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Pyoderma gangrenosum in Infant/Neonate
See also in: Cellulitis DDx,Anogenital
Other Resources UpToDate PubMed

Pyoderma gangrenosum in Infant/Neonate

See also in: Cellulitis DDx,Anogenital
Contributors: Erin X. Wei MD, Jeffrey M. Cohen MD, Vivian Wong MD, PhD, Nnenna Agim MD, FAAD, 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. PG occurs most often in middle-aged adults, but it can affect individuals of any age, although it is rare in the pediatric population (estimated to account for less than 5% of PG cases). There is no predilection for sex or any race or ethnicity.

PG can take on a number of differing clinical presentations. The 2 most common presentations in adults are a classic ulcerative form, which often involves the lower extremities, and a vesiculobullous form, which is more superficial and tends to occur on the upper extremities, including the hands, and is most commonly seen in patients with an associated lymphoproliferative disorder. Other variants include pustular, vegetative, and peristomal PG. PG can affect any age, but it is rare in the pediatric population. Pediatric PG has more pustular lesions and has a strong association with inflammatory bowel disease (IBD).

Fever, toxicity, and pain can be associated with the onset of PG. Rarely, PG can involve the eyes. Extracutaneous manifestations may take the form of sterile neutrophilic abscesses, such as in the lungs, heart, gastrointestinal (GI) tract, liver, eyes, central nervous system (CNS), and lymphatic tissue.

Necrotizing neutrophilic dermatosis describes a subset of patients with severe acute febrile neutrophilic dermatosis (Sweet syndrome) or PG who, in addition to their cutaneous disease, develop fever, leukocytosis (or a leukemoid reaction), and features of shock. Skin pain is a prominent symptom. Additionally, soft tissues underlying areas of skin involvement may be affected with neutrophilic infiltrates and necrosis.

Although the exact cause of PG 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 IBD (Crohn disease or ulcerative colitis), but other associations in this population include juvenile idiopathic arthritis, inherited and acquired immunodeficiencies, leukemia and other hematologic disorders, and a range of rheumatologic disorders, including systemic lupus erythematosus and autoinflammatory syndromes (eg, PAPA syndrome [pyogenic arthritis, PG, and acne], PASH syndrome [PG, acne, and hidradenitis suppurativa], and PAPASH syndrome [pyogenic arthritis, PG, acne, and hidradenitis suppurativa]).

PG can have either an acute or chronic course and result in extensive scarring (which can be cribriform or keloidal with dyspigmentation, especially in patients with darker skin colors). 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

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Ulcers:
  • Infection – As many infectious processes can cause a similar picture (eg, Progressive bacterial synergistic gangrene, Blastomycosis, other deep fungal infections, Cutaneous amebiasis, Sporotrichosis, Atypical mycobacterial infection), PG is a diagnosis of exclusion. If a patient has traveled to tropical countries within the last 6 months, diagnoses such as Cutaneous leishmaniasis, tropical ulcer, and Buruli ulcer must be considered.
  • Calciphylaxis – Rapidly progressive and can be associated with eschars.
  • Chancroid – Usually present around genital skin.
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
  • Herpes simplex virus (HSV) – Usually grouped, punched-out erosions.
  • Ecthyma
  • Ecthyma gangrenosum
  • Cutaneous squamous cell carcinoma – Associated with keratotic plaques.
  • Lymphoma
  • Stasis ulcer or Ischemic ulcer
  • Granulomatosis with polyangiitis (Wegener granulomatosis)
  • Traumatic ulceration
  • Necrobiosis lipoidica – Usually associated with atrophic plaques.
  • Factitial ulcer – Sharp geometric borders.
  • Factitial panniculitis
Pustules / nodules:
  • Cellulitis
  • Folliculitis
  • Furunculosis
  • Arthropod bite or sting or Spider bite
  • Sporotrichosis
  • Mycobacterium marinum infection
  • Impetigo (Bullous impetigo , Nonbullous impetigo)
  • Panniculitis
  • Acute febrile neutrophilic dermatosis
  • Bromoderma
Pyostomatitis vegetans:
  • Aphthous stomatitis
The differential diagnosis for an immunocompromised patient also includes:
  • Chronic Herpes simplex virus
  • Ulcerative Kaposi sarcoma (AIDS-associated Kaposi sarcoma and Non-AIDS Kaposi sarcoma)

Best Tests

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Management Pearls

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Therapy

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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.

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References

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Last Reviewed:10/22/2023
Last Updated:11/26/2023
Copyright © 2024 VisualDx®. All rights reserved.
Pyoderma gangrenosum in Infant/Neonate
See also in: Cellulitis DDx,Anogenital
A medical illustration showing key findings of Pyoderma gangrenosum (Classic Ulcerative Form)
Clinical image of Pyoderma gangrenosum - imageId=798076. Click to open in gallery.  caption: 'A close-up of a large ulcer with surrounding re-epithelialization.'
A close-up of a large ulcer with surrounding re-epithelialization.
Copyright © 2024 VisualDx®. All rights reserved.