Rheumatoid neutrophilic dermatitis

ARTICLE

Rheumatoid arthritis (RA) is often accompanied by various cutaneous disorders. Rheumatoid neutrophilic dermatitis (RND) is a rare condition, first described by Ackerman in his textbook in 1978 [1], which occurs in patients with severe, seropositive RA. Only fourteen cases have been reported in the English and Japanese literature. We present a case of RND which occurred in a male patient who had RA and rheumatoid lung.

Case report

A 63-year-old Japanese man had suffered from RA for seven years and had been treated with a nonsteroidal anti-inflammatory agent (acemetacin) and an antirheumatic drug (bucillamine). The patient had suffered from interstitial pneumonia related to the RA for one year. We saw him at our clinic because for the previous eight months, erythematous plaques had appeared on his extremities and had occasionally ulcerated. On examination, asymptomatic symmetric erythematous plaques, accompanied by pustules or small ulcers, were seen on his buttocks and extremities (Fig. 1). Slightly raised, indurated erythema was seen on his left palm. Painful, violescent erythema was seen on his soles. Subcutaneous nodules accompanied by erythema and pustules were evident on both elbows (Fig. 2). He had pain and swelling in the finger joints bilaterally. Laboratory examinations revealed a positive rheumatoid factor at a titer of 1:1,280 and a positive antinuclear antibody at 1:20. The white blood cell count was 8,000/mm³ with 70% neutrophils. The erythrocyte sedimentation rate was 79 mm/h, and the C-reactive protein was 7.2 mg/dl. The other blood chemistry findings were within normal limits. An X-ray of the finger joints showed no obvious changes related to RA. Chest X-ray examination revealed interstitial pneumonia on the bilateral lower lobes.

A skin biopsy from an erythematous lesion on his left knee showed a dense neutrophilic infiltration with leukocytoclasis throughout the dermis with the formation of microabscesses in the dermal papillae (Fig. 3). Some collagen fibers showed basophilic degeneration. There was no evidence of vasculitis. Direct immunofluorescence study revealed no deposits of immunoglobulin or complement. A subcutaneous nodule on his right elbow was revealed to be a palisading granuloma which consisted of a rheumatoid nodule with patchy neutrophilic infiltration. Salazosulfapyridine was administered. During the follow-up period of ten months, his arthritis was controlled well, while small areas of erythema continued to appear.

Discussion

Clinically, RND exhibits red-purple plaques and papules, often associated with vesicles or pustules, which are symmetrically distributed on the exterior surfaces of the extremities. Histologically, RND consists of a dense and diffuse dermal infiltration of neutrophils with nuclear dust. Sometimes neutrophils in the dermal papillae form microabscesses. Our patient had seropositive RA and interstitial pneumonia, which we diagnosed as rheumatoid lung because there was no other apparent cause of the pneumonia, and showed clinically and histologically typical RND. To date, only fourteen cases of RND have been reported in the English and Japanese literature [2-10] (Table). The patients were described as having seropositive, severe, disabling, and progressive RA. In addition, one patient was reported to have rheumatoid lung [5], and one with rheumatoid nodules [6], but no other skin manifestations or visceral involvement related to RA were mentioned. There was no obvious therapy for RA that had any effect on RND. However some medications, colchicine [5, 10], topical steroids [6], dapsone [7, 8], and etretinate [9] were reported to be effective for RND.

The pathogenesis of RND remains unknown. Jorrizo et al. [11] proposed the concept of "neutrophilic vascular reaction" characterized by neutrophilic infiltration and dermal vessel changes. Leukocytoclastic vasculitis, Sweet's syndrome, Behcet's disease, and pyoderma gangrenosum are included in this concept. Neutrophilic vessel-based dermal inflammation is a feature of these diseases. Circulating immune complexes, immunoreactants in the dermal vessels, and conspicuous neutrophil migration participate to varying degrees in the pathogenesis of these cutaneous lesions [4, 11]. RND occurs in patients with RA, which is an immune complex-mediated inflammatory disorder. It is quite possible that circulating immune complexes involving the rheumatoid factor play a role in the development of RND. We therefore believe that RND could also be included within the concept of neutrophilic vascular reaction as mentioned by Lowe et al. [4].

REFERENCES

1. Ackerman AB. Histologic diagnosis of inflammatory skin disease: a method by pattern analysis. Philadelphia: Lea & Feiber, 1978: 449-51.

2. Scherbenske JM, Benson PM, Lupton GP, et al. Rheumatoid neutrophilic dermatitis. Arch Dermatol 1989; 125: 1105-8.

3. Sanchez JL, Cruz A. Rheumatoid neutrophilic dermatitis. J Am Acad Dermatol 1990; 22: 922-5.

4. Lowe L, Kornfeld B, Clayman J, et al. Rheumatoid neutrophilic dermatitis. J Cutan Pathol 1992; 19: 48-53.

5. Kobayashi H, Ogawa N, Nakauchi Y. A case of rheumatoid neutrophilic dermatitis. Jpn J Dermatol (Japanese) 1994; 104: 893-7.

6. Yamamoto T, Ohkubo H, Nishioka K. Rheumatoid neutrophilic dermatitis. Int J Dermatol 1994; 33: 798-800.

7. Harkaway K, Elenitas R, Margolis DJ. Erythematous papules in a patient with rheumatoid arthritis. Arch Dermatol 1995; 131: 1195-200.

8. Chin K, Asai T, Katsuoka K. Rheumatoid neutrophilic dermatitis. Hifubyo Shinryo (Japanese) 1996; 18: 1097-100.

9. Yamada T, Itou Y. A case of rheumatoid neutrophilic dermatitis. Skin Research (Japanese) 1996; 38: 55-9.

10. Kurose K, Mori O, Hashimoto T, et al. A case of rheumatoid neutrophilic dermatitis. Hifuka-no-Rinsho (Japanese) 1997; 51: 707-9.

11. Jorrizo JL, Solomon AR, Zanolli MD, et al. Neutrophilic vascular reactions. J Am Acad Dermatol 1988; 19: 983-1005.