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Case Report We describe a 75-year-old man with a 10-year history of rheumatoid arthritis (RA) who presented to our clinic with recurrent outbreaks of edematous, infiltrated, annular, erythematous plaques with a violaceous center. The lesions first appeared ∼3 years before admission, were located on the patient’s trunk, arms, legs, neck, and scalp (Figures 1A–1C), and lasted for 3 weeks. The family history was negative for similar conditions. Upon admission, the patient was afebrile, and did not have malaise, myalgia, or lymphadenopathy. He had been taking nonsteroidal anti-inflammatory drugs for RA for 10 years. A complete blood count revealed anemia of chronic disease, and the results of routine biochemical testing were normal. His C-reactive protein level was 81 mg/L (reference range, 0–5 mg/L) and erythrocyte sedimentation rate was 71 mm/h (reference range, <20 mm/h). Chest radiography revealed sequelae of tuberculosis in both pulmonary apices. Abdominal ultrasound showed a solid intravesical LY335979 mass. The histopathological findings of a biopsy of the mass were consistent with papillomatous hyperplasia. Serological testing for evidence of infection with hepatotropic viruses, human immunodeficiency virus, and syphilis was negative. No other serological testing was performed. The patient was negative for the tumor markers prostate-specific antigen, carcinoembryonic antigen, and cancer antigen-19.9, and his stool was negative for occult blood. Serum immunoelectropheresis findings were also normal. Together with the patient’s physical examination and history, the clinical assessment was negative for an underlying malignancy, and no additional investigations for the presence of an underlying malignant tumor were performed. A skin biopsy specimen showed neutrophilic infiltrate in the upper and mid-dermis, as well as leukocytoclasis. No vasculitis was observed (Figures 2A and 2B). Colchicine and dapsone, which inhibit neutrophil chemotaxis, were used to treat the infiltrate. However, because of the patient’s low hemoglobin level and the occurrence of diarrhea, they were discontinued. The lesions were attributed to poorly controlled RA, and the patient was referred to a rheumatologist, who prescribed methylprednisolone (10 mg/d) and methotrexate (10 mg/wk) for 2 months. The lesions gradually disappeared. However, the patient stopped taking the methylprednisolone and methotrexate because he was worried about the side effects. Two weeks after the treatment was stopped, the lesions reappeared. At a 6-month follow-up visit, the patient complained of severe arthralgia and widespread lesions involving his entire body. He again refused systemic treatment with methylprednisolone and methotrexate.
Discussion In 1989, Christensen et al reported two patients with chronically recurring outbreaks of generalized annular erythematous, edematous plaques. The histopathological findings were consistent with Sweet syndrome; however, the patients presented without fever or other findings characteristic of Sweet syndrome. Christensen et al regarded these two cases as a unique entity because of the annular lesions and the absence of systemic manifestations, and used the descriptive term “chronic recurrent annular neutrophilic dermatosis” for the condition, instead of considering it to be a variant of Sweet syndrome. Romero Aguilera et al reported four cases that manifested as afebrile recurrent outbreaks occurring over a course of several years. The clinical appearance and histopathological features of the skin lesions of all four patients were suggestive of Sweet syndrome, but none of the patients at any time developed leukocytosis or neutrophilia. However, these authors considered this presentation to be a variant of Sweet syndrome and not a separate entity. Cabanillas et al reported a patient with a 6-month history of generalized painful annular erythematous, edematous plaques. The patient did not present with fever or leukocytosis, but did have neutrophilia and an elevated erythrocyte sedimentation rate. The lesions improved after a course of oral prednisone, and the patient only had occasional mild recurrences. The authors considered their case to resemble more closely Christensen et al’s CRAND instead of being a variant of Sweet syndrome.