Unfolding dermatological spectrum of Still’s disease: a cohort study from the International AIDA Network Still’s Disease Registry
Year: 2025
Type: article
Study design: Observational Study
Abstract: OBJECTIVES: To investigate cutaneous manifestations in Still's disease patients, evaluating any correlation with ethnic origin, age at disease onset, disease patterns, occurrence of macrophage activation syndrome (MAS) and systemic activity scores. METHODS: Data were retrospectively drawn from the International AutoInflammatory Disease Alliance (AIDA) Network Registry dedicated to Still's disease. RESULTS: A total of 518 patients (41.3% males) were enrolled. Salmon-coloured evanescent skin rash (n = 304, 63.9%), macules (n = 40, 7.7%), urticarial eruptions (n = 31, 5.9%), erythema (n = 27, 5.2%) and persistent pruritic papules and plaques (PPPP) (n = 25, 4.8%) accounted for the most frequent skin manifestations observed in Still's disease. Overall, atypical skin rash were described in 110 (21.2%) patients. Salmon-coloured evanescent skin rash and pruritus were more common among patients aged <16 years compared with patients aged 16-60 (P = 0.002 and P = 0.008, respectively). Pruritus was significantly more frequent among White than among Arab patients (P = 0.008) and in polycyclic vs monocyclic course (P = 0.049). Hispanics showed a significantly higher rate of atypical skin manifestations compared with Arabs (P = 0.036) and White (P = 0.036). Also, macules were more frequent among Hispanics than White (P = 0.027), while PPPP was more frequent among Hispanics than Arabs (P = 0.023) and White (P = 0.002). Salmon-coloured evanescent skin rash was significantly more frequent among patients with a systemic activity score ≥7 (P < 0.001). CONCLUSION: The present study enhances dermatologists' awareness of the diverse cutaneous lesions that may represent heterogeneous manifestations of Still's disease, shedding new light on the difference related to the age at disease onset, the patients' ethnic origin and the severity of the disease.
Source: Lara D. Veeken
Authors: Laura Calabrese, Martina D’Onghia, Alessandra Cartocci, Andrea Hinojosa-Azaola, Jiram Torres-Ruiz, Giuseppe Lopalco, Jessica Sbalchiero, Valeria Caggiano, Henrique A Mayrink Giardini, Ibrahim A Almaghlouth, Piero Ruscitti, Ilenia Di Cola, Petros P Sfikakis, Katerina Laskari, Paolo Sfriso, Lorenzo Dagna, Corrado Campochiaro, Abdurrahman Tufan, Hamit Kucuk, Riza Can Kardas, Abdulsamet Erden, Gaafar Ragab, Mohamed Tharwat Hegazy, Ahmed Hatem Laymouna, Luca Navarini, Onorina Berardicurti, Francesco Ciccia, Daniela Iacono, Flavia Riccio, Lampros Fotis, Haner Direskeneli, Joanna Makowska, Annamaria Iagnocco, Alessandro Conforti, Donato Rigante, Maissa Thabet, Florenzo Iannone, Daniele Domanico, Marcello Govoni, Maria Cristina Maggio, Emanuela Del Giudice, Francesco La Torre, Ezgi D Batu, Seza Ozen, Carla Gaggiano, Eduardo Martín-Nares, Guillermo Arturo Guaracha-Basañez, Anastasios Karamanakos, Alberto Lo Gullo, Benedetta Monosi, Elena Bartoloni, José Hernández-Rodríguez, Verónica Gómez-Caverzaschi, Giacomo Emmi, Sukran Erten, Francesco Carubbi, Maria Francesca Gicchino, Amato De Paulis, Giovanni Conti, Benson Ogunjimi, Ewa Wiesik-Szewczyk, Anna Nowakowska-Płaza, Ombretta Viapiana, Piercarlo Sarzi-Puttini, Samar Tharwat, Francesca Crisafulli, Paola Parronchi, Antonio Gidaro, Ludovico De Stefano, Luciana Breda, Lidia La Barbera, Giuliana Guggino, Albero Balistreri, Claudia Fabiani, Pietro Rubegni, Bruno Frediani, Roberto Giacomelli, Luca Cantarini, Antonio Vitale, International AIDA (AutoInflammatory Diseases Alliance) Network and the Autoinflammatory Diseases Working Group of the Italian Society of Rheumatology (SIR), Jessica Sbalchiero, Valeria Caggiano, Piero Ruscitti, Ilenia Di Cola, Paolo Sfriso, Lorenzo Dagna, Corrado Campochiaro, Francesco Ciccia, Dianela Iacono, Flavia Riccio, Annamaria Iagnocco, Florenzo Iannone, Giuseppe Lopalco, Marcello Govoni, Carla Gaggiano, Elena Bartoloni, Giacomo Emmi, Ombretta Viapiana, Francesca Crisafulli, Lidia La Barbera, Carla Guggino, Claudia Fabiani, Bruno Frediani, Roberto Giacomelli, Luca Cantarini, Antonio Vitale
Institutions: University of Siena, Università Cattolica del Sacro Cuore, Azienda Ospedaliera Universitaria Senese, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, University of Bari Aldo Moro, Universidade de São Paulo, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, King Saud University, University of L'Aquila, National and Kapodistrian University of Athens, University of Padua, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, Gazi Hastanesi, Gazi University, Cairo University, New Giza University, Università Campus Bio-Medico, Campus Bio Medico University Hospital, University of Campania "Luigi Vanvitelli", University General Hospital Attikon, Marmara University, Medical University of Lodz, University of Turin, ASL Roma, Agostino Gemelli University Polyclinic, Hôpital Farhat Hached, University of Sousse, University of Ferrara, Arcispedale Sant'Anna, University of Palermo, Sapienza University of Rome, Ospedale Pediatrico Giovanni XXIII, Hacettepe University, Evangelismos Hospital, Ospedale Garibaldi, University of Rome Tor Vergata, Policlinico Tor Vergata, University of Perugia, Hospital Clínic de Barcelona, Consorci Institut D'Investigacions Biomediques August Pi I Sunyer, Universitat de Barcelona, University of Trieste, Monash Medical Centre, Ospedale di Cattinara, Monash University, Ankara Yıldırım Beyazıt University, San Salvatore Hospital, University of Naples Federico II, Azienda Ospedaliera Universitaria Policlinico "G. Martino", University of Antwerp, Antwerp University Hospital, Wojskowy Instytut Medyczny, University of Verona, Azienda Ospedaliera Universitaria Integrata Verona, University of Milan, IRCCS Ospedale Galeazzi - Sant'Ambrogio, Mansoura University, Horus University – Egypt, Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia, University of Brescia, University of Florence, Luigi Sacco Hospital, University of Pavia, Policlinico San Matteo Fondazione, University of Chieti-Pescara, Azienda Ospedaliera Universitaria Policlinico "Paolo Giaccone" di Palermo
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,597
Funder: Università degli Studi di Siena
OpenAlex ID: W4414667003