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Rheumatoid Neutrophilic Dermatitis/Sweet’s Syndrome in a Patient with Seronegative Rheumatoid Arthritis

2000; Karger Publishers; Volume: 201; Issue: 2 Linguagem: Inglês

10.1159/000018452

1421-9832

Fabienne Gay-Crosier, J.-M. Dayer, Pierre Chavaz, Carl J. Hauser,

Autoimmune Bullous Skin Diseases

A 56-year-old female with polyarthralgia, symmetric polyarthritis of the IPP, MCP, wrists, MTP, ankles and the left knee for 5 years presented with skin lesions. She received NSAID, methotrexate and hydroxychloroquine for 1 year and subsequently NSAID alone. She never received gold salts. One year ago, she developed erythematous skin lesions with histologic features of spongiotic dermatitis which were treated with intramuscular and topical corticosteroids. She reported fever 2 days previously and occasional diarrhea for 20 years.At presentation, she had isolated erythematous papules on the extensor aspects of the extremities, up to 3 cm in diameter with a smooth surface and some with pustules on the top (fig. 1). She had active arthritis of the left wrist and swelling of the right forearm. May-Grünwald-Giemsa and gram stains of smears from pustules showed neutrophils without bacteria, filaments or spores. Bacterial and fungal cultures of pustular content remained sterile. A skin biopsy showed a dense neutrophilic infiltrate with edema in the superficial dermis, an intraepidermal abcess of neutrophils reaching the stratum corneum, nonspongiotic adjacent epidermis and a mixed neutrophilic mononuclear infiltrate in the deep dermis (fig. 2). There were no signs of vasculitis. The ESR was 28, CRP 65 mg/l, Hb 12.6 g/dl, the white blood cell count 5.0 ×109/l. Routine blood chemistry, urinalysis, complement, ANA, latex, ANCA, serology for HIV, HBV, HCV were normal or negative, except for IgG at 5.38 g/l (N: >6.7 g/l). No erosions were detected on the hand radiographs; a chest X-ray was normal. Colonoscopy with biopsy was normal. The patient developed hemolysis when treated with dapsone at 100 mg/day, despite normal G6PDH values. The skin lesions responded to topical corticosteroids. The mild decrease in the serum IgG level was persistent but not associated with symptoms of immunodeficiency.Clinical presentation and patient history were suggestive of rheumatoid neutrophilic dermatitis (RND), the biopsy findings being compatible with this diagnosis. RND is a rare condition in patients with rheumatoid arthritis (RA). All patients with reported serologic tests for RA were positive [1, 2, 3, 4, 5, 6, 7, 8, 9]. Similarly, articular involvement when described was severe and disabling. Our patient had radiologically nonerosive arthritis and a repeated negative latex test, but her condition fulfilled the American Rheumatism Association's criteria for RA [10]. Neither the histological nor the clinical presentation looked like subcorneal pustular dermatosis of Sneddon and Wilkinson, also reported in association with RA [11]. Other neutrophilic dermatoses associated with RA include pyoderma gangrenosum, Sweet's syndrome [12, 13, 14]and erythema elevatum et diutinum [15, 16, 17]. It is controversial whether RND is different from Sweet's syndrome and even from erythema elevatum et diutinum, as they all have many features in common. We have no explanation for the transient and slight decrease in serum IgG level in our patient, but we cannot formally exclude a relation to the patient's RA or neutrophilic dermatosis. Several forms of arthritis, including RA and Still's disease, have repeatedly been described to be associated with hypogammaglobulinemia. In these cases, the deficiency was always severe and symptomatic, unlike in our patient who did not have a history or other clinical manifestations of clinically relevant hypogammaglobulinemia. We found only 2 case reports of neutrophilic dermatosis (pyoderma gangrenosum) associated with hypogammaglobulinemia; one in a patient with thymoma and the other in a patient with cutaneous T cell lymphoma [18, 19].In conclusion, negative serology for RA may not preclude from RA-associated neutrophilic dermatosis.