6 - 37 - PYODERMA GANGRENOSUM Flashcards
what are the variants of pyoderma gangrenosum
- classic ulcerative
- bullous
- pustular
- vegetative
give examples of associated systemic diseases of PG
- IBD
- Hematologic disease
- Malignancy
- Monoclonal gammopathy
- Inflammatory arthritis
- Hidradenitis suppurativa
MC hematologic malignancy associated with pyoderma gangrenosum
Acute myeloid leukemia
MC monnoclonal gammopathy associated with pyoderma gangrenosum
IgA monoclonal gammopathy
MC variant og pyoderma gangrenosum
ulcerative
MC location of ulcerative PG
leg
MC location of bullous PG
upper limbs
this variant of PG is commonly associated with hematologic disease
bullous PG
This variant of PG may show clinical and histologic overlap with Sweet syndrome
Bullous variant
sweet syndrome - one of the neutrophilic dermatoses which is itself often associated with hematologic disease
a generalized eruption that occurs almost exclusively in the setting of an exacerbation of acute IBD (usually UC)
Pustular PG
sometimes called atypical PG
bullous PG
other term for vegetative PG
superficial granulomatous pyoderma
area of predilection of pustular PG
trunk
to a lesser extent, the face and limbs
MC location of vegetative PG
trunk
In contrast to the other variants, this uncommon variant is gradual in its onset, mild in the discomfort it generates, and not usually associated with the presence of systemic disease.
vegetative PG
This form of PG is** usually more responsive to localized or mild forms of systemic therapy **than the other variants
vegetative PG
diagnostic criteria of PG
2 Major and 2 Minor required
1. Major criteria:
a. Sudden onset of a painful lesion with the characteristic morphology described in “Cutaneous Findings” section in a patient who does not have fever, significant toxemia, or relevant drug intake.
b. Histopathologic exclusion of significant vasculitis, malignancy, and infective organisms by special histologic studies/stains and negative tissue
2. Minor criteria that are supportive of the diagnosis are as follows:
a. Occurrence in an individual with systemic disease, as described in “Disease Associations” section.
b. Classic histologic PG findings. (Note: In patients who have received systemic corticosteroids, the presence of neutrophils may be blunted and mononuclear cells may instead predominate.)
c. Rapid reduction of pain and inflammation on initiation of high-dose systemic corticosteroid therapy and rapid ulcer healing response to high-dose systemic corticosteroid therapy with a 50% decrease in ulcer size within 1 month.
d. History suggestive of pathergy or a clinical finding of cribriform scarring.
Treatment Options for Pyoderma Gangrenosum
