Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa.
Epidermolysis bullosa (EB) is a rare disorder characterized by inherited skin adhesion defects with abnormal disruption of the epidermal-dermal junction in response to mechanical trauma. Our aim was to investigate a set of cytokine levels in serum samples from patients suffering from epidermolysis bullosa simplex (EBS), dystrophic epidermolysis bullosa (DEB), and healthy controls (HCs), exploring their potential correlations with antiskin autoantibody titers and disease activity. Forty patients afferent to the Dermatological Ward of Bari City Hospital and 9 HCs were enrolled and subdivided according to the dystrophic (DEB) and simplex forms (EBS). We found a significant increase in interleukin (IL)-1β plasmatic levels of DEB (P = 0.0224) and EBS (P = 0.0465) patients compared to HCs; IL-6 levels were significantly higher in DEB than in EBS patients (P = 0.0004) or HCs (P = 0.0474); IL-2 levels were significantly increased in DEB compared with EBS (P = 0.0428). Plasmatic tumor necrosis factor-β and interferon-γ were higher in DEB patients than in HCs (P = 0.0448 and 0.0229). Conversely, tumor necrosis factor-α was significantly decreased in DEB (P = 0.0034). IL-5 correlated with anti-BP180 (r = -0.5018, P = 0.0338), anti-BP230 (r = -0.6097, P = 0.0122), and anticollagen VII (r = -0.5166, P = 0.0405) autoantibodies; interferon-γ correlated with anti-BP180 (r = 0.9633, P < 0.0001), anti-BP230 (r = 0.9071, P < 0.0001), and anticollagen VII (r = 0.8619, P = 0.0045) autoantibodies. Score of disease severity was significantly correlated with IL-6 (r = 0.6941, P = 0.029) and IL-12 (r = 0.5503, P = 0.0272). The present study supports that EB might be considered a systemic inflammatory disease rather than a skin-limited disorder; clinical disease activity scores could be also integrated by laboratory data such as IL-6 and IL-12 dosage; biotherapies targeting specific cytokine networks probably represent a way to go in the future.
From the Regional Coordination for Rare Diseases-Ares Puglia, Bari (GA); Department of Clinical and Experimental Medicine, University of Foggia, Foggia (MGM); Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, University of Milan, Milan (SE); Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari, Bari (G Lopalco, G Lapadula, FI); Pharmaceutical Department of Medicine (ML); Laboratory of Clinical Pathology (MT); Section of Dermatology, Department of Biomedical Science and Human Oncology (DB); Department of Biomedicine of the Developmental Age, Pediatric Unit "S. Maggiore" (LB); and Research Center of Systemic Autoinflammatory Diseases and Behcet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy (AV, LC).
Giuseppina
G
Annicchiarico
Maria Grazia
MG
Morgese
Susanna
S
Esposito
Giuseppe
G
Lopalco
Michele
M
Lattarulo
Marilina
M
Tampoia
Domenico
D
Bonamonte
Luigia
L
Brunetti
Antonio
A
Vitale
Giovanni
G
Lapadula
Luca
L
Cantarini
Florenzo
F
Iannone
D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D016207:Cytokines / Q000276:immunology*; D004820:Epidermolysis Bullosa / Q000097:blood* / Q000235:genetics / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D012867:Skin / Q000276:immunology*; D055815:Young Adult
D001323:Autoantibodies; D016207:Cytokines