Langerhans cell histiocytosis (LCH) is a rare disorder of proliferation of myeloid derived dendritic cells. Nail involvement in LCH is extremely uncommon. We report a child who presented with dystrophic changes in all 20 nails along with skin, dental, respiratory and abdominal findings who was diagnosed as pulmonary tuberculosis (PTB) and multisystem LCH. This is the fourth case being reported having nail changes with multisystem involvement, including high risk organs (liver and spleen), and the first case worldwide to be reported as microbiological confirmed PTB with multisystem LCH and dystrophy of 20 nails. In the setting of a multisystem presentation in an endemic country for tuberculosis, where diagnosis is challenging, the characteristic nail changes of LCH help in the diagnosis.
Key words: Langerhans cell histiocytosis; Nail changes; Pulmonary tuberculosis; Multisystem Langerhans cell histiocytosis.