Hemorrhagic Vesiculobullous Lesions of Ecthyma Gangrenosum in a Diabetic Patient with Myelofibrosis: A Rare Presentation of Septic Shock without Pseudomonas aeruginosa Bacteremia.

BACKGROUND Ecthyma gangrenosum is a rare skin lesion associated with Pseudomonas aeruginosa, an aerobic gram-negative opportunistic bacterial pathogen. In non-bacteremia patients, sepsis is not a common complication. Immunocompromised patients are more commonly affected. If diagnosis and therapy are delayed, the mortality rate is 18-96%. This report is of a 52-year-old man with diabetes mellitus and myelofibrosis presenting with hemorrhagic vesiculobullous lesions of ecthyma gangrenosum on the upper and lower extremities, oral mucosa, and anogenital area with, interestingly, no associated Pseudomonas aeruginosa bacteremia. CASE REPORT A 52-year-old diabetes patient with myelofibrosis presented with hemorrhagic vesiculobullous and necrotic eschar-covered erosions over the upper and lower extremities, oral mucosa, and anogenital area. Although he appeared septic looking initially, with signs of end-stage organ failure, and he was later determined to have septic shock, the clinical diagnosis was not possible without a positive culture swab of the cutaneous lesions showing growth of Pseudomonas aeruginosa. The diagnosis of cutaneous ecthyma gangrenosum-induced septic shock was confirmed, though bacteremia was not detected. This patient was successfully managed with the early initiation of proper antibiotics. CONCLUSIONS Early detection and vigilance when confronted with the clinical presentation of ecthyma gangrenosum are a vital part of patient management to reduce the high mortality risk of the disease. Although bacteremia is associated with a high risk for fatalities, cutaneous ecthyma gangrenosum can be complicated by septic shock and serious adverse events. The involvement of multidisciplinary teams in patient management is an essential aspect of ecthyma gangrenosum disease management.