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1.
Dermatol Online J ; 26(8)2020 Aug 15.
Article in English | MEDLINE | ID: mdl-32941714

ABSTRACT

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disorder that must be distinguished from systemic lupus erythematosus (SLE). Although a minority of patients with KFD develop SLE, most patients have a self-limited disease. Importantly, KFD can have skin manifestations resembling cutaneous lupus. Therefore, the diagnosis of SLE should be predicated on a complete rheumatologic workup and not on the constellation of skin disease and lymphadenitis. Nonetheless, as our exceedingly rare case illustrates, patients who do not initially meet diagnostic criteria for SLE require dermatologic follow-up. We present a young adult woman who had a remote history of KFD and later presented with combined features of discoid lupus and lupus erythematosus panniculitis (LEP). On subsequent rheumatologic workup, she fulfilled criteria for SLE. We discuss the differential diagnosis of both KFD and LEP and emphasize how strong communication among dermatologists and other healthcare providers is essential in the management of patients with KFD.


Subject(s)
Histiocytic Necrotizing Lymphadenitis/complications , Panniculitis, Lupus Erythematosus/complications , Skin/pathology , Adult , Diagnosis, Differential , Female , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/pathology , Humans , Lupus Erythematosus, Systemic/diagnosis , Panniculitis, Lupus Erythematosus/diagnosis , Panniculitis, Lupus Erythematosus/drug therapy
3.
JCI Insight ; 1(17): e88912, 2016 Oct 20.
Article in English | MEDLINE | ID: mdl-27777975

ABSTRACT

While respiratory failure in cystic fibrosis (CF) frequently associates with chronic infection by Pseudomonas aeruginosa, no single factor predicts the extent of lung damage in CF. To elucidate other causes, we studied the autoantibody profile in CF and rheumatoid arthritis (RA) patients, given the similar association of airway inflammation and autoimmunity in RA. Even though we observed that bactericidal permeability-increasing protein (BPI), carbamylated proteins, and citrullinated proteins all localized to the neutrophil extracellular traps (NETs), which are implicated in the development of autoimmunity, our study demonstrates striking autoantibody specificity in CF. Particularly, CF patients developed anti-BPI autoantibodies but hardly any anti-citrullinated protein autoantibodies (ACPA). In contrast, ACPA-positive RA patients exhibited no reactivity with BPI. Interestingly, anti-carbamylated protein autoantibodies (ACarPA) were found in both cohorts but did not cross-react with BPI. Contrary to ACPA and ACarPA, anti-BPI autoantibodies recognized the BPI C-terminus in the absence of posttranslational modifications. In fact, we discovered that P. aeruginosa-mediated NET formation results in BPI cleavage by P. aeruginosa elastase, which suggests a novel mechanism in the development of autoimmunity to BPI. In accordance with this model, autoantibodies associated with presence of P. aeruginosa on sputum culture. Finally, our results provide a role for autoimmunity in CF disease severity, as autoantibody levels associate with diminished lung function.


Subject(s)
Autoimmunity , Cystic Fibrosis/immunology , Extracellular Traps/immunology , Adolescent , Adult , Anti-Citrullinated Protein Antibodies/blood , Antimicrobial Cationic Peptides/immunology , Arthritis, Rheumatoid/immunology , Blood Proteins/immunology , Cross Reactions , Female , Humans , Male , Membrane Proteins , Middle Aged , Pseudomonas Infections/immunology , Pseudomonas aeruginosa , Young Adult
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