Multifocal Langerhans Cell Granulomatosis (Hand-schuller - Christian Disease)

A 47 year old female developed multifocal Langerhans cell granulomatosis (Hand-Schuller-Christian disease) manifesting as papulo-pustular, nodular, crusted and scaly lesions, xanthelasma, ulceration of gingival mucosa with loss of teeth, vaginal granulomatosis, diabetes insipidus, multiple osteolytic bone lesions and honeycombing of the lungs. Skin biopsy confirmed the diagnosis. Treatment with prednisolone 45 mg methotrexate 2.5 mg daily, led to regression of lesions, reduction of bone of partial clearance of lung lesions and osteolytic bone lesions. However, the patient died due to widespread nature of the disease.