Adult Langerhans’ cell histiocytosis with multisystem involvement

Abstract

RATIONALE: Langerhans' cell histiocytosis (LCH), also called histiocytosis X, is an uncommon disorder manifesting in a variety of ways. Although LCH can involve various organs including bone, skin, and lymph nodes, multisystem involvement of LCH is rare in adults. PATIENT CONCERNS: A 31-year-old woman first presented to our hospital with left leg pain. She had a history of a 20-kg weight gain over three months. DIAGNOSES: X-ray, magnetic resonance imaging (MRI), computed tomography (CT), and bone scan images revealed enhancing lesions in the left femur and right temporal bone, multiple cystic lesions in the lung, enhancing mass in the pituitary stalk, and fat density lesions in the liver. The patient underwent excisional biopsy for the femoral lesion and histologic examination confirmed the diagnosis of LCH. INTERVENTIONS: Excisional biopsy was performed for the bony lesion in the left femur. She received chemotherapy with vinblastine and prednisolone. OUTCOMES: The patient expired after 21 months from initial admission following recurrent episodes of pneumothorax, pneumonia, and sepsis. LESSONS: Our case showed LCH involvement in bone, lung, central nervous system (CNS), and liver. Although it is occasionally difficult to discriminate LCH from other disorders, systemic evaluation might be helpful for differential diagnosis. Familiarity with the various multisystemic involvements of LCH on imaging is vital for diagnosing and managing patients in daily practice.