Seven cases of ecthyma gangrenosum caused by Pseudomonas aeruginosa in
infants and young children are reviewed, The skin lesions typically c
onsisted of necrotic ulcers surrounded by a red areola, Three patients
also had septicemia, All seven patients had underlying abnormalities,
including panhypogammaglobulinemia in a five-year-old, hyaline membra
ne disease requiring intensive care therapy in a premature infant, Sch
wachman syndrome in a 7-month-old, chronic neutropenia in a 16-month-o
ld, defective polymorphonuclear cell chemotaxis in a 23-month-old, cyc
lic neutropenia in an 18-month-old with atopy, and neutropenia with ly
mphopenia and absent T-cell function In a six-month-old, Ecthyma gangr
enosum was the inaugural manifestation of the immune deficiency in fou
r patients, All patients received antimicrobial therapy, and three als
o required surgical drainage. The outcome was favorable in every case,
Ecthyma gangrenosum is a rare, often misdiagnosed condition with high
ly suggestive clinical features. Most patients have a debilitating dis
ease and/or an immune deficiency. The outcome is frequently fatal in t
he absence of an early diagnosis with prompt initiation of appropriate
antimicrobial therapy. The immune status of the patient should be inv
estigated after the episode since ecthyma gangrenosum can be the first
manifestation of an immune deficiency.