Background: Sturge-Weber Syndrome (SWS) is a neurocutaneous defect involving facial Port Wine Stain (PWS) with vascular deficits in the brain and ipsilateral eyes. Facial PWS can be the initial marker and help predict the severity of SWS. Aims and objectives: To study the association of facial PWS characteristics, neurological and ophthalmological deficits in SWS with the degree of severity. Materials and Methods: A retrospective descriptive study of patients with SWS was conducted at Dr. Sardjito Hospital between January 2010 - December 2020. Facial PWS characteristics were assessed from photographs. Assessments of neurological and ophthalmological deficits used physical and supportive examinations, including Electroencephalogram (EEG), Magnetic Resonance Imaging (MRI), and Intelligent-Quotient (IQ). Results: From 19 female patients aged 1 month to 26 years with facial PWS, seven fulfilled the inclusion criteria. “High risk” facial PWS V1 and P1 b distribution patterns were found in all patients. Most patients had neurological deficits of rare focal seizures (83%). Fine motor disorders (50%) were the most severe motoric deficits found. All patients experienced glaucoma with decreased visuality to blindness and hemianopsia. EEG epileptiform patterns, MRI showing angiomatosis and brain atrophy, and low IQ scores were recorded. Conclusions: Pathogenesis and degree of severity in SWS were related to facial PWS, neurological and ophthalmological deficits. Keywords: Sturge-weber syndrome; facial port-wine stain; neurological deficit; ophthalmological deficit; neurocutaneous disorder.

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Vitiligo adalah kelainan kulit yang ditandai dengan hilangnya pigmentasi akibat hilangnya melanosit epidermis fungsional. Pajanan sinar matahari dapat memicu peningkatan reactive oxygen species (ROS) pada kulit vitiligo. Simple 1-week sunlight exposure recall (S1WSER) telah digunakan untuk mengetahui hubungan pajanan sinar matahari dengan kadar serum vitamin D yang berperan pada penyakit vitiligo. Penelitian ini bertujuan untuk mengetahui peran pajanan sinar matahari terhadap perubahan fungsi biologis kulit vitiligo. Subjek penelitian ini adalah pasien vitiligo yang menjalani fototerapi narrow band ultraviolet B (NB -UVB). Jumlah pajanan sinar matahari harian dinilai dengan S1WSER yang terdiri atas total body areas exposed by sunlight (TBAES) dan time exposed by sunlight (TTES). Fungsi biologis kulit yang diukur adalah trans epidermal water loss (TEWL) menggunakan tewameter dan hidrasi kulit menggunakan korneometri pada kulit lesi yang terpajan sinar matahari (sun exposed lesion/SEL), lesi yang tidak terpajan sinar matahari (non sun exposed lesion/ NSEL), kulit tanpa lesi yang terpajan sinar matahari (sun exposed non lesion/SENL), dan kulit tanpa lesi yang tidak terpajan sinar matahari (non sun exposed non lesion/NSENL). Hasil penelitian menunjukkan hubungan bermakna (p<0.05) antara TBAES dengan selisih nilai korneometri SEL dan NSEL dengan kekuatan korelasi lemah (r<0.6).Sinar matahari berperan dalam perubahan fungsi biologis kulit pada vitiligo, pada penelitian ini didapatkan korelasi positif antara TBAES dengan selisih nilai korneometri SEL dan NSEL.Kata kunci: vitiligo, fungsi biologis kulit, pajanan sinar matahari