The Neutrophilic Dermatoses

David Farhi, MD; Daniel Wallach, MD

Disclosures

Dermatology Nursing. 2008;20(4):274-282. 

In This Article

Pyoderma Gangrenosum (PG)

Pyoderma gangrenosum, one of the most dramatic skin conditions, was described in 1930 (Brunsting, Goeckerman, & O'Leary, 1930).

PG is characterized by a centrifugal ulceration aggravated by minimal trauma. Histology shows a subacute or chronic deep dermal neutrophilic infiltrate.

PG typically begins with pustules, which evolve rapidly to a painful deep ulceration that extends in a centrifugal pattern (see Figure 2A) (Callen, 1998). The size of the lesions may vary from 1 cm to more than 20 cm. The boundaries of the ulceration are elevated and violaceous. The inner aspect of the peripheral border is classically undermined by pustules. In general, systemic symptoms (fever, asthenia) are discrete or absent. PG lesions are chiefly located on lower limbs, although PG is an ubiquitous dermatosis.

Figure 2.

(2A) Pyoderma gangrenosum: Typical (ulcerated). (2B) Pyoderma gangrenosum: Atypical (granulomatous).

Figure 2.

(2A) Pyoderma gangrenosum: Typical (ulcerated). (2B) Pyoderma gangrenosum: Atypical (granulomatous).

Atypical PG may present as either pustular and/or bullous plaques, or in filtrated plaques (see Figure 2B). The later form has been called "vegetative" (Wilson-Jones & Winkelmann, 1988) or "granulomatous."

The tendency of PG to develop in a previously uninvolved site of mild trauma has been termed "pathergy," and occurs in 25% to 50% of the cases (Brooklyn, Dunnill, & Probert, 2006).

The diagnosis of PG relies mainly on the clinical picture. Skin biopsy aids to rule out an ulcerated tumor or an infection (Wallach & Vignon-Pennamen, 2006). Only 20% to 30% of PG cases are idiopathic. Other cases occur in the context of a systemic disease that can be revealed by PG: blood disorders, digestive inflammatory diseases, chronic rheumatologic conditions, etc. (see Table 2 ).

First-line treatment usually relies on systemic steroids (Reichrath, Bens, Bonowitz, & Tilgen, 2005). Refractory cases may be treated with cyclosporine or infliximab (Brooklyn et al., 2006).

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