Skip to Main Content

++
Patient Story
++

During a medical mission trip to Africa a child was seen with pyoderma gangrenosum on the dermatology ward of a hospital. She has had a long history of pyoderma gangrenosum with ulcerations on her face, neck, and chest (Figure 149-1). The scarring has caused adhesions between the face, neck, and chest.

++
FIGURE 149-1

Pyoderma gangrenosum on the face, neck, and chest of a child in Africa. The scarring has caused adhesions between the face, neck, and chest. (Used with permission from Richard P. Usatine, MD.)

Graphic Jump Location
++
Introduction
++

Pyoderma gangrenosum (PG) is an uncommon ulcerative disease of the skin of unknown origin that affects both children and adults. It is a type of neutrophilic dermatosis.

++
Epidemiology
++

  • PG occurs in approximately 1 person per 100,000 people of all ages each year.1

  • Children account for only 3 to 4 percent of the total number of cases. There is nothing clinically distinctive about pyoderma gangrenosum in children and adolescents other than the age of the patients.2

  • No racial predilection is apparent.

  • A slight female predominance may exist.

  • Predominately occurs in fourth and fifth decade, but all ages may be affected.

++
Etiology and Pathophysiology
++

  • Etiology is poorly understood.

  • Pathergy (initiation at the site of trauma or injury) is a common process and it is estimated that 30 percent of patients with PG experienced pathergy.1

  • Up to 50 percent of all cases are idiopathic.3

  • At least 50 percent of cases are associated with systemic diseases such as inflammatory bowel disease, hematologic malignancy, and arthritis.3

  • It occurs in up to 5 percent of patients with ulcerative colitis and 2 percent of those with Crohn disease (Figure 149-2).4,5

  • In one study of 46 patients 18 years of age or younger with pyoderma gangrenosum, an underlying systemic disease was present in 74 percent of the older children, and as in adults it was most commonly ulcerative colitis.2

  • The lesions tend to affect the lower extremities (Figure 149-3), but infants tend to have ulcers more commonly than adults in the genital and perianal distribution, 6 the head and face (Figure 149-1), and the buttocks (Figure 149-4).7

  • Biopsies usually show a polymorphonuclear cell infiltrate with features of ulceration, infarction, and abscess formation.

++
FIGURE 149-2

Friable inflamed mucosa of the colon in Crohn disease. (Used with permission from Shashi Mittal, MD.)

Graphic Jump Location
++
FIGURE 149-3

Pyoderma gangrenosum on the foot of a child. The lower extremity is the most common location for this rare disease in children. (Used with permission from Weinberg SW, Prose NS, Kristal ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessPediatrics Full Site: One-Year Subscription

Connect to the full suite of AccessPediatrics content and resources including 20+ textbooks such as Rudolph’s Pediatrics and The Pediatric Practice series, high-quality procedural videos, images, and animations, interactive board review, an integrated pediatric drug database, and more.

$595 USD
Buy Now

Pay Per View: Timed Access to all of AccessPediatrics

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.