Skin Signs in Pulmonary Disease

Necrobiotic xanthogranuloma (NXG) is a chronic, progressive non-Langerhans histiocytic granulomatous disease. While case reports describing periorbital involvement of NXG are frequent, only a few case reports describing ocular involvement, such as scleritis and uveitis, exist. Herein, we present a case presenting initially as bilateral anterior and posterior scleritis, as well as a chronic bilateral granulomatous panuveitis, and discuss the immunosuppressant options that should be considered for this disease with protean manifestations.

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Spontaneous pneumothorax as a clinical manifestation of neurofibromatosis type 1

Lorentzen

Madsen

Lausten-Thomsen

et al. 2021

BMJ Case Rep

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Neurofibromatosis type 1 (NF1) is a genetic disorder affecting the skin, nervous system, eyes and bones. Pulmonary involvement is unknown to many physicians. Yet, patients may be affected by lung bullae and cysts, which represent an increased risk for secondary spontaneous pneumothorax (SSP). We present a 56-year-old patient with a pathogenic variant of the NF1 gene, who suffered from NF1 with lung manifestations and recurrent SSP. It is essential to identify the patients having an increased risk of developing SSP as preventive surgery seem to decrease the risk of new events. Pneumothorax can be a clinical manifestation of NF1 but is not yet widely acknowledged as such.

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Skin Signs in Pulmonary Disease

Cited by 4 publications

References 41 publications

History and Physical Examination

History and Physical Examination

Management of Uveitis and Scleritis in Necrobiotic Xanthogranuloma

Spontaneous pneumothorax as a clinical manifestation of neurofibromatosis type 1

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