Rheumatoid neutrophilic dermatitis M06.8

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

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

Rare. So far, only individual cases have been published in the literature. The disease occurs in less than 5% of patients with rheumatoid arthritis (Zhu M et al. 2024).

The average age of manifestation is 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, accentuated, rather sharply defined, 0.5-1.5 cm large, reddish-purple, also hemorrhagic plaques and papules of moderately coarse consistency. Vesicles, pustules or ulcers are rarer. Subepithelial blistering occurs less frequently. A transition to neutrophilic vasculitis(leukocytoclastic vasculitis) has been described (Zhu M et al. 2024)

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

1. Ackerman AB (1978) Histologic diagnosis of inflammatory skin disease: a method by pattern analysis. Lea & Feiber, Philadelphia, pp. 449-45
2. Below J et al (2015) Rheumatoid neutrophilic dermatitis. Dermatology 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
4. Gay-Crosier F et al. (2000) Rheumatoid neutrophilic dermatitis/sweet's syndrome in a patient with seronegative rheumatoid arthritis. Dermatology 201: 185-187
5. Lazarov A et al. (2002) Rheumatoid neutrophilic dermatitis: an initial dermatological manifestation of seronegative rheumatoid arthritis. J Eur Acad Dermatol Venereol 16: 74-76
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
7. Nakamura T et al. (2011) Cutaneous nodules in patients with rheumatoid arthritis: a case report and review of literatures. Clin
   Rheumatol 30:719-722
8. Yamamoto T et al. (2003) Rheumatoid neutrophilic dermatitis, rheumatoid papules, and rheumatoid nodules in a patient with seronegative rheumatoid arthritis. J Am Acad Dermatol 48: 634-635
9. Youn CS et al. (2000) Colchicine treatment in a patient with neutrophilic dermatosis associated with rheumatoid arthritis. J Dermatol 27: 782-787
10. Yoshida Y et al (2003) Rheumatoid neutrophilic dermatitis. J Dermatol 30: 255-256

11. Zhu M et al. (2024) A case of rheumatoid neutrophilic dermatitis progressed to rheumatoid vasculitis. J Dtsch Dermatol Ges 22:1430-1431.

12. Żuk G et al. (2019) Rheumatoid neutrophilic dermatitis. Reumatologia 57:350-353.