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Journal : JURNAL BIOMEDIK

SINDROM HIPERSENSITIVITAS DAPSON PADA PASIEN MORBUS HANSEN MULTI- BASILER: LAPORAN KASUS Palimbong, Florencia; Mellyanawati, .; Kandou, Renate T.
JURNAL BIOMEDIK : JBM Vol 11, No 3 (2019): JURNAL BIOMEDIK : JBM
Publisher : UNIVERSITAS SAM RATULANGI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.35790/jbm.11.3.2019.26331

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
TRACHYONYCHIA ASSOCIATED WITH ALOPECIA AREATA AND SECONDARY ONYCHOMYCOSIS Anggowarsito, Jose L.; Kandou, Renate T.
Jurnal Biomedik : JBM Vol 6, No 1 (2014): JURNAL BIOMEDIK : JBM Maret 2014
Publisher : UNIVERSITAS SAM RATULANGI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.35790/jbm.6.1.2014.4164

Abstract

Abstract: Trachyonychia is an idiopathic nail inflammatory disorder that causes nail matrix keratinization abnormality, often found in children, and associated with alopecia areata, psoriasis, atopic dermatitis, or nail lichen planus. Trachyonychia could be a manifestation of associated pleomorphic or idiopathic disorders; therefore, it may occur without skin or other systemic disorders. There is no specific diagnostic criteria for tracyonychia. A biopsy is needed to determine the definite pathologic diagnosis for nail matrix disorder; albeit, in a trachyonychia case it is not entirely necessary. Trachyonychia assessment is often unsatisfactory and its management is focused primarily on the underlying disease. We reported an 8-year-old girl with twenty dystrophic nails associated with alopecia areata. Cultures of nail base scrapings were performed two times and the final impression was trichophyton rubrum. Conclusion: Based on the clinical examination and all the tests performed the diagnosis of this case was trachyonychia with twenty dystrophic nails associated with alopecia areata and secondary onychomycosis.The majority of trachyonychia cases undergo spontaneous improvement; therefore, a specific therapy seems unnecessary. Onychomycosis is often difficult to be treated. Eradication of the fungi is not always followed by nail restructure, especially if there has been dystrophy before the infection.Keywords: trachyonychia, alopecia areata, onychomycosis.  Abstrak: Trakionikia adalah inflamasi kuku idiopatik yang menyebabkan gangguan keratinisasi matriks kuku, sering terjadi pada anak, dan terkait dengan alopesia areata, psoriasis, dermatitis atopik atau lichen planus kuku. Trakionikia bisa merupakan manifestasi dan asosiasi dari gangguan pleomorfik atau idiopatik, sehingga dapat terjadi tanpa kelainan kulit dan gangguan sistemik lainnya. Tidak terdapat kriteria diagnosis khusus untuk trakionikia. Diagnosis patologik definitif untuk kelainan matriks kuku ialah melalui biopsi, namun hal ini tidak disarankan. Penanganan trakionikia sering tidak memuaskan dan fokus manajemen terutama ditujukan pada penyakit yang mendasarinya. Kami melaporkan seorang anak perempuan berusia 8 tahun dengan dua puluh kuku distrofik disertai alopesia areata. Kultur dari kerokan dasar kuku dilakukan dua kali dengan hasil trichophyton rubrum. Simpulan: Berdasarkan pemeriksaan klinis dan pemeriksaan penunjang, diagnosis kasus ini ialah trakionikia dengan 20 kuku distrofik disertai alopesia areata dan onikomikosis sekunder. Mayoritas kasus trakionikia dilaporkan mengalami perbaikan spontan sehingga terapi khusus untuk trakionikia sering tidak diperlukan. Onikomikosis sering sulit diobati. Eradikasi jamur tidak selalu disertai perbaikan struktur kuku, terutama bila telah terjadi distrofi kuku sebelum infeksi.Kata kunci: trakionikia, alopesia areata, onikomikosis.
MORBUS HANSEN MULTIBASILER RELAPS DENGAN REAKSI ERITEMA NODOSUM LEPROSUM BULOSA PADA SEORANG ANAKMORBUS HANSEN MULTIBASILER RELAPS DENGAN REAKSI ERITEMA NODOSUM LEPROSUM BULOSA PADA SEORANG ANAK Tangkidi, Damayanti; Sondakh, Oktavia R. L.; Kandou, Renate T.
JURNAL BIOMEDIK : JBM Vol 7, No 3 (2015): JURNAL BIOMEDIK : JBM
Publisher : UNIVERSITAS SAM RATULANGI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.35790/jbm.7.3.2015.9491

Abstract

Abstract: The clinical manifestations of ENL bullous reaction are generally in the form of nodes and erythematous plaques, but in a severe reaction, bullae, ulcerations, and necrosis may occur. Relapses occur one year or more after the RFT whilst the symptoms emerge slowly and gradually. We reported a case of multibacillary leprosy relapse with ENL bullous reaction in a 14-year-old boy. The clinical picture consisted of reddish nodes and thickening red plaques since 10 days before admission to the hospital, followed by fluid-filled blisters associated with fever. The dermatological status showed multiple erythematous nodes and plaques, as well as multiple bullae. The laboratory tests indicated AFB (+), MI 6.5%, and BI 4+. The patient was stated as RFT after 1 year of MDT-MB treatment. The patient was treated with methylprednisolone 1x40 mg intravenous injection for 10 days, followed by oral methylprednisolone 4 mg in reduced dosage. Conclusion: This case was diagnosed as MHMB relapse with ENL bullous reaction based on the anamnesis, clinical features such as multiple erythematous nodules and plaques with bullae, and laboratory tests. Treatment with corticosteroids and MDT-MB resulted in good improvement.Keywords: ENL bullous reaction, relapseAbstrak: Manifestasi klinis reaksi ENL bulosa umumnya berupa nodi dan plak eritematosa, namun pada reaksi berat dapat berupa bula, ulserasi, dan nekrosis. Relaps terjadi 1 tahun atau lebih setelah RFT, dimana gejala timbul lambat dan bertahap. Kami melaporkan suatu kasus MHMB relaps dengan reaksi ENL bulosa pada seorang anak berusia 14 tahun. Gambaran klinis didapatkan bentol kemerahan dan bercak merah meninggi sejak 10 hari lalu, kemudian timbul lepuh berisi cairan disertai demam badan. Status dermatologis generalisata ditemukan nodul dan plak eritematosa multipel serta bula multipel. Pemeriksaan laboratorium menunjukkan BTA (+), MI 6,5%, dan BI 4+. Pasien dinyatakan RFT setelah 1 tahun pengobatan MDT-MB. Pasien diobati dengan metilprednisolon injeksi 1x40 mg intravena selama 10 hari, dilanjutkan dengan metilprednisolon oral 4mg dengan dosis diturunkan. Lesi bulosa pada kusta merupakan manifestasi dari reaksi ENL berat dengan MI dan BI yang tinggi. Simpulan: Pada kasus ini, diagnosis MHMB relaps dengan reaksi ENL bulosa ditegakkan berdasarkan anamnesis, gambaran klinis berupa nodul dan plak eritematosa multipel disertai bula, dan pemeriksaan laboratorik. Pengobatan dengan kortikosteroid dan MDT-MB memberikan hasil yang baik.Kata kunci: reaksi ENL bulosa, relaps