Langerhans’ cell histiocytosis is an uncommon disease in children with varied clinical presentation. Multisystem form of this disorder usually affects organs like the bones, skin, liver, spleen, lungs, and the central nervous system. We describe here the clinical details of a 2-year-old girl with involvement of unusual sites like the parotid glands and the nails. This child also had multiple cystic lesions in the liver leading to a misdiagnosis of Caroli’s disease. Knowledge about the uncommon manifestations of this rare disorder helps in early diagnosis and treatment.
A 9-year-old girl presented with lower motor neuron type of paralysis involving limbs, trunk and multiple cranial nerves (7, 9 and 10) with preceding history of mumps 1 week before the onset of weakness. There were no features to suggest either a meningitis or encephalitis in the child. Cerebrospinal fluid showed hypoglycorrhachia and mild protein elevation; magnetic resonance imaging of the brain was normal. Nerve conduction study showed motor axonal neuropathy. Serology for mumps IgM was positive, consistent with a diagnosis of post-mumps acute motor axonal polyneuropathy. The girl made a complete recovery within 3 weeks.
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