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Palisaded neutrophilic granulomatous dermatitis
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Palisaded neutrophilic granulomatous dermatitis

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Contributors: Yun Xue MD, Susan Burgin MD
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Synopsis

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an extremely rare inflammatory cutaneous reaction seen in the setting of connective tissue disease or rheumatoid arthritis. It is almost never seen independently from an underlying systemic illness.

Although the exact etiology is unknown, PNGD is likely a reactive process with histological patterns ranging from early neutrophilic inflammation and leukocytoclastic vasculitis to collagen degeneration, granuloma formulation, and associated fibrosis.

PNGD is seen in patients of all ages, though reports of affected children are rare. Women are affected more than men in a 3 to 1 ratio, which likely reflects the demographics of common underlying autoimmune diseases. Systemic lupus erythematosus and rheumatoid arthritis are the most common associations, although inflammatory bowel disease and systemic vasculitides have also been reported.

PNGD presents as symmetrically distributed, skin-colored to erythematous, umbilicated or crusted papules, typically on the elbows and extensor surfaces.

Codes

ICD10CM:
L30.9 – Dermatitis, unspecified

SNOMEDCT:
402432002 – Rheumatoid neutrophilic dermatitis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Interstitial granulomatous dermatitis – Classically presents as linear subcutaneous cords or bands on the proximal trunk but may have substantial morphological overlap with PNGD. Proximal limbs and trunk tend to be involved. The absence of vasculitis on histology distinguishes it from PNGD.
  • Interstitial granulomatous drug reaction – Erythematous to violaceous annular or nonannular plaques on the trunk, inner upper arms, proximal thighs, and intertriginous skin; typically preceded by a new drug exposure.
  • Granuloma annulare – Nonscaly erythematous papules or plaques on the trunk or extremities. Histology demonstrates a lymphocytic infiltrate with mucin deposition rather than neutrophilic infiltrate.
  • Bowel-associated dermatosis arthritis syndrome

Best Tests

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Therapy

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References

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Last Updated: 07/12/2018
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Palisaded neutrophilic granulomatous dermatitis
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Palisaded neutrophilic granulomatous dermatitis : Erythema, Symmetric extremities, Smooth papules, Umbilicated papules
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