Pulmonary IgG4+ Rosai-Dorfman disease

El‐Kersh, Karim; Perez, Rafael L.

doi:10.1136/bcr-2012-008324

Cited by 11 publications

(7 citation statements)

References 15 publications

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“…Rosai–Dorfman disease is a rare chronic systemic non‐Langerhans cell histiocytosis. Although Rosai–Dorfman disease can present with increased IgG4‐positive plasma cells and can even be coincidental with IgG4‐related skin disease; consistent and unique histopathologic and immunohistochemical findings in the skin make their distinction from IgG4‐related skin disease straightforward. For example, while histiocytes can be observed in cutaneous IgG4‐RD, they are negative for S‐100, and emperipolesis is not observed, as was the case for the patient described at our institution (Fig.…”

Section: Methodsmentioning

confidence: 99%

IgG4‐related skin disease may have distinct systemic manifestations: a systematic review

et al. 2016

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IgG4-related disease (IgG4-RD) is an increasingly prevalent protean multisystem disorder characterized by single or multi-organ infiltration of IgG4-bearing plasma cells. Skin involvement has been recognized and is relevant to proper diagnosis. A systematic literature review of 50 cases involving the skin reveals that patients with IgG4-related skin disease show predominant involvement of the head and neck and have a distinct pattern of systemic involvement, also favoring the head and neck - lymphatics, orbit, salivary, and lacrimal glands - but generally lacking pancreaticobiliary involvement (16% of cases), which by contrast is a predominant manifestation in systemic IgG4-RD (60% with pancreaticobiliary involvement). We summarize clinical and pathologic descriptive data from this systematic review. We review differential diagnosis and propose a diagnostic scheme for stratifying probability of disease based upon comprehensive integration of clinical, histopathologic, and laboratory data. Plasmacyte infiltration and storiform fibrosis are prominent in IgG4-related skin disease, but obliterative venulitis is less common than in the prototypical IgG4-related disease manifestation of autoimmune pancreatitis. IgG4 tissue and serum values, with a mean (±95% CI) in the reviewed cases of 132.8 ± 32.6 IgG4-positive plasma cells per high-power field and 580 ± 183.8 mg/dl, respectively, are incorporated into the suggested criteria. The distinct set of manifestations identified by this systematic review and the proposed diagnostic considerations, while requiring further validation in prospective studies, highlight the need to consider that IgG4-related skin disease defines a unique systemic disease complex along the spectrum of IgG4-RD.

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Section: Methodsmentioning

confidence: 99%

IgG4‐related skin disease may have distinct systemic manifestations: a systematic review

et al. 2016

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“…His identical twin brother carries a diagnosis of Rosai-Dorfman disease, a histiocytic disorder characterized by S100 + and CD68 + histiocytes with intact lymphocytes within their cytoplasm (emperipolesis). Interestingly Rosai-Dorfman disease has also shown overlap with IgG4-RD [14] , [15] , [16] .…”

Section: Discussionmentioning

confidence: 99%

Adult-onset asthma and periocular xanthogranulomas associated with systemic IgG4-related disease

Burris

Rodríguez

Raven

et al. 2016

American Journal of Ophthalmology Case Reports

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PurposeThe aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD).ObservationsA 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity. The IgG and IgG4 stained slides showed increased IgG4 positive plasma cells but did not meet the criteria for IgG4-related orbital disease. His IgG4 serology was elevated, and IgG4 staining of his tissue previously diagnosed as autoimmune sclerosing pancreatitis was found to meet the criteria for IgG4-RD.Conclusion and importanceAAPOX can be associated with systemic IgG4-RD.

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“…При ИГХ-исследовании гистиоциты положительны к CD11c, CD14, CD15, CD68, CD163 и α 1 -антихимотрипсину, но отрицательны к CD1a и лангерину [28]. В отличие от синусного гистиоцитоза в клетках образования отмечается экспрессия S100 [29].…”

Section: рис 3 эпителиоидная гемангиоэндотелиома в сочетании с амилunclassified

“…Смертность наблюдается в 7% случаев болезни Розаи-Дорфмана, однако описаны и случаи спонтанной регрессии заболевания [27]. В отличие от других локализаций поражение легких при болезни Розаи-Дорфмана характеризуется плохим прогнозом, больные умирают в 1 / 3 наблюдений [28].…”

Section: рис 3 эпителиоидная гемангиоэндотелиома в сочетании с амилunclassified

Solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal amyloidosis of the lung and Rosai-Dorfman disease

et al. 2018

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The paper describes a case of solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal AL amyloidosis of the lung and Rosai-Dorfman disease in a 70-year-old woman. The core of the tumor was represented by bone tissue with dendriform ossification, as well as by amyloid that showed green apple birefringence at polarized light microscopy. The peripheral portions of the tumor and the myxohyaline stroma exhibited slit-like structures, epithelioid and fusiform cells with small cytoplasmic vacuoles. These cells expressed CD31, CD34, factor VIII, and cytokeratins 7 and 18. The Ki-67 proliferation index was 10%. S100- and CD68-positive histiocytes with the phenomenon of emperipolesis were revealed in the tumor and in the lymph nodes of the mediastinum and lung hilum. There was a positive reaction to immunoglobulin lambda light chains in the lymphocytic infiltration around amyloid clumps. The frequency of epithelioid hemangioendothelioma was less than 1 case per million people annually. We found only one case of its concurrence with pulmonary amyloidosis in the English-language literature. No relationship could be revealed between this tumor and Rosai-Dorfman disease.

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Pulmonary IgG4+ Rosai-Dorfman disease

Cited by 11 publications

References 15 publications

IgG4‐related skin disease may have distinct systemic manifestations: a systematic review

IgG4‐related skin disease may have distinct systemic manifestations: a systematic review

Adult-onset asthma and periocular xanthogranulomas associated with systemic IgG4-related disease

Solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal amyloidosis of the lung and Rosai-Dorfman disease

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