II. Epidemiology

  1. Rare

III. Pathophysiology

  1. Neutrophilic dermatosis
    1. Also occurs in Behcet Disease and Sweet Syndrome
    2. Neutrophils release elastase and other enzymes that result in skin breakdown

IV. Symptoms

  1. Painful Pustules and ulcers that form on the legs, buttocks, Abdomen and face in response to minor Skin Trauma

V. Signs

  1. Lesion characteristics
    1. Initial
      1. Hemorrhagic, red Pustule or painful, tender Nodule
    2. Next
      1. Lesion ulcerates
      2. Ulcer has irregular, raised, undermined border and advances peripherally
      3. Ulcer base with exudate that is pustular and hemorrhagic
      4. Necrotic eschar may form
    3. Next
      1. Ulcer heals with thin, atrophic scar
  2. Distribution (in order of most common to least common)
    1. Legs
    2. Buttocks
    3. Abdomen
    4. Face
  3. Pathergy
    1. See Pathergy Test
    2. Blistering and ulceration in response to minor Trauma
    3. Also seen with Behcet Disease

VII. Differential Diagnosis

VIII. References

  1. Fitzpatrick (1992) Color Atlas and Synopsis of Clinical Dermatology
  2. Jhun and DeClerck in Herbert (2015) EM:Rap 15(8): 19-20
  3. Heiner (2015) Ann Emerg Med 66(2):115-8 [PubMed]

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Ontology: Pyoderma Gangrenosum (C0085652)

Definition (MSH) An idiopathic, rapidly evolving, and severely debilitating disease occurring most commonly in association with chronic ulcerative colitis. It is characterized by the presence of boggy, purplish ulcers with undermined borders, appearing mostly on the legs. The majority of cases are in people between 40 and 60 years old. Its etiology is unknown.
Concepts Disease or Syndrome (T047)
MSH D017511
ICD9 686.01
ICD10 L88
SnomedCT 74578003
English Pyoderma Gangrenosum, PYODERMA GANGRENOSUM, pyoderma gangrenosum (diagnosis), pyoderma gangrenosum, Pyoderma gangenosum, Pyoderma gangrenous, Pyoderma Gangrenosum [Disease/Finding], pyoderma gangenosum, pyoderma gangrenosa, pyoderma gangrenous, gangrenosum pyoderma, gangrenous pyoderma, Pyoderma gangrenosum, Pyoderma gangrenosum (disorder), gangrenosum; pyoderma, pyoderma; gangrenosum
Spanish PIODERMA GANGRENOSO, piodermia gangrenosa (trastorno), piodermia gangrenosa, Pioderma gangrenoso, Pioderma Gangrenoso
German PYODERMA GANGRENOSUM, Pyoderma gangraenosum, Pyoderma gangenosum, Pyoderma gangrenosum
Dutch pyoderma gangreneus, gangraenosum; pyodermie, pyodermie; gangraenosum, Pyoderma gangraenosum, pyoderma gangrenosum, Pyoderma gangrenosum
French Pyodermite gangréneuse, PYODERMA GANGRENOSUM, Pyoderma gangrenosum, Pyodermie phadégénique, Pyoderma Gangrenosum
Portuguese Pioderma gangrenosa, PIODERMITE GANGRENOSA, Piodermite gangrenosa, Pioderma Gangrenoso
Japanese 壊疽性膿皮症, エソセイノウヒショウ
Swedish Pyoderma gangrenosum
Czech pyoderma gangrenosum, Gangrenózní pyodermie, Pyoderma gangrenosum
Finnish Pyoderma gangraenosum
Russian PIODERMIIA MOLNIENOSNAIA IAZVENNAIA, DERMATIT TOKSIKO-ALLERGICHESKII PUSTULEZNYI IAZVENNYI, PIODERMIIA GANGRENOZNAIA, ДЕРМАТИТ ТОКСИКО-АЛЛЕРГИЧЕСКИЙ ПУСТУЛЕЗНЫЙ ЯЗВЕННЫЙ, ПИОДЕРМИЯ ГАНГРЕНОЗНАЯ, ПИОДЕРМИЯ МОЛНИЕНОСНАЯ ЯЗВЕННАЯ
Korean 괴저성 농피증
Polish Piodermia zgorzelinowa
Hungarian Pyoderma gangraenás, Pyoderma gangraenosum, Pyoderma gangrenosum
Norwegian Pyoderma gangrenosum
Italian Pioderma gangrenoso