2026-06-16 | Management of Recurrent Linear IgA Dermatosis of Childhood in the Absence of Histopathology and Immunofluorescence: A Case Report from a Low-Resource Setting in South-Western Uganda.
Linear IgA Dermatosis of Childhood (LAD of childhood) is a rare autoimmune sub-epidermal blistering disorder classically confirmed by direct immunofluorescence demonstrating linear IgA deposition along the basement membrane zone. However, in resource-limited settings, access to immunopathological diagnostics is often restricted, posing significant challenges to timely diagnosis and management. We report the case of a 12-year-old girl from rural South-Western Uganda with a history of recurrent vesiculobullous eruptions beginning at 2 years of age, with subsequent episodes at 7 years and a current severe presentation at 12 years. The disease demonstrated a progressive increase in severity with each recurrence. On examination, she had multiple large, tense bullae arranged in annular and polycyclic patterns over the trunk, limbs, thighs, and groin, with a characteristic "string-of-pearls" configuration and no mucosal involvement. Due to unavailability and unaffordability of histopathology and immunofluorescence studies, a clinical diagnosis of LAD of childhood was made based on history, morphology, distribution, recurrence pattern, and exclusion of close differentials. The patient was treated with dapsone, systemic corticosteroids, antibiotic prophylaxis, and local wound care. Within one week of initiating therapy, there was marked clinical improvement, with cessation of new blister formation, resolution of existing bullae, and progressive re-epithelialization. This case underscores the critical role of clinical acumen in diagnosing LAD of childhood in resource-constrained settings where gold-standard investigations are inaccessible. It highlights that a thorough history, careful physical examination, recognition of characteristic lesion patterns, and therapeutic response to dapsone can provide sufficient diagnostic confidence. Importantly, lack of advanced diagnostic tools should not delay initiation of appropriate treatment. This report also emphasizes the need to strengthen diagnostic capacity in low-resource settings while reinforcing the value of clinical judgment in managing rare dermatological conditions.
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2026-03-06 | Autoimmune Bullous Diseases Associated With Immune Checkpoint Inhibitors: An Analysis Based on a Systematic Review.
Autoimmune bullous diseases (AIBDs) constitute a rare yet potentially life-threatening subset of immune-related adverse events induced by immune checkpoint inhibitors (ICIs). However, the characteristics of ICI-induced AIBDs (ICI-AIBDs) and the factors influencing patient survival remain incompletely characterized. Therefore, we aimed to synthesize the available information on ICI-AIBD patients and sought to explore factors potentially influencing the survival outcome of this population. A systematic search of 5 databases was conducted. Cox regression analysis was used to identify potential factors affecting patient survival outcomes. Finally, a total of 188 studies with 319 participants were analyzed. The spectrum of AIBDs comprised bullous pemphigoid (n = 254, 79.6%), lichen planus pemphigoides (n = 34, 10.7%), mucous membrane pemphigoid (n = 11, 3.4%), pemphigus group (n = 7, 2.2%), linear IgA bullous dermatosis (n = 5, 1.6%), primarily induced by programmed cell death protein 1 inhibitors. In the exploratory analysis, female (hazard ratio [HR], 2.35; 95% confidence interval [CI], 1.10-5.02; p = 0.020) and pemphigus group (HR, 7.09; 95% CI, 1.87-26.96; p = 0.003) were potentially associated with higher mortality, whereas topical glucocorticoid therapy was potentially protective (HR, 0.44; 95% CI, 0.21-0.93; p = 0.025). In conclusion, we delineate the full clinical spectrum of ICI-AIBDs, tentatively exploring factors potentially affecting patients' survival, which provides insights for individualized therapy and may inform future clinical practice.
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2026-03-01 | Refractory linear IgA dermatosis in childhood: a successful response to rituximab
Refractory linear IgA dermatosis in childhood: a successful response to rituximab
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