Diabetes Insipidus is secondary to infiltration of the hypothalamus or pituitary gland. Skin lesions are common, manifesting as vesiculo-pustular lesions especially in the acute disseminated form. Intracranial involvement may be due to contiguous spread of skull lesions or to granulomatous infiltration of the brain, although it is usually limited to the hypothalamus. A late neurologic syndrome that includes cerebellar ataxia may occur in patients years after they appeared to be disease-free. Other manifestations include involvement of lymph nodes and spleen, bone marrow involvement with anemia, neutropenia and thrombocytopenia, hepatomegaly with abnormal liver function and involvement of the genital tract in young women.
Ocular involvement includes orbital, intraocular, neuro-ophthalmological, and lid disturbances.
It is usually an unifocal granuloma with a predilection for the superotemporal orbital bone. Orbital soft tissue involvement without an obvious bone defect is rare. Intraocular involvement is rare. It is usually seen in infants with the subacute disseminated form and consists of infiltration of intraocular structures, especially of the uvea. Optic neuropathy and cranial nerves palsies are rare as the orbital mass is usually extraconal and destruction of the orbital wall may result in orbital decompression.