Sindrom Hipersensitivitas Dapson pada Pasien Morbus Hansen Multi- Basiler: Laporan Kasus

Abstract

Abstract: Dapsone hypersensitivity syndrome (DHS) is a rare case. Classic triad consists of fever, exfoliative dermatitis, and internal organs involvement. Its pathogenesis is still unknown, thought to be caused by hydroxylamine, a toxic compound which plays a role in the incidence of haemolytic anemia. The diagnosis is made when the hypersensitivity reaction occurs in 2-8 weeks during dapsone therapy with at least two of these symptoms are present: fever, skin eruption, lymphadenopathy, and liver function disorder. Treatment of the DHS involves prompt discontinuation of dapsone. Corticosteroids have been proved helpful with doses of up to 1 g/day of methylprednisolone for 3 days and tapering off for 4-6 weeks. We reported a case of a 33-year-old female diagnosed as multibacillary leprosy and received multibacillary multi-drug therapy (MDT-MB) for the second month. Six weeks after starting the therapy, fever and exfoliating skin on almost the entire body appeared. On clinical examination, multiple erythematous to hyperpigmented macules were found with crusting and rough scales. There were also anemic conjunctiva, subicteric sclera, and hepatomegaly. Laboratory tests revealed anemia, lymphocytosis, and increased liver enzymes. Based on these clinical findings and investigations, diagnosis of DHS was established. After discontinuation of dapsone and oral administration of 32 mg/day of methylprednisolone which was reduced by 8 mg every 2 weeks, clinical and laboratory finding showed improvement. The clinical manifestations of DHS can resemble other drug allergy, however, DHS has fatal consequence if it is not handled properly.

Keywords: dapsone hipersensitivity syndrome, multibacillary leprosy, corticosteroids

 

Abstrak: Sindrom hipersensitivitas dapson merupakan kasus yang jarang terjadi. Trias klasik yaitu demam, dermatitis eksfoliatifa, dan keterlibatan organ internal. Patogenesis tidak diketahui, diduga disebabkan oleh hidroksilamin, senyawa toksik yang berperan dalam kejadian anemia hemolitik. Diagnosis dibuat ketika reaksi hipersensitivitas terjadi dalam 2-8 minggu selama terapi dapson dengan setidaknya timbul dua dari gejala berikut: demam, erupsi kulit, limfadenopati, dan gangguan fungsi hati. Pengobatan sindrom hipersensitivitas dapson ialah menghentikan segera obat dapson dan pemberian kortikosteroid yaitu metilprednisolon dengan dosis hingga 1 g/hari selama 3 hari dan penurunan dosis selama 4-6 minggu. Kami melaporkan kasus seorang perempuan berusia 33 tahun didiagnosis dengan morbus hansen multibasiler dan menerima terapi multi drug therapy-multibasiler (MDT-MB) untuk bulan kedua. Enam minggu setelah memulai terapi, muncul demam dan kulit mengelupas di hampir seluruh tubuh. Pada pemeriksaan fisik, ditemukan beberapa makula eritematosa hingga hiperpigmentasi dengan skuama kasar dan krusta pada kulitnya. konjungtiva anemis, sklera subikterik, dan hepatomegali. Pada uji laboratorium didapatkan anemia, limfositosis, dan peningkatan enzim hati. Berdasarkan temuan klinis dan pemeriksaan penunjang, diagnosis sindrom hipersensitivitas dapson ditegakkan. Setelah penghentian dapson dan pemberian metilprednisolon oral 32 mg/hari yang diturunkan 8 mg setiap 2 minggu, temuan klinis dan laboratorium menunjukkan perbaikan. Manifestasi klinisnya dapat menyerupai alergi obat lain dan memiliki konsekuensi fatal jika tidak ditangani dengan benar.

Kata kunci: sindrom hipersensitivitas dapson, morbus hansen multibasiler, kortikosteroid