NLRP1-associated autoinflammatory disease with epithelial dyskeratosis.

Several gain-of-function variants in NLRP1 cause a distinctive autoinflammatory disease reported under different names featuring mainly skin and mucosal involvement and variable systemic signs. Here, we report a new case of NLRP1-associated autoinflammatory disease in a 6-year-old Peruvian girl, who presented with confluent hyperkeratotic plaques that drained purulent material with subsequent scarring. A c.

Dermatologic Review in Pediatric Vascular Lesions.

Vascular anomalies (VAs) can be present in any organ; however, the skin being the largest one, it is there where many of them are evident; some are visible at birth, others develop throughout life. Pediatric dermatologists are specially trained to distinguish VAs from their mimickers, which require different treatments and may harbor distinct prognoses. We resume the diagnostic and therapeutic tasks of pediatric dermatologist at our vascular anomaly clinics, as well as the differential diagnoses of mimickers of VAs.

[Disseminated infection by Bacillus Calmette-Guérin in an immunodeficient infant: case report].

The Bacillus Calmette-Guerin (BCG) vaccine given to newborns in countries with a high incidence of tuberculosis may cause local reactions up to disseminated infection in immunocompromised patients. We report the case of a six-monthold male infant with a history of having received the BCG vaccine at birth, and presenting repeated infectious, soft violet nodules in the trunk and extremities with the presence of acid-alcohol-resistant bacilli (BAAR) in histopathology and skin culture; the molecular study reported the presence of Mycobacterium bovis BCG. In the tomography, interstitial opacities were observed in the lungs and in the gastric lavage BAAR was identified.