Swan neck deformity is a hyperextension of the proximal interphalangeal (PIP) joints and flexion of the distal interphalangeal (DIP) joints. Claw hand is a hyperextension of the metacarpal joints and flexion of the PIP joints, accompanied by reduced motor strength. A 23-year-old female, who was released from leprosy treatment, presented with a bend of the second to fifth fingers of both hands. There was hyperextension of the PIP joints and flexion of the DIP joints from the second to fifth fingers of both hands, thickening of the ulnar nerves, and hypoesthesia without motor impairment of the fourth and fifth fingers of both hands. Radiograph examination revealed cupping of the base of the proximal phalangeal joints of the second, third, and fifth fingers of the left hand and of the second and fifth fingers of the right hand. Additionally, narrowing of the metacarpophalangeal joints of the second, third, and fifth fingers of the right hand and sclerosis of the second and fifth fingers of the right hand were also observed. Claw hand is frequently reported in leprosy, while swan neck deformity is frequently reported in rheumatoid arthritis. To our knowledge, this is the first reported case with swan neck deformity caused by arthritis in leprosy.
Psoriasis disertai penyakit bulosa autoimun yang terjadi pada satu orang sangat jarang terjadi. Diduga terdapat peranan plasminogenactivator, predisposisi genetik, atau faktor pencetus infeksi dalam patogenesis psoriasis yang disertai pemfigus foliaseus. Metotreksat dilaporkan efektif sebagai terapi psoriasis pustulosa dan azatioprin sebagai terapi penyakit bulosa autoimun menimbulkan efek samping yang lebih dapat ditoleransi.Dilaporkan seorang pasien laki-laki berusia 38 tahun dengan diagnosis psoriasis pustulosa generalisata disertai pemfigus foliaseus. Selain gambaran klinis, diagnosis psoriasis pustulosa ditegakkan dengan pemeriksaan histopatologis, sedangkan diagnosis pemfigus foliaseus ditegakkan melalui pemeriksaan histopatologis dan direct immunofluorescence (DIF). Pasien diterapi dengan kombinasi metotreksat 3x5 mg/minggu dan azatioprin 2x100 mg/hari. Setelah dua minggu mendapat terapi kombinasi, terjadi perbaikan klinis dan tidak ditemukan lesi baru. Psoriasis dan penyakit bulosa autoimun pada satu orang sulit terapinya karena penggunaan dan penghentian kortikosteroid sistemik dapat mencetuskan psoriasis pustulosa. Pada pasien ini, dipilih metotreksat sebagai terapi psoriasis pustulosa generalisata karena efektivitasnya baik dan tersedia di Indonesia. Azatioprin diberikan untuk terapi pemfigus foliaseus atas pertimbangan efek samping yang jarang terjadi dibandingkan obat imunosupresan lainnya. Dilaporkan satu pasien usia 38 tahun dengan psoriasis pustulosa generalisata disertai pemfigus foliaseus yang mendapatkan terapi kombinasi metotreksat 3x5 mg/minggu dan azatioprin 2x100 mg/hari. Perbaikan klinis didapatkan setelah dua minggu pengobatan. Kata kunci: azatioprin, metotreksat, pemfigus foliaseus, psoriasis pustulosa generalisata
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