Rheumatoid neutrophilic dermatitis M06.8

Very rare neutrophilic dermatosis (trunk and extremity exanthema with papules, plaques, pustules) in patients with severe forms of seropositive primary chronic polyarthritis (rheumatoid arthritis). Rheumatoid arthritis usually occurs well before the skin symptoms (7-8 years).

Notice. The entity of this disease is still controversial.

In particular, neutrophilic rheumatoid dermatitis must be distinguished from the adult form of Still's syndrome or, more likely, it is identical with it.

Very rare. So far, only single case reports are published in the literature.

Age at manifestation averages 57.4 years.

w:m=2:1;

Hips, on the extensor sides of the upper and lower extremities (preferably lower legs and forearms, preferably near the joints).

Mostly persistent, symmetrically distributed, rather sharply circumscribed, 0.5-1.5 cm, reddish-purple plaques and papules of moderately coarse consistency. Less common are vesicles, pustules, or ulcers. Less commonly, subepithelial blistering occurs.

Relative and absolute neutrophil leukocytosis in the blood count; rheumatoid factor highly positive, anti-CCP-Ac significantly increased. BSG and CRP increase. Case studies with seronegative rheumatoid factor have also been described in the literature.

Dense, interstitial or periadnexal accentuated, dermal neutrophilic infiltrate with leukocytoclasia but without signs of vasculitis. Neutrophilic granulocytes are also located along the dermo-epidermal junctional zone. Focal epitheliotropy with spongiotic vesiculation of pustule formation is possible. Also abscess-like condensations of neutrophilic granulocytes . The adjacent subcutaneous adipose tissue may be involved (septal panniculitis).

Acute febrile neutrophilic dermatosis

Erythema elevatum et diutinum

Urticariavasculitis

Interstitial granulomatous dermatitis with arthritis.

Symptomatic antiphlogistic external and internal therapy with glucocorticoids in combination with methotrexate (15-20mg/week s.c.).

Alternative: Dapsone or colchicine

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2. Below J et al (2015) Rheumatoid neutrophilic dermatitis. Dermatologist 66: 228-230
3. Brown TS et al (2001) Rheumatoid neutrophilic dermatitis in a woman with seronegative rheumatoid arthritis. J Am Acad Dermatol 45: 596-600.
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6. Lu CI et al (2004) A bullous neutrophilic dermatosis in a patient with severe rheumatoid arthritis and monoclonal IgA gammopathy. J Am Acad Dermatol 51(2 Suppl): 94-96.
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9. Youn CS et al (2000) Colchicine treatment in a patient with neutrophilic dermatosis associated with rheumatoid arthritis. J Dermatol 27: 782-787
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11. Żuk G et al (2019) Rheumatoid neutrophilic dermatitis. Reumatologia 57:350-353.