Terapi necrobiotic xanthogranuloma dengan siklofosfamid-metilprednisolon

Bayu Lesmono, Yussy Afriani Dewi, Sinta Sari Ratunanda, Nur Akbar Aroeman

Abstract


Latar belakang: Necrobiotic xanthogranuloma (NXG) merupakan suatu penyakit yang langka, kronis, dan progresif. NXG ditandai dengan lesi kulit ulseratif pada daerah indurasi, dan berwarna kuning, atau berupa nodul yang mengenai sel histiosit non Langerhans. Daerah predileksi tersering ialah pada wajah, orbita, dan ekstremitas. Etiologi belum diketahui, tetapi sering dikaitkan dengan monoclonal gammopathy. Gambaran histopatologi NXG yaitu ditemukan makrofag dan terdapat sel busa pada dermis, jaringan subkutan, nekrobiosis luas, sel datia Touton, dan folikel limfoid. Penderita memiliki lesi yang bersifat asimtomatik, parestesi, rasa terbakar, dan terkadang timbul rasa nyeri. Tata laksana NXG sampai saat ini masih sangat bervariasi. Tujuan: Memaparkan hasil penatalaksanaan dua pasien NXG. Laporan kasus: Dilaporkan pasien pertama, laki-laki 44 tahun dengan lesi pada kedua pipi dan dahi sejak 5 bulan sebelumnya. Terapi diberikan metilprednisolon dengan dosis 0,8 mg/kgBB tappering off selama 1 bulan dengan hasil perbaikan. Pasien kedua, wanita 29 tahun dengan lesi pada kedua pipi dan telinga sejak 5 bulan sebelumnya. Terapi diberikan siklofosfamid 750 mg/m2 per 3 minggu dengan hasil membaik. Metode: Pencarian dilakukan melalui PubMed dan Dermatol. Setelah menyaring judul dan abstrak yang sesuai dengan kriteria inklusi dan eksklusi, didapatkan lima artikel utuh. Kemudian, tiga artikel terpilih untuk ditelaah secara konsensus, meliputi kesahihan (validity), kepentingan (importancy), dan aplikabilitas (applicability) pada pasien necrobiotic xanthogranuloma. Hasil: Telaah artikel-artikel tersebut menunjukkan bahwa semua artikel memiliki karakteristik yang serupa dalam hal kesahihan (validity), hasil atau kesimpulannya. Walau demikian, ada beberapa kekuatan dan kelemahan pada masing-masing artikel. Kesimpulan: Penatalaksanaan NXG masih diperlukan penelitian lebih lanjut dengan jumlah sampel yang banyak untuk mengetahui keefektifitasan penatalaksanaan NXG.

Kata kunci: Siklofosfamid, metilprednisolon, necrobiotic xanthogranuloma

ABSTRACT

Background: Necrobiotic Xanthogranuloma (NXG) is a rare, chronic, and progressive disease that provokes skin lesions, such as damage of the histiocytes of non-Langerhans cell, skin lesions (yellowish or noduled ulcerative lesions) in the induration skin. The most common predilection areas of this are on the face, orbital, and extremities. The etiology is still unknown, but sometimes associates with monoclonal gammopathy. NXG histopathologic contained macrophages and foam cells in the dermis, subcutaneous tissue, extensive necrobiosis, touton giant cells, and lymphoid follicles. Some patients who had lesions are asymptomatic, sometimes they will feel paresthesias, burning pain. Nowadays, this management is still vary widely. Objective: Sets forth the results of two patients NXG. Case: There were two patients. Patient number one, a 44-years-old man, with some lesions on both cheeks and forehead since 5 months ago. He was treated with methylprednisolon 0.8 mg/kg body weight, and tappered off for a month with improved results. Patient number two, a 29-years-old woman, with some lesions on both cheeks and ears since 5 months ago. She was treated with Cyclosphosphamide 750 mg/m2 with improved results within three weeks. Method: Searching for literature was conducted in PubMed and Dermatol. After screening titles and abstracts with inclusion and exclusion criteria, five articles were available as full texts. Three articles were selected to be reviewed for their validity, importancy and applicability in patient with necrobiotic xanthogranuloma. Results: The review showed that all those articles had a similar characteristic in validity, result, and conclusion. Nevertheless, each articles had its own strong points and weaknesses. Conclusion: NXG treatment still required further research by the number of samples that much to find out the efficiency management NXG.

Keywords: Cyclophosphamide, methylprednisolon, necrobiotic xanthogranuloma


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DOI: https://doi.org/10.32637/orli.v46i2.168

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