Kechichian E, Haber R, Mourad N, El Khoury R, Jabbour S, Tomb R, et al.
International journal of dermatology. Date of publication 2017 May 1;volume 56(5):486-495.
1. Int J Dermatol. 2017 May;56(5):486-495. doi: 10.1111/ijd.13584. Epub 2017 Feb 23.
Pediatric pyoderma gangrenosum: a systematic review and update.
Kechichian E(1)(2), Haber R(1)(2), Mourad N(1)(2), El Khoury R(1)(2), Jabbour
S(2)(3), Tomb R(2)(4).
Author information:
(1)Department of Dermatology, Hotel Dieu de France University Hospital, Beirut,
Lebanon.
(2)Faculty of Medicine, Saint Joseph University, Beirut, Lebanon.
(3)Department of Plastic and Reconstructive Surgery, Hotel Dieu de France
University Hospital, Beirut, Lebanon.
(4)Chief of Department of Dermatology, Hotel Dieu de France University Hospital,
Beirut, Lebanon.
Pyoderma gangrenosum (PG) is a sterile neutrophilic disorder that rarely affects
children. Clinical, epidemiological, and therapeutic data on pediatric PG is poor
as there are many newly reported associated diseases and drugs. This paper aims
to review all recent available data on pediatric PG. A systematic review of the
literature was conducted using Embase, Medline, and Cochrane databases. A total
of 132 articles were included in the review. The most commonly reported
underlying diseases in pediatric PG are inflammatory bowel diseases followed by
hematologic disorders, vasculitis, immune deficiencies and Pyogenic Arthritis,
Pyoderma gangrenosum and Acne (PAPA) syndrome. More than half of the cases occur
with no underlying disease. The most frequently reported clinical presentation is
multiple disseminated ulcers. Treatment should be tailored according to the
underlying etiology. It includes systemic steroids, corticosteroid sparing agents
such as dapsone and cyclosporine, and TNF-alpha inhibitors such as adalimumab and
infliximab. Response to treatment is high with cure rates reaching 90%. A high
index of suspicion and a thorough workup are mandatory in the management of
pediatric PG.
© 2017 The International Society of Dermatology.
DOI: 10.1111/ijd.13584
PMID: 28233293 [Indexed for MEDLINE]