Clinico-epidemiological features of chronic urticaria in children: A retrospective analysis of 296 children from a tertiary care institute in Northern India.

BACKGROUND: Urticaria is a common dermatosis affecting approximately 25% of the population. Childhood chronic urticaria is frequently encountered, however, epidemiologic data on pediatric urticaria are limited. AIM: The objective of this study was to study the clinico-epidemiological profile of children with chronic urticaria. METHODS: A retrospective study including children less than 14 years with chronic urticaria was conducted from January 2010 to December 2015. Detailed history, clinical examination, investigation results, treatment taken, and follow-up details were recorded on a prefixed proforma. As per the practice of the urticaria clinic, the children were investigated only in case of inadequate therapeutic response or had features of atopy/autoimmune disorders. RESULTS: Two hundred and ninety-six children (166 boys, 130 girls; mean age, 11.3 years) with chronic urticaria were included in the study. Urticaria was spontaneous in onset in 57.1% (169) children; precipitating factors were reported in 42.9% children, most common being physical factors, food allergy, drug intake and infections. Investigations were done in 48 (16.2%) patients; Antinuclear antibody was negative in all patients, raised serum IgE in 20/48 (41.6%), positive autologous serum skin test in 32/48 (66.6%) and raised anti-TPO titre in 10/48 (20.8%) children. A diagnosis of chronic spontaneous urticaria was made in 245 (82.77%) children, chronic dermographic urticaria in 35 (11.82%), cholinergic urticaria and drug-induced urticaria in 5 (1.69%) each, aquagenic urticaria in 4 (1.35%) and cold-induced urticaria in 2 (0.68%) children. Two hundred and fifteen (72.6%) children responded to nonsedating antihistamines alone, 61 (20.6%) required addition of a sedating antihistamine, 7 (0.02%) required addition of montelukast, 3 (0.01%) ranitidine and 10 (20.8%) required a short course of oral corticosteroids to control acute flare. None of the patients required any long-term immunomodulatory or immunosuppressive agent. The mean duration of treatment required was 3 to 12 months. LIMITATIONS: The main limitation is the study being retrospective in nature with associated drawbacks of data loss. In addition, we did not use objective scoring system such as urticaria severity score and not all children were extensively investigated. CONCLUSIONS: Chronic spontaneous urticaria is the most common type of chronic urticaria in children. Majority of these children can be managed conservatively with long-term antihistamines.

Epidermolysis bullosa acquisita and anti-p200 pemphigoid as major subepidermal autoimmune bullous diseases diagnosed by floor binding on indirect immunofluorescence microscopy using human salt-split skin.

BACKGROUND: Subepidermal autoimmune bullous diseases are a diverse group of diseases with overlapping clinical and immunopathological features. Indirect immunofluorescence microscopy on artificially split skin helps to classify these conditions into those with staining on the epidermal side of the split ("roof-binding") and those with staining on the dermal side ("floor-binding"). Epidermolysis bullosa acquisita is the prototype of "floor-binding" subepidermal autoimmune bullous diseases. However, not all floor-binding sera are associated with epidermolysis bullosa acquisita. AIM: The aim of this study was to evaluate the clinical and immunological profile of patients with floor-binding subepidermal autoimmune bullous disease by indirect immunofluorescence microscopy and to identify the target antigens in them. METHODS: Ten patients who showed a floor-binding pattern were studied with regard to their clinical and immunopathological characteristics. Target antigens were identified by modified indirect immunofluorescence microscopy using recessive dystrophic epidermolysis bullosa skin, enzyme linked immunosorbent assay, and immunoblotting. RESULTS: Diagnosis of epidermolysis bullosa acquisita was confirmed in six patients. Three patients with an inflammatory subepidermal autoimmune bullous disease mimicking bullous pemphigoid reacted with a 200 kDa protein on immunoblotting with dermal extract, as is characteristic of anti-p200 pemphigoid. One serum showed both roof and floor binding, and reacted with the BP180 antigen. LIMITATION: We could not perform serration pattern analysis in our patients. CONCLUSION: In this study, we report three cases of anti-p200 pemphigoid from India. These cases, though indistinguishable clinically from bullous pemphigoid, revealed a floor-binding pattern on indirect immunofluorescence using salt-split skin.