Desquamative interstitial pneumonia in a non-smoker with neurofibromatosis type 1 (Von Recklinghausen syndrome)

Neurofibromatosis, type 1 (von Recklinghausen disease).

Type 1 neurofibromatosis (von Recklinghausen disease).

Type 1 neurofibromatosis (NF1), or von 
Recklinghausen disease, is a genetic disorder that is well known for its clinical features. 
Effective treatment modalities for NF1 have not yet been established. The advent of new treatment options for NF1 such as topical vitamin D3 analogues, lovastatin, rapamycin (or sirolimus), and imatinib mesylate has added new dimensions that require further invest...

Von Recklinghausen disease (neurofibromatosis type 1): beyond skin involvement.

To cite: Rocha SM, Ferreira MB, Ribeiro R, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2013-200033 DESCRIPTION A 35-year-old black woman with Von Recklinghausen disease (or neurofibromatosis type 1) presented multiple café-au-lait spots, axillary freckling and multiple cutaneous neurofibromas, which were more expressive in the abdomen and trunk (figure...

Desquamative interstitial pneumonia.

The clinical and pathologic findings of 13 cases of desquamative interstitial pneumonia (D IP) are presented. The disease is characterized by large num­ bers of intra-alveolar mononuclear cells. Eight cases showed mild degrees of fibrosis at the time of biopsy. Six cases had arthritis or clinical and serologic features suggesting altered immunological activity. Three patients died of progressiv...

Desquamative interstitial pneumonia: relationship to interstitial fibrosis.

Patchefsky, A. S., Israel, H. L., Hoch, W. S., and Gordon, Gloria (1973). Thorax, 28, 680-693. Desquamative interstitial pneumonia: relationship to interstitial fibrosis. The clinical course and radiographic and pathological findings in 14 patients having the histological pattern of desquamative interstitial pneumonia (DIP) have been studied. Four deaths occurred from cardiorespiratory failure ...