69 year old male with neurofibromatosis and emphysema

Neurofibromatosis with diffuse lung disease is a definable clinical entity, characterised by upper lobe cystic and bullous disease and lower lobe fibrosis. Its relationship to smoking remains unclear.

Links and References

Zamora, A.C et al Neurofibromatosis-associated lung disease: a case series and literature review   European Respiratory Journal 2007 29: 210-214;