Pseudomonas aeruginosa septic shock associated with ecthyma gangrenosum in an infant with agammaglobulinemia

Abstract

SUMMARYEcthyma gangrenosum (EG) due to Pseudomonas aeruginosa is a rare and invasive infection that can be associated with agammaglobulinemia. The cornerstone of the treatment is based on prompt recognition with appropriate antibiotic coverage and intravenous immunoglobulin. The authors report a case of EG emphasizing the clinical and therapeutic aspects of this condition.

Ecthyma Gangrenosum in a 67-Year-Old Man with Chronic Lymphocytic Leukemia

“…Pseudomonas septicemia reflects a state of immunocompromise and most commonly presents in patients with neutropenia, burns, prolonged courses of antibiotics, chemotherapy, complement deficiencies, or hematologic malignancies, as in our patient (3,4). Ecthyma gangrenosum has been reported to occur in up to 30% of patients with Pseudomonas septicemia, and mortality rates range from 38% to 96% (5,6). Ecthyma gangrenosum also can occur in the absence of bacteremia, but skin lesions are few or solitary, thought to be due to direct inoculation, and the mortality rate is significantly lower (3,6).…”

“…However, the macules rapidly progress in size and develop central bullae that rupture, revealing painful, infarcted, and necrotic lesions with hemorrhagic borders (7). The most common sites of involvement are the gluteal cleft and perineum, seen in more than 50% of affected patients, followed by the axilla, trunk, and extremities (5,8).…”

Ecthyma gangrenosum, a skin manifestation of Pseudomonas aeruginosa sepsis in a previously healthy child

“…It generally causes infection in immunocompromised patients with conditions such as neutropenia, immunodeficiency, and hypogammaglobulinemia. [4] The presence of Pa infection in healthy subjects is very uncommon.…”

X-linked agammaglobulinaemia (XLA) presenting with neutropenia and Pseudomonasaeruginosa cellulitis

“…Fulminant pseudomonal sepsis in the setting of XLA has also been reported 3 4. We suggest measurement of serum immunoglobulin and when abnormal, lymphocyte subsets, in the evaluation of otherwise unexplained neutropaenia with soft tissue infections and sepsis, especially in boys.…”