Sweet syndrome (SS) merupakan dermatosis netrofilik yang bersifat akut dan dapat rekuren. Gambaran klinis SS dapat menyerupai keganasan pada kulit, misalnya cutaneous T cell lymphoma (CTCL) atau leukemia kutis. Pada 50% kasus SS terdapat penyakit yang mendasarinya, infeksi, keganasan, atau autoimun. Dilaporkan satu kasus SS pada laki-laki berusia 46 tahun. Kelainan kulit timbul sejak 4 bulan sebelumnya, berupa plak keunguan pada wajah, dada, dan punggung serta nodus pada kedua lengan dan tungkai yang terasa nyeri. Keluhan disertai malaise, penurunan berat badan drastis, dan demam hilang timbul. Limfadenopati generalisata didapatkan pada pemeriksaan fisis, sehingga pada awalnya diduga proses keganasan. Pemeriksaan histopatologis dan imunohistokimia tidak menunjukkan keganasan pada kulit, melainkan gambaran dermatosis netrofilik. Pemeriksaan antinuclear antibody, SS-A, dan dsDNS positif, serta didapatkan proteinuria, netrofilia, anemia, dan trombositopenia, mendukung diagnosis lupus eritematosus sistemik (LES). Diagnosis SS ditegakkan berdasarkan dua kriteria mayor, yaitu lesi kulit khas dan gambaran histopatologis sesuai SS, serta tiga kriteria minor, yaitu netrofilia, terdapat penyakit yang mendasari, dan memberi respons baik dengan terapi kortikosteroid sistemik. Lupus eritematosus sistemik pada kasus ini ditegakkan berdasarkan empat kriteria American College Rheumatology yang positif, yaitu artralgia, kelainan darah, proteinuria, serta ANA, SS-A, dan dsDNS positif. Setiap kasus SS memerlukan berbagai pemeriksaan penunjang untuk mengetahui penyakit yang mendasarinya, yaitu keganasan, autoimun, atau infeksi. (MDVI 2011; 38/s: 49s - 54s)
Kata kunci : Sweet syndrome, keganasan, autoimun |
Sweet syndrome (SS) is an acute neutrophillic dermatoses which can be reccurent. Clinical appearances of SS can resemble some cutaneous malignancy, such as cutaneous T cell lymphoma (CTCL) or leukemia cutis. About 50% SS has underlying diseases, which can be either infection, malignancy, or autoimmunity. A case of SS in a-46-year-old man is reported. Skin lesions occured since 4 months before admission, consisting of painful violaceous plaque on the face, neck, back, and chest, and nodus on both extremities. The patient also complained of malaise, dramatical weight loss, and fever. Physical examination showed generalized lymphadenopathy. These findings supported suspicion of malignancy. There were also anemia, neutrophilia, thrombocytopenia, and proteinuria. Histopatological and immunohistochemical examinations revealed no cutaneous malignancy, but a neutrophillic dermatosis. Positive antinuclear antibody, SS-A, and dsDNS, supported the diagnosis of systemic lupus erythematosus (SLE). Diagnosis of SS in this case was based on two mayor criteria, typical lesions and appropriate histopathologic appearances, and three minor criteria, such as neutrophillia, positive underlying disease, and a good response to systemic corticosteroid therapy. Diagnosis of SLE was based on American College Rheumatology (ACR) criteria, such as arthralgia, hematological disorders, proteinuria, and positive ANA, SS-A, and dsDNS. On every SS cases further examination should be performed to find the underlying disease, such as malignancy, autoimmunity, or infection. (MDVI 2011; 38/s: 49s - 54s)
Keyword: Sweet syndrome, malignancy, autoimmunity |