ABSTRAKGiant congenital melanocytic nevus (GCMN) merupakan penyakit langka yang timbul saat lahir akibat sel melanosit tumbuh berlebih. Lesi pada GCMN sering ditemukan pada area punggung dan paha. Gambaran lesi berupa bercak hiperpigmentasi dengan ukuran lebih dari 20 cm dan terdapat rambut. Kondisi ini sering dihubungkan dengan neurokutaneus melanositosis dan melanoma maligna. Risiko neurokutaneus melanositosis meningkat signifikans bila lesi giant nevi terdapat pada regio kranial atau garis midline atau disertai lesi satelit. Rerata kumulatif risiko terjadinya melanoma dalam 5 tahun sebesar 4,5%. Pendekatan terapi masih menjadi tantangan dan bersifat individual bergantung pada usia, lokasi lesi, ukuran, risiko melanoma dan kemungkinan kelainan fungsi akibat tindakan invasif yang dilakukan serta dampak fisiologis terhadap luka pasca tindakan. Dilaporkan bayi perempuan usia 2 hari, lahir pervaginam, cukup bulan dari seorang ibu P4A0 menderita SLE yang diterapi metilprednisolon selama kehamilan. Status generalis dalam batas normal. Status dermatologikus pada regio skalp, fasialis, trunkus, ekstremitas superior dan inferior bilateral tampak makula-plak hiperpigmentasi, multipel, bulat-irregular, lentikuler-plakat, diskret; sebagian terdapat rambut, kulit sekitar normal; terdapat lesi satelit. Pada regio ekstremitas inferior sinistra terdapat lesi giant nevi dengan ukuran lebih dari 20 cm. Pada kasus ini diperlukan observasi yang baik dan pendekatan multidisiplin dalam tatalaksana GCMN.
ABSTRACT
Giant congenital melanocytic nevus (GCMN) is a rare disease caused by excessive growth of melanocytes. It appeared since birth. This disease often found on the back and thigh. Lesion of GCNM often appear as a solitary or multiple well defined hyperpigmented hairy plaque measuring 20 cm or larger. This condition is frequently associated with neurocutaneous melanocytosis and malignant melanoma. The risk of neurocutaneous melanocytosis significantly increases if giant nevus lesion located on cranial or midline region or accompanied by satellite lesions. Cumulative 5-year risk for the growth of melanoma is 4,5%.Therapeutic approach is still a challenge and varies among each individual according to age, location, size of lesion, risk of melanoma and possibility of function abnormalities from invasive treatment and physiological impact due to post treatment effect. We reported a case of two-days-old full term female, born naturally and full term with GCMN. The mother suffered systemic lupus erythematosus and received methylprednisolone during pregnancy. General appearance was unremarkable. Dermatological physical examination present with a multiple well defined round -irregular hyperpigmented macules and plaques, some area were covered with hair and some surrounded with satellite papules on the truncus, scalp, facialis, extremities superior et inferior bilateral region. There is giant nevi lesion with size > 20 cm on the left lower extremity. Close observation dan multidiscipline approach is needed for management of GCMN in our ca...