper interpretation. The yield of DIF depends on multiple factors including the adequacy, transportation, storage, processing, and interpretation of the biopsy specimen. Effective collaboration between the dermatologist and dermatopathologist along with meticulous clinico-pathological correlation is crucial for accurately interpreting DIF in the appropriate clinical context. In this narrative review of DIF in dermatology, we discuss the indications of DIF, recent updates on the selection of optimum biopsy sites, basic techniques of DIF including the classical transport medium and its alternatives, processing and staining technique, patterns in various diseases, advancements such as serration pattern analysis, and latest recommendations on the use of DIF in cutaneous disorders.