ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (14)
Pyoderma gangrenosum in Child
See also in: Cellulitis DDx,Anogenital
Print Captions OFF
Other Resources UpToDate PubMed

Pyoderma gangrenosum in Child

See also in: Cellulitis DDx,Anogenital
Print Images (14)
Contributors: Jeffrey M. Cohen MD, Vivian Wong MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Pyoderma gangrenosum (PG) is an inflammatory, noninfectious, ulcerative neutrophilic skin disease of uncertain etiology commonly misdiagnosed as an aggressive skin infection. Pustules form and give way to ulcers with a necrotic, undermined margin. PG can affect any age, but is rare in the pediatric population. It may take on a number of differing clinical presentations. PG can have either an acute or chronic course and result in extensive scarring (which can be keloidal or have dyspigmentation, especially in patients with darker skin types). There is no predilection for sex or any population. The disease occurs most often in middle-aged adults.

The two primary variants are a classic ulcerative form, which often involves the lower extremities, and a vesicobullous form, which is more superficial and tends to occur on the upper extremities, including hands. Fever, toxicity, and pain can be associated with the onset of PG. Extracutaneous manifestations may take the form of sterile neutrophilic abscesses, such as in the lungs, heart, gastrointestinal tract, liver, eyes, central nervous system, and lymphatic tissue.

Although the exact cause is unknown, neutrophil dysfunction, inflammation, and genetics are all thought to play a role. Additionally, PG has associations with a number of systemic illnesses. In the majority of pediatric cases, there is an association with ulcerative colitis or Crohn disease, but other associations in this population include inherited and acquired immunodeficiencies, leukemia and other hematologic disorders, and a range of rheumatologic disorders including juvenile idiopathic arthritis and systemic lupus erythematosus.

PG tends to be self-limited. First-line therapies are widely accepted, while alternative therapeutic recommendations are largely based on anecdotal evidence. Surgical intervention is a common exacerbating factor because PG demonstrates pathergy, a phenomenon by which skin trauma can lead to worsening disease.

Codes

ICD10CM:
L88 – Pyoderma gangrenosum

SNOMEDCT:
74578003 – Pyoderma gangrenosum

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Ulcers:
Pustules / nodules:
Pyostomatitis vegetans:
The differential diagnosis for an immunocompromised patient also includes:

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required

References

Subscription Required

Last Reviewed: 03/05/2016
Last Updated: 03/25/2019
Copyright © 2019 VisualDx®. All rights reserved.
Pyoderma gangrenosum in Child
See also in: Cellulitis DDx,Anogenital
Captions OFF Print 14 Images Filter Images
View all Images (14)
(with subscription)
 Reset
Pyoderma gangrenosum : Painful skin lesions, Skin ulcer
Clinical image of Pyoderma gangrenosum
A close-up of a large ulcer with surrounding re-epithelialization.
Copyright © 2019 VisualDx®. All rights reserved.