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Sengkey, Triomega F. X.
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Penyakit Adamantiades-Behcet Koinfeksi dengan Herpes Genital Korompis, Charly M. M.; Sengkey, Triomega F. X.; Gaspersz, Shienty; Niode, J.
Jurnal Biomedik : JBM Vol 10, No 2 (2018): JURNAL BIOMEDIK : JBM
Publisher : UNIVERSITAS SAM RATULANGI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.35790/jbm.10.2.2018.20092

Abstract

Abstract: Adamantiades-Behcet (AB) disease is a rare multi-systemic inflammatory disorder with unknown cause. It is characterized by recurrent mucocutaneous ulcer in the mouth and genitalia, and mostly affects the age group of 20 to 30 ys. Genital lesion of AB must be differentiated from others caused by sexually transmitted disease, such as genital herpes. Co-infection of AB with genital herpes is very rare. We reported a male 72-year old, came with painful ulcers in the mouth and genital area since a week before visit. Symptoms were recurrent since three years ago. History of arthritis, recurrent headache, eye and skin lesions were denied. Patient was sexually promiscuous. Physical examination revealed multiple ulcers on the labium oris sized 0.5cm-1cm, irregular border, base covered by fibrin tissue, associated with erosion and crust. Multiple ulcers were also found on penile glans, sized 0.1x1x0.2 cm, irregular border, with pus and necrotic tissue. The ulcers were punched out. Pathergy test and anti HSV-1 IgM were negative meanwhile anti HSV-1 IgG, anti HSV-2 IgM as well as anti HSV-2 IgG were positive. Acyclovir 200mg 5x/day for five days, triamcinolone acetonide lotion bid for the mouth ulcer, NaCl 0,9% dressing applied tid for 30 minutes, and fucidic acid cream bid were given and the symptoms improved after 10 days. The prognosis was bonam for ad vitam and dubia for ad functionam and ad sanationam. Conclusion: The diagnosis of AB was based on the International Criteria for Behcet Disease, with a total score of 4 for the recurrent ulcer in mouth and genital area. The positive result of anti HSV-1 IgG, anti HSV-2 IgM and anti HSV-2 IgG supported the coinfection with genital herpes. This coinfection of AB and genital herpes was the first reported in Manado. Immunosenescence was a possible risk factor of the recurrent genital herpes. Symptomatic and antiviral treatment improved the symptoms with possible recurrent genital herpes.Keywords: Adamantiades-Behcet, genital herpes, coinfectionAbstrak: Penyakit Adamantiades-Behçet (AB) merupakan kelainan inflamasi multisistemik yang tidak diketahui penyebabnya dengan manifestasi mukokutan tersering berupa ulkus berulang pada mulut dan genital. Penyakit ini terutama terjadi pada usia 20-30an. Lesi genital pada AB perlu dibedakan dengan ulkus genital akibat infeksi menular seksual termasuk herpes genitalis. Koinfeksi AB dan herpes genitalis jarang terjadi. Kami melaporkan kasus seorang laki-laki, usia 72 tahun, dengan keluhan luka di bibir dan kelamin yang nyeri sejak 1 minggu lalu, bersifat hilang-timbul selama 3 tahun terakhir. Riwayat nyeri sendi, sakit kepala berulang, serta kelainan kulit disangkal. Terdapat riwayat promiskuitas yang tinggi. Pada pemeriksaan fisik di regio labialis oris ditemukan ulkus multipel, diameter ±0,5-1 cm, tepi tidak teratur, dasar tertutup jaringan fibrin, dengan erosi dan krusta. Di regio glans penis ditemukan ulkus multipel, ukuran bervariasi ± 1x2x0,2cm, tepi tidak teratur, dasar tertutup pus dan jaringan nekrotik, terdapat punch out dan erosi. Tes patergi negatif. Pemeriksaan anti HSV-1 IgM (-), anti HSV-1 IgG (+), anti HSV-2 IgM dan IgG (+). Terapi asiklovir 5 x 200 mg/hari selama 5 hari, salep triamsinolon asetonid 2 kali oles, kompres terbuka NaCl 0,9% 3 x30 menit/hari, krim asam fusidat 2 kali oles, memberikan perbaikan klinis setelah 10 hari pengobatan. Prognosis quo ad vitam bonam, quo ad functionam, quo ad sanationam ad dubia. Simpulan: Pada kasus ini, diagnosis AB ditegakkan berdasarkan International Criteria for Behcet Disease yaitu ditemukannya ulkus berulang di mulut dan di genital, masing-masing mendapat nilai 2, sehingga nilai total ialah 4. Ditemukannya anti HSV-1 IgG, anti HSV-2 IgG dan IgM positif, menunjang diagnosis tambahan herpes genital (rekuren). Koinfeksi AB dengan herpes genital baru pertama kali dijumpai di Manado. Keadaan immunosenescence kemungkinan menjadi faktor pencetus terjadinya rekurensi herpes genital. Pasien sembuh dengan terapi simtomatis dan antivirus, meskipun kemungkinan rekurensi dapat terjadi lagi.Kata kunci: Adamantiades-Behcet, herpes genital, koinfeksi
Iktiosis Harlequin di Manado - Laporan Kasus Sengkey, Triomega F. X.; Adji, Aryani; Kapantow, Grace M. 3Johny Rompis; Rompis, Johny
Jurnal Biomedik : JBM Vol 10, No 1 (2018): JURNAL BIOMEDIK : JBM
Publisher : UNIVERSITAS SAM RATULANGI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.35790/jbm.10.1.2018.19004

Abstract

Abstract: Harlequin ichthyosis is a severe autosomal recessive congenital ichthyosis (ARCI) due to mutation of ABCA12 gene, resulting defects in lamellar granules of stratum granulosum and extracellular lipid of the skin. It is rarely found and patients generally die a few days after birth. Harlequin ichthyosis is characterized by thickened stratum corneum separated by reddish fissures forming a geometric Harlequin pattern, impaired formation of ears, ectropion, and eclabium. Skin hydration and lubrication are recommended. Administration of systemic retinoid (acitretin 1mg/kg/day) may increase life expectancy. We reported the third Harlequin ichthyosis case since 1994 in Manado. A full-term, 2-day old baby, came with yellowish black crust on most of the body surface area since birth. Physical examination showed severely ill condition, generalized yellowish black hyperkeratosis, and fissures, auricle hypoplasia, ectropion, and eclabium. Diagnosis was based on specific clinical features. The patient was nursed in the incubator with NGT inserted, given amoxicillin 3x50mg IV drip, emollient (pseudoceramide, phytosterols, stearic acid, vegetable oil, squalene, and Zn oxide), fusidic acid cream, and NaCl 0.9% dressing. Chloramphenicol eye ointment 2xapp/day and artificial tear drops 1 drop/hour. The prognosis was poor and the patient died on the 7th day of treatment (aged 8 days) due to sepsis. Conclusion: A Harlequin ichtyosis case was diagnosed based on the specific clinical features. Management was not optimal due to the difficulties in maintaining peripheral venous system access for the provision of systemic therapy, nutrition, and laboratory examination. Moreover, the unavailability of acitretin worsened the prognosis.Keywords: Harlequin ichtyosisAbstrak: Iktiosis Harlequin adalah tipe autosomal recessive congenital ichthyosis (ARCI) yang berat akibat mutasi gen ABCA12 sehingga tidak terbentuk badan lamelar pada stratum granulosum dan hilangnya lamela lipid ekstrasel. Penyakit ini sangat jarang ditemukan dan umumnya pasien meninggal beberapa hari setelah kelahiran. Gambaran klinis berupa lempengan stratum korneum tebal yang dipisahkan oleh fisura kemerahan membentuk pola geometrik, gangguan pembentukan telinga, ektropion. dan eklabium. Diagnosis ditegakkan berdasarkan gambaran klinis yang khas. Penatalaksanaan umumnya simtomatis terutama hidrasi dan lubrikasi. Retinoid sistemik (acitretin 1mg/kgBB/hari) dapat meningkatkan harapan hidup. Kami melaporkan kasus iktiosis Harlequin ketiga sejak tahun 1994 di Manado. Seorang bayi aterm usia 2 hari dikonsul dari Bagian Anak dengan kulit berkerak hitam kekuningan pada hampir seluruh tubuh sejak lahir. Pada pemeriksaan fisik pasien tampak sakit berat, ditemukan hiperkeratosis hitam kekuningan, fisura, hipoplasi aurikula, ektropion, dan eklabium. Diagnosis ditegakkan berdasarkan anamnesis dan pemeriksaan fisik yang khas. Pasien dirawat dalam inkubator dan pemberian nutrisi melalui NGT. Terapi yang diberikan amoksisilin drip 3x50mg (0,5ml)/hari, emolien (pseudoceramide, phytosterol, stearic acid, vegetable oil, squalene, Zn oxide) 2xoles/hari, krim asam fusidat 2xoles/hari, kompres NaCl 0,9% 2x30menit/hari. Pada mata diberikan salep kloramfenikol 2xoles/hari dan airmata artifisial 1 tetes/jam. Prognosis quo ad vitam, quo ad functionam, quo ad sanationam malam. Pasien meninggal pada hari perawatan ke 7 (usia bayi 8 hari) karena sepsis. Simpulan: Kasus iktiosis Harlequin dengan diagnosis ditegakkan berdasarkan gambaran klinis yang khas. Penanganan pada kasus ini tidak maksimal karena hambatan akses vena perifer untuk pemberian terapi sistemik, nutrisi, dan pemeriksaan laboratorium, serta ketidaktersediaan acitretin yang memperburuk prognosis.Kata kunci: iktiosis Harlequin