Hook, Kristen P.

AbstractBackgroundSystemic sirolimus (rapamycin) has recently been found effective in treating complex vascular anomalies by reducing the size and associated complications. Many vascular anomalies have a cutaneous component, and thus, we sought to determine whether topical administration of sirolimus may be an effective therapy, as data on the use of topical sirolimus are limited.ObjectiveWe reviewed the efficacy and tolerability of topical formulations of sirolimus in the treatment of various simple and combined vascular malformations and tumors.MethodsEighteen patients with any vascular anomaly treated exclusively with topical sirolimus were retrospectively reviewed.ResultsEleven patients had combined venous lymphatic malformations, three had tufted angiomas, two had a lymphatic malformation, one had a venous malformation, and one had a verrucous venous malformation. All (100%) patients reported some degree of improvement and 50% of patients reported marked improvement in one or more symptoms, most commonly blebs and lymphatic drainage, and bleeding.LimitationsThe retrospective nature, small number of patients, and differences in topical preparations limit the broad application of the results.ConclusionTopical sirolimus appears to be a safe and useful non‐invasive therapy that is well‐tolerated in the treatment of the cutaneous portion of a variety of vascular anomalies.

AbstractPemphigus herpetiformis (PH) is characterized by grouped vesicular or papular pruritic lesions with histologic and immunopathologic features of pemphigus. PH can manifest at any age, and paraneoplastic cases have been reported. We describe a healthy boy born with acral crateriform erosions of the hands and feet whose 35‐year‐old mother had similar lesions. Biopsies from both patients were most consistent with PH. The mother was diagnosed with high‐grade B‐cell non‐Hodgkin lymphoma and began chemotherapy with dexamethasone, and her lesions quickly improved. The infant had no additional lesions after 3 weeks of age and his acral erosions healed. To our knowledge, this is the first report of a diagnosis of PH in an infant and the first case of transplacental transmission of PH. This is also the first report of paraneoplastic PH in lymphoma. This case adds to the growing differential diagnosis of skin disease in postpartum women and their neonates.