INTRODUCTION
Ecthyma gangrenosum (EG) is a relatively uncommon cutaneous infection
that mostly occurs in individuals with immunosuppression due to
different underlying diseases including hematologic disorders,
malignancies, lymphoproliferative disorders and autoimmune diseases.
However, it has also been reported among previously healthy
individuals1-4, both adults and children.Pseudomonas aeruginosa is the most common cause of EG, with or
without bacteremia, although it is also known to be caused by a variety
of pathogens, including different bacteria, fungi and
viruses1. Lesions first appear as painless papules
which rapidly evolve in gangrenous ulcers. The macroscopic appearance
corresponds to the microvascular thrombosis and subsequent dermal
ischemic necrosis5. Single or multiple lesions might
involve one or more sites in the body, mainly the axillary and perineal
regions. Prompt recognition and timely adequate treatment are crucial
for the prognosis1.
In patients with hematological malignancies and cancer, many factors
(immunosuppression, mucositis and epithelial damage, microbiome
dysbiosis and prolonged use of central venous devices) predispose to
bacterial infections, usually affecting integumental surfaces such as
respiratory tract, gastrointestinal tract and skin. The frequently
atypical clinical presentation of these infections makes the prompt
recognition often challenging6.
There are scant reports describing the occurrence of EG in
onco-hematological patients, mainly as case reports or small case
series7-16. We report the results of a large
multicenter pediatric retrospective collection of 38 cases of proven EG
diagnosed between 2009 and 2019 in children with hematological disease
and cancer. The aim is to describe the clinical picture and
characteristics of EG, to early recognize and diagnose it, since timely
treatment influences the outcome of this infection. In fact,
particularly in this setting of patients, prompt interventions may
change the course of both this serious infection and the underlying
disease. The wide spectrum of clinical pictures at different levels of
severity in our sample allowed a more complete evaluation of EG
infection at different stages, considering that cases reported in the
literature have mainly a devastating clinical presentation.