Nocturnal Vulval Pain in Girls-A Subset of Vulvodynia?

OBJECTIVES: The aims of the authors' case series were to outline the clinical features of prepubertal nocturnal vulval pain syndrome and to look at management and outcomes. METHODS: Clinical details of prepubertal girls experiencing episodes of nocturnal vulval pain with no identifiable cause were recorded and analyzed. Parents completed a questionnaire to look at outcomes. RESULTS: Eight girls with age at onset of symptoms between 3.5 and 8 years (mean 4.4 years) were included. Each patient described intermittent episodes of vulval pain lasting between 20 minutes and 5 hours, starting 1-4 hours after falling asleep. They were crying and rubbing or holding the vulva with no obvious cause seen. Many were not fully awake and 75% had no recollection of the events. Management focused on reassurance alone. The questionnaire showed that 83% had full resolution of symptoms with a mean duration of 5.7 years. CONCLUSIONS: Prepubertal nocturnal vulval pain syndrome may be a subset of vulvodynia (generalized, spontaneous, intermittent) to be included in the clinical spectrum of night terrors. Recognizing the clinical key features should aid prompt diagnosis and reassurance of the parents.

Cutaneous Squamous Cell Carcinoma in Epidermolysis Bullosa: a 28-year Retrospective Study.

Epidermolysis bullosa (EB), notably severe recessive dystrophic EB (RDEB-S), is associated with increased risk of aggressive mucocutaneous squamous cell carcinomas, the major cause of mortality in early adulthood. This observational, retrospective case review describes a series of EB patients with cutaneous squamous cell carcinomas over a 28-year period. Forty-four EB patients with squamous cell carcinomas were identified with a total of 221 primary tumours. They comprised: 31 (70%) with RDEB-S, 4 (9%) with other RDEB subtypes, 5 (11.4%) with dominant dystrophic EB, 3 (6.8%) with intermediate junctional EB and 1 (2.3%) with Kindler EB. Squamous cell carcinomas occurred earlier in RDEB-S (median age 29.5 years; age range 13-52 years) than other groups collectively (median age 47.1 years; age range 30-89 years) and most had multiple tumours (mean 5.8; range 1-44). Squamous cell carcinoma-associated mortality was high in RDEB-S (64.5%), with median survival after first squamous cell carcinoma of 2.4 years (range 0.5-12.6 years), significantly lower than previous reports, highlighting the need for early surveillance and better treatments.