Acta Dermato-Venereologica 98-4CompleteContent | Page 17

452 SHORT COMMUNICATION Generalized Purpura as an Atypical Skin Manifestation of Adult-onset Still’s Disease in a Patient with Behçet’s Disease Chika OMIGAWA, Takashi HASHIMOTO*, Takaaki HANAFUSA, Takeshi NAMIKI, Ken IGAWA and Hiroo YOKOZEKI Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45, Yushima, Bunkyo-ku, 113-8519, Tokyo, Japan. *E-mail: [email protected] Accepted Jan 8, 2018; Epub ahead of print Jan 9, 2018 Adult-onset Still’s disease (AOSD) is a systemic inflam- matory condition featuring a high spiking fever, arthral- gia, leukocytosis with neutrophilia, and skin rash. The typical skin rash in AOSD is an evanescent, salmon-pink erythema that mainly involves the extremities (1). While various skin symptoms are reported as atypical skin rashes in AOSD (1–3), few reports mention generalized purpura (1, 4). CASE REPORT A 65-year-old Japanese woman presented with a 2-week history of pyrexia, arthralgia, sore throat and skin rash. Ten years pre- viously she had been diagnosed with Behçet’s disease (BD) due to the presence of recurrent oral aphthous ulcers and erythema nodosum-like skin lesions. She had been treated with colchicine (1.5 mg/day) and tacrolimus (1 mg/day) and had been in remission. Physical examination revealed multiple, non-palpable, erythema- tous macules (5–10 mm in diameter) as linear stripes on her trunk and limbs (Fig. 1a). These macules were obvious when she had a fever, but disappeared in the absence of fever. Bilateral swollen tonsils were noted. Laboratory data revealed a high white blood cell count (16,100/µl with 81% neutrophils), elevated C-reactive protein levels (CRP 17.09 mg/dl; reference < 0.3 mg/dl) and serum hepatic/biliary enzyme levels (aspartate transaminase, 84 IU/l; alanine transaminase 110 IU/l; γ-glutamyltransferase, 105 IU/l (reference ≤  35 IU/l); lactate dehydrogenase 615 U/l (reference 124–222 U/l)) and normal bilirubin levels (1.0 mg/dl). Serum ferritin levels were elevated (3,380 ng/ml; reference 3–120 ng/ ml). Serum immunoglobulin, IgD, IgG, IgA, and IgM, levels were normal. Negative results were obtained for the presence of antinu- clear antibodies and rheumatoid factor. Serum interleukin (IL)-1β (2.87 pg/ml; reference < 0.928 pg/ml), IL-6 (121 pg/ml; reference < 2.41 pg/ml), and IL-18 (1,250,000 pg/ml; reference < 211 pg/ml) levels were elevated. The patient expressed both human leukocyte antigen (HLA)-B51 and HLA-B54. Urinalysis was normal. Blood bacterial cultures yielded negative results. Whole-body computed tomography revealed splenomegaly, but no lymphadenopathies, infection foci, or malignant tumours. Ophthalmological examina- tion and echocardiogram were both normal. A skin biopsy obtai- ned from a macule showed dyskeratotic cells in all levels of the epidermis, vacuolar degeneration in the epidermis (Fig. 1b), and slight perivascular and interstitial lymphoneutrophilic infiltrates in the upper dermis (Fig. 1c). Neither vasculitis nor periadnexal infiltrate was found. Therefore, a diagnosis of AOSD was made. Intravenous pulse methylprednisolone therapy (1 g/day for 3 days) and oral corticosteroids (40 mg/day) were administered; a switch from tacrolimus to azathioprine (50 mg/day) was also made. Her symptoms partially improved. However, 1 day after the completion of methylprednisolone therapy, when the peripheral platelet count was normal, multiple, slightly-palpable purpura (approximately 5 mm in diameter) appeared on the trunk (Fig. 2a, b) and disappeared spontaneously within 3 days. When serum ferritin and CRP levels increased again 2 weeks later, generalized purpura recurred on the trunk and proximal limbs. Negative results were obtained for the presence of anti-neutrophil cytoplasmic antibodies. A bone marrow biopsy was negative for haemophagocytic syndrome. A second skin biopsy of the purpura revealed scattered dyskeratotic cells and vacuolar degeneration in the epidermis with erythrodiapedes and perivascular lymphocytic infiltrates in the upper dermis (Fig. 2c). Neither vasculitis nor periadnexal infiltrate was detected. AOSD relapse was considered, and steroid therapy was restarted, with subsequent improvement in symptoms. DISCUSSION The patient’s initial skin eruptions featured a linear con- figuration suggestive for Koebner phenomenon. Histo- pathological examination showed scattered dyskeratotic cells in the epidermis. These clinical and pathological findings are characteristic of atypical skin manifesta- tions in AOSD, termed “persistent pruritic papules and Fig. 1. Clinical and histopathological features at initial presentation. (a) Non-palpable, erythematous papules and macules (5–10 mm in diameter) were obvious as linear stripes on the proximal limbs, suggestive of Koebner phenomenon. (b and c) Histopathological findings of erythematous macules. (b) Dyskeratotic cells (yellow arrowheads) in all levels of the epidermis and (c) vacuolar degeneration in the epidermis were accompanied by perivascular and interstitial lymphoneutrophilic infiltrates in the upper dermis. No vasculitis was seen (haematoxylin and eosin staining; original magnification ×200). doi: 10.2340/00015555-2880 Acta Derm Venereol 2018; 98: 452–453 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.