Langerhans Cell Histiocytosis: Clinical, Histopathological and Radiological Case Series Study: An Institutional Experience
Maha El-Gayyar
Department of Dermatology, Faculty of Medicine, Mansoura University, Egypt.
Manar Sallam
Department of Dermatology, Faculty of Medicine, Mansoura University, Egypt.
Mohamed Khaled
Department of Dermatology, Faculty of Medicine, Mansoura University, Egypt.
Mohamed El-Mougy
Department of Dermatology, Faculty of Medicine, Mansoura University, Egypt.
Amany El-Hawwary
Department of Histology and Cell Biology, Faculty of Medicine, Mansoura University, Egypt.
Amal Halim *
Department of Clinical Oncology and Nuclear Medicine, Faculty of Medicine, Mansoura University, Egypt.
Hala El. Shenshawy
Department of Clinical Oncology and Nuclear Medicine, Faculty of Medicine, Mansoura University, Egypt.
Adel El-Badrawy
Department of Radiology, Faculty of Medicine, Mansoura University, Egypt.
*Author to whom correspondence should be addressed.
Abstract
Background: Langerhans cells histiocytosis (LCH) is a rare disease characterized by the uncontrolled proliferation of Langerhans cells (LCs). This study aimed to explore the clinic-epidemiological and pathological data of that disease in our population.
Methods: Sixteen patients were referred to the Dermatology and the Clinical Oncology & Nuclear Medicine Departments of our university during the 6 years from 2007 to 2012. Records data were retrospectively analyzed. Pathologic specimens and radiologic films were reassessed by consultants.
Results: Pediatric age and male sex predominated. The multifocal uni system (MUS) was the commonest presentation (50% of cases) while the multifocal multisystem (MMS) was found in 37.5% of cases including a case of Letterer-Siwe disease. Microscopic examination revealed dense infiltrate mainly of LCs with characteristic features and positive immunostaining for S100, CD1a and CD68. The treatment was heterogeneous. The follow-up time was well documented for 12 cases (mean=5 years). The prognosis was variable. Limitation of this study was the retrospective nature.
Conclusions: This case series showed the predominance of males, pediatric age, bone involvement and good treatment response. Diversity of system involvement necessitates a multidisciplinary approach. This diversity also could be a cause of underdiagnosis.
Keywords: LCH, Histiocytosis-x, skull infiltrative disease, hand schuller cristian disease.