Genital and Intertriginous Rashes Refractory to Antimicrobial Treatments: Have You Thought about Crohn's Disease?

Crohn's disease is an inflammatory bowel disease that can have multiple extraintestinal manifestations and can develop prior to, following, or simultaneously with gastrointestinal tract involvement (Aberumand et al. (2017), Georgious et al. (2006), Larsen et al. (2010), Levine and Burakoff (2011), Louis et al. (2018)). This report examines the case of a 16-year-old male with a rash of the genital, intergluteal, and inguinal regions refractory to antimicrobial treatments suspicious for an extraintestinal manifestation of Crohn's disease. The patient was diagnosed with inflammatory, nonfistulizing colonic Crohn's disease following presentation with gastrointestinal symptoms including abdominal pain and bloody stools 6 months after the onset of the rash. The genital lesions resolved after starting treatment for Crohn's disease with adalimumab.

Liver transplantation as a novel strategy for resolution of congenital afibrinogenemia in a pediatric patient.

Fibrinogen replacement therapy is a treatment mainstay for patients with afibrinogenemia and significant bleeding. A male infant with congenital afibrinogenemia and several spontaneous hemarthroses commenced cryoprecipitate prophylaxis but developed severe urticarial reactions. He transitioned to a human fibrinogen concentrate (HFC) (RiaSTAP® , CSL Behring; 70 mg/kg biweekly) but continued experiencing hemarthroses (estimated annualized bleeding rate [ABR]: 5-6) and severe anaphylactic reactions, despite pre- and postinfusion medications. Following switching to a new HFC (Fibryga® , Octapharma; 50 mg/kg biweekly), ABR was 0-1 with no further infusion reactions. Aged 9 years, because of limited quality of life, development of obesity and fatty liver disease, he underwent orthotopic liver transplant (OLT) under HFC coverage. Pharmacokinetic analysis guided presurgical fibrinogen levels > 150 mg/dL. No intraoperative HFC infusions were required. Coagulation profile and fibrinogen levels remained within normal limits during and posttransplant. To our knowledge, this is the first pediatric report of afibrinogenemia successfully treated with OLT.