Localized vulvar bullous pemphigoid of childhood: A rare cause of persistent vulvar erosions in children.

Localized vulvar bullous pemphigoid of childhood is an excessively rare variant of bullous pemphigoid and affects almost exclusively young girls of 7-12 years of age. In contrast to adult-onset bullous pemphigoid, a prompt response to potent topical corticosteroids is observed in the majority of cases, with a favorable prognosis and rare relapses. We report the case of a 7-year-old girl who presented with this condition. Our case reinforces the recognition of this rare subtype of childhood bullous pemphigoid as a distinct entity and enlightens the importance of performing a cutaneous biopsy when clinically indicated.

Patterned cutaneous hypopigmentation phenotype characterization: A retrospective study in 106 children.

BACKGROUND: Cutaneous patterned hypopigmentation's phenotype is highly variable and may be associated with extracutaneous anomalies. OBJECTIVE: We evaluated the phenotypic and clinical characteristics of patients with cutaneous patterned hypopigmentation to determine whether certain patterns were more likely to be associated with underlying anomalies. METHODS: The charts of 106 children with cutaneous patterned hypopigmentation were reviewed retrospectively (2007-2018) at Sainte-Justine University Hospital Centre, in Montreal, Canada. Retrieved information included sex, age at diagnosis, phototype, pattern, and distribution of the cutaneous lesions and the presence of extracutaneous findings. Data were recorded on a software tool which collects and analyzes phenotypic information. RESULTS: The predominant types of cutaneous patterned hypopigmentation were along Blaschko's lines in narrow (38.7%) and broad bands (53.8%). Mixed patterns were observed in 22.5% of children. The anterior trunk and posterior trunk were most frequently affected (69% and 56%, respectively). Extracutaneous involvement, especially neurological and developmental, was present in 28.3% of patients and was significantly associated with ≥ 4 involved body sites. CONCLUSION: Distribution and types of cutaneous patterned hypopigmentation were not predictive of extracutaneous findings, with the exception of multiple sites involvement and possibly centrofacial location and blocklike lesions. Follow-up until school entry should help identify subtler associated extracutaneous anomalies.

Pediatric SJS/TEN Subdued by a Combination of Dexamethasone, Cyclosporine, and Etanercept.

We report a case of an 17-year-old male with a drug reaction in the spectrum of Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), triggered by carbamazepine, who was succesfully treated with the combination of dexamethasone, cyclosporine, and etanercept. This triple therapy halted and prevented skin epidermolysis without immediate or late onset complications.