Yazc Nalan, Yalçn Bilgehan, Ciftci Arbay O, Orhan Diclehan, Haliloglu Mithat, Büyükpamukçu Münevver
Department of Pediatric Oncology, Hacettepe University,Ankara, Turkey.
J Pediatr Hematol Oncol. 2008 Jan;30(1):77-80. doi: 10.1097/MPH.0b013e31815b1aa5.
Skin and/or pulmonary involvement occur frequently in Langerhans cell histiocytosis (LCH) whereas nail involvement is rare. Herein, we present a case of LCH with initial nail disease and subsequent lung involvement causing recurrent pneumothoraces. Systemic chemotherapy was applied and intrapleural bleomycine and thoracoscopic pleurodesis with talc were performed. Although prognosis is not satisfactory in LCH with recurrent pneumothorax and nail involvement, the patient is under follow-up with no evidence of skin lesions, nail involvement, and pneumothorax for the last 10 months.
皮肤和/或肺部受累在朗格汉斯细胞组织细胞增多症(LCH)中很常见,而指甲受累则很少见。在此,我们报告一例LCH患者,最初表现为指甲病变,随后出现肺部受累导致反复气胸。应用了全身化疗,并进行了胸膜腔内注射博来霉素和滑石粉胸腔镜胸膜固定术。尽管伴有反复气胸和指甲受累的LCH预后并不理想,但该患者在过去10个月的随访中未出现皮肤病变、指甲受累和气胸的迹象。