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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Which diagnosis is most appropriate?Chronic lymphocytic leukaemiaLöffler syndromeLöfgren syndromeSapho syndromeSever disease
Musculoskeletal Side Effects
Published in Ayse Serap Karadag, Berna Aksoy, Lawrence Charles Parish, Retinoids in Dermatology, 2019
Filiz Cebeci Kahraman, Vefa Aslı Turgut Erdemir, Melek Aslan Kayıran
Patients with acne fulminans may also have sacroiliitis as part of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome; however, these patients may also have sacroiliitis related to isotretinoin (67,85). As a result, patients with SAPHO syndrome should be followed for the development of a possible sacroiliitis during systemic isotretinoin treatment.
Role of Bacteria in Dermatological Infections
Published in K. Balamurugan, U. Prithika, Pocket Guide to Bacterial Infections, 2019
Thirukannamangai Krishnan Swetha, Shunmugiah Karutha Pandian
P. acnes also exhibits infectious role by causing inflammatory acne vulgaris majorly and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome rarely. The free fatty acids produced by P. acnes as the result of triglyceride metabolism trigger the inflammatory response. Acne vulgaris is ailment of pilosebaceous follicles caused as result of hormonal imbalance, immune hypersensitivity, P. acnes infection, and follicular keratinization. It is frequently witnessed in 80% youngsters of the U.S. population. Several putative predisposal factors such as genetic factors, stress, androgens, follicular pattern of the individual, and use of steroids are known to influence the onset of acne vulgaris.
Anti-IL17 treatment in childhood chronic rheumatic diseases
Published in Expert Opinion on Biological Therapy, 2023
Valerio Maniscalco, Ilaria Maccora, Flavio Girodo, Marta Tomaselli, Gaia Priolo, Edoardo Marrani, Maria Vincenza Mastrolia, Ilaria Pagnini, Gabriele Simonini
The IL-17 pathway is involved in the pathogenesis of several chronic rheumatologic disorders, and its blockade through IL17i is becoming a central therapeutic option for an increasing number of diseases. Secukinumab is currently approved for ax-SpA, PsA, ERA, and JPsA. Moreover, in a small series of adult patients with BS and SAPHO syndrome, it has been shown to be effective in achieving response in difficult-to-treat cases. However, the same efficacy has not been proven in treating NIU. Ixekizumab is approved for ax-SpA and PsA and is currently under study for ERA and JPsA in a phase three RCT. Additionally, both secukinumab and ixekizumab showed good efficacy in a novel autoinflammatory disease called DITRA even when anti-IL1 drugs failed to maintain disease control. Other agents antagonizing the IL-17 pathway have been proven efficacy in the adult population of AxSpA and PsA, namely netakimab, brodalumab, and bimekizumab. Overall, IL17i have shown an overall favorable safety profile and most AEs were transient and did not necessitate drug discontinuation.
Chronic Recurrent Multifocal Osteomyelitis (CRMO): A Study of 12 Cases from One Institution and Literature Review
Published in Fetal and Pediatric Pathology, 2022
Eric Chang, Jasmine Vickery, Nadeen Zaiat, Eman Sallam, Abdul Hanan, Scott Baker, Mohamed Alhamar, Janet Poulik, Ereny Demian, Bahig M Shehata
Overall, CRMO is considered a disease spectrum with a wide clinical presentation ranging from mild to severe, time-limited to chronically active, and remitting to unremitting course [8]. It is important to note that a similar disease process may occur in adult population, where patients have sterile bone lesions. When it occurs in the adult population, it presents as synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO), and hence it is called SAPHO syndrome [9]. This adult onset disease has a predilection for the axial skeleton as compared to the extremities in CRMO [7]. Adult patients have joint and skin inflammation which is seen infrequently in children [8]. Although cutaneous disease, especially acne, may occur in children with CRMO, SAPHO is associated with more prominent cutaneous manifestations including palmoplantar and non-palmoplantar pustulosis, psoriasis vulgaris, acne, pyoderma gangrenosum, and sweets syndrome [7]. Patients diagnosed with SAPHO often have more frequent and long-lasting disease manifestations [7]. Our patient cohort exhibited none of these cutaneous and joint symptoms, failing to fulfill the diagnosis of SAPHO syndrome.
Diagnostic challenge of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome in pediatric age: A monocentric case series
Published in Modern Rheumatology, 2021
Ilaria Maccora, Edoardo Marrani, Valerio Maniscalco, Maria Vincenza Mastrolia, Ilaria Pagnini, Gabriele Simonini
SAPHO syndrome is a rare disease that typically begins in the third-fifth decade of life, and is characterized by osteoarticular and dermatologic features [1]. The acronym summarizes the key clinical features: synovitis, acne, palmoplantar pustulosis, hyperostosis and osteitis [2]. The prevalence of this condition is estimated to be less than 1 per 10,000 people [3]. Several cases have been described in childhood, mostly in adolescents, some associated with other comorbidities. Indeed, the SAPHO clinical spectrum encompasses a wide range of different manifestations which overlap with other autoimmune or autoinflammatory disorders, thus often challenging the diagnosis, particularly in paediatric age [4–6]. If promptly recognized and adequately treated, SAPHO syndrome shows a favourable course. Herein we present a case series of 5 children affected by SAPHO syndrome, diagnosed at our Rheumatology Unit from March 2015 to June 2020, experiencing an excellent response to anti-TNFα.