Document Detail

Rheumatoid Neutrophilic Dermatitis: A Male Chinese Case with Multiple Lesions.
MedLine Citation:
PMID:  23069309     Owner:  NLM     Status:  Publisher    
Rheumatoid neutrophilic dermatitis (RND), first described by Ackerman in 1978, is a rare condition that occurs often in patients with severe rheumatoid arthritis (RA) suffering from the disease for at least five years. 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. Histologic manifestation consists of a dense and diffuse dermal infiltration of neutrophils with nuclear dust, with absence of vasculitis and fibrin deposits. The characteristic laboratory finding is a high-titer of rheumatoid factor (RF) (1-3). To date, only about thirty cases of RND have been reported. Here, we report on a case with several unusual characteristics. A 59-year-old Chinese man with seropositive RA presented with painful erythematous papules and pustules on his trunk, ears, scalp, and blisters on his palms and soles, that had first appeared two months before. The patient had suffered from RA for three years and had been treated with Tripterygium wilfordii off and on. A burning pain over the affected areas preceded the appearance of the lesions. On dermatological examination, the papules and pustules, or small ulcers, were apparent on his trunk and blisters were seen on his palms and soles, some of which were hemorrhagic and up to 1 cm in diameter (Fig. 1). The patient's nails and mouth were clear of lesions and no similar lesions were noted elsewhere; however, the patient did have pain and swelling in his bilateral finger joints. On laboratory examination, his RF was 687 kU/L (reference range: 0-15), and RF-IgG, IgA, and IgM were all higher than 300 μ/mL (reference range: 0-15); other autoantibodies were all negative, including ANA, ANCA, anti-ENA antibodies, etc. The erythrocyte sedimentation rate was 89 mm/h and C-reactive protein was 77.1 mg/L (reference range: 0-5). The patient refused x-ray examination due to financial reasons. Two skin biopsies were taken from an erythematous lesion on the patient's trunk and from a blister on his palm. Both showed diffuse neutrophilic infiltration throughout the dermis, primarily around vesicles. Scattered histiocytes and lymphocytes were apparent and there was no bullae formation. The dermal blood vessels appeared unaffected, and there was no evidence of vasculitis or vasculopathy (Fig. 2). A direct immunofluorescence study revealed no deposits of immunoglobulin or complement. Methylprednisone (50 mg/d) was administered along with azithromycin (500 mg/d for 1 week), Tripterygium wilfordii (60 mg/d), and colchicine (1 mg/d). One week after treatment, the blisters began to dry and crust, the erythematous papules turned darker, the pustules disappeared, and the ulcers became smaller (Fig. 3). During the ten-month follow-up period, the patient's arthritis was well controlled. The papules and blisters reappeared when the dose of prednisone was decreased to 30 mg/d; however, they healed soon. In conclusion, the patient was a middle-aged male, with a three-year history of RA, and had a high-titer of RF. He had multiple lesions, which included blisters as well as erythematous papules and pustules. The histological manifestation showed a dense neutrophilic infiltration throughout the dermis. The diagnosis of RND was verified. In addition, our patient had several unusual characteristics: 1) although RND occurs more often in females, our patient was a middle-aged male; 2) RND patients usually have RA for more than five years, while our patient's case history was of only three years, and the appearance of his lesions may have been associated with high RF titer; and 3) our patient had blisters clinically, but no bullae formation histologically, which is most interesting. We think his blisters may be pseudo blisters, which are similar to the lesions seen in Sweet's syndrome. In fact, not only did the skin lesions in our case closely resemble those in Sweet's syndrome histologically, but they could also be confused with Sweet's syndrome clinically. However, Sweet's syndrome lesions most commonly occur on the face and extremities. In addition, fever, malaise and conjunctivitis often accompany the onset of Sweet's syndrome, but these symptoms are not seen in patients with RND. Other neutrophilic dermatoses must be included in the differential diagnosis, including Behcet syndrome, pyoderma gangrenosum, and erythema elevatum diutinum, which invariably show vasculitis on pathology and usually can be ruled out by clinical appearance or other findings (2,4). To date, blister formation in RND has only appeared 3 times. In those cases, two patients resembled dermatitis herpetiformis and one case mimicked bullous pemphigoid (5). Gay-Crosier et al. report on a patient with seronegative RA who had erythematous papules that resembled Sweet's syndrome. In this case, clinical presentation and patient history were suggestive of RND, and biopsy findings were compatible with this diagnosis (6). Our patient is the second patient with RND that resembled Sweet's syndrome. Based on the pathogenesis of RND and medical documentation, we decided to administer azithromycin (7), Tripterygium wilfordii and colchicine in addition to methylprednisone, which have nonspecific anti-inflammatory effects to inhibit the migration of neutrophilic cells and the release of cytokines. These medications proved effective in our patient; however, experience in more patients is needed to make any firm conclusions.
Song Yali; Jin Dehui; Zhou Shuhua
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Publication Detail:
Journal Detail:
Title:  Acta dermatovenerologica Croatica : ADC     Volume:  20     ISSN:  1847-6538     ISO Abbreviation:  Acta Dermatovenerol Croat     Publication Date:    2012 OKTOBER
Date Detail:
Created Date:  2012-10-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9433781     Medline TA:  Acta Dermatovenerol Croat     Country:  -    
Other Details:
Languages:  ENG     Pagination:  204-206     Citation Subset:  -    
Zhou Shuhua, MD, Provincial Hospital affiliated to Shandong University, Jinan 250021, China;
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