title: Multiple cutaneous, oropharyngeal and pulmonary nodules.
abstract: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis defined by heterogeneous multiorgan involvement. Due to the rarity of this disease strong evidence-based therapies have yet to be established and prognosis has previously been considered to be poor, with more than half of patients dying within 3 years of initial presentation. We describe an 86-year-old woman with a 34-year history of extensive cutaneous and internal nodules with typical pathological and immunophenotypical (CD68(+) /CD1a(-) ) features of ECD without evidence of the BRAF V600E mutation. The cosmetic appearance of cutaneous nodules and hoarse voice caused by vocal cord nodules has been managed surgically. More aggressive therapies reported for ECD were trialled for this patient, such as radiotherapy and interferon-α, with no response. This case demonstrates a relatively good prognosis in ECD that has been managed conservatively.
authors: St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, SE1 4HT, U.K.
authors: O
authors: O
authors: Veraitch
authors: St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, SE1 4HT, U.K.
authors: K
authors: K
authors: Bhargava
authors: St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, SE1 4HT, U.K.
authors: B
authors: B
authors: Moriarty
authors: St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, SE1 4HT, U.K.
authors: E
authors: E
authors: Calonje
authors: St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, SE1 4HT, U.K.
authors: R
authors: R
authors: Mallipeddi
authors: St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, SE1 4HT, U.K.
authors: S
authors: S
authors: Whittaker
mesh_terms: D000369:Aged, 80 and over; D001847:Bone Diseases / Q000000981:diagnostic imaging; D002908:Chronic Disease; D031249:Erdheim-Chester Disease / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009059:Mouth Diseases / Q000473:pathology*; D064847:Multimodal Imaging; D055613:Multiple Pulmonary Nodules / Q000473:pathology*; D010608:Pharyngeal Diseases / Q000473:pathology*; D011877:Radionuclide Imaging; D012871:Skin Diseases / Q000473:pathology*; D014057:Tomography, X-Ray Computed