Epidermolysis bullosa in a mother-infant dyad.

Epidermolysis Bullosa is an inherited mechanobullous disorder which presents in the neonatal period as blistering skin lesions. In this case report, we describe an uncommon presentation of Epidermolysis Bullosa Simplex in a term infant, weighing 2640 g, born to a mother who was also diagnosed with Epidermolysis Bullosa Pruriginosa during the course of the evaluation of her newborn. The clinical situation presented us with a unique dilemma with regard to routine newborn care practices including handling, skin and diaper care. Though the presentation was typically characteristic of EB, we illustrate with images the diagnostic modalities and challenges faced in the hospital while caring for this fragile skin in a low and middle-income country's neonatal intensive care unit. This is the first reported case of a neonate with Epidermolysis Bullosa Simplex born to a mother with Epidermolysis Bullosa Pruriginosa.

Cross-sectional study to describe the severity, bio-chemical associations, and final outcomes of COVID-19-associated rhino-orbital-cerebral mucormycosis in a tertiary hospital of East India.

Purpose: The second wave of coronavirus disease 2019 (COVID-19) pandemic triggered a mucormycosis epidemic in India. Diabetes mellitus and dysregulated immune response were contributors, and rhino-orbital-cerebral mucormycosis (ROCM) was the most common presentation. It is however not known whether bio-chemical parameters at presentation correlate with stage of ROCM or final outcome in terms of vision or mortality. Methods: This retrospective, hospital-based study included all in-patients of mucormycosis with ophthalmic manifestations at presentation admitted during June 1, 2021 to August 31, 2021. It aimed to evaluate the association between severity of infection, serum levels of HbA1c, ferritin, interleukin-6 (IL-6), C-reactive protein (CRP), and D-dimer levels at presentation and outcome. Results: There were altogether 47 eligible cases having a mean age of 48.8 ± 10.9 years with a male:female ratio of 2.6:1; forty-two (89.4%) had pre-existing diabetes, and five (10.6%) had steroid-induced hyperglycemia. The mean HbA1c among diabetics was 9.7 ± 2.1. HbA1c and serum CRP showed an increase over subsequent stages, which was not statistically significant (P = 0.31). IL-6 values for all stages were similar (P = 0.97). Only serum ferritin levels showed a statistically significant increase over stages (P = 0.04). IL-6 was significantly lower (P = 0.03) in patients who survived, whereas CRP levels were significantly lower in patients who had final visual acuity (VA) better than only perception of light (P = 0.03). Conclusion: Uncontrolled diabetes mellitus is a significant association of ROCM. Serum ferritin levels at presentation best correlate with extent of the disease. CRP levels are best to prognosticate cases that will have sufficient VA to carry on activities of daily living, whereas IL-6 levels are best associated with survival.

Overreligious beliefs with neuropsychiatric manifestations in a young lady-Rule out Marchiafava-Bignami disease.

Marchiafava- Bignami disease is a rare condition characterized by demyelination of corpus callosum due to alcohol or malnutrition. Here we report a young lady who, due to her religious beliefs had stopped taking food and presented with neuropsychiatric manifestations. Neuroimaging was suggestive of Marchiafava Bignami disease and with adequate nutritional and thiamine therapy she had dramatic improvement clinically and had near complete resolution of lesions in neuroimaging.