An integrated transcriptomics and network pharmacology approach to explore the mechanism of Wang-Bi tablet against SAPHO syndrome.

BACKGROUND: SAPHO syndrome is recognized as a rare entity with damage to skin and bones due to inflammation. Currently, the treatment for SAPHO syndrome is still a challenge in clinical practice. In this study, an integrated transcriptomics and network pharmacology approach was applied to explore the therapeutic effect and mechanism of Wang-Bi tablet (WBT) on SAPHO syndrome. METHODS: The main components of WBT and their targets, as well as the targets of SAPHO syndrome, were collected from databases. Network visualization was performed using Cytoscape software. The GO and KEGG enrichment analysis was executed by David dataset. Then, the molecular mechanism of WBT improving SAPHO syndrome was validated by transcriptomics of peripheral blood neutrophils in SAPHO syndrome. Finally, the above results were validated by molecular docking. RESULTS: The Network Pharmacology results showed there are 152 core targets for WBT treatment on SAPHO syndrome. RNA-seq data showed 442 differentially expressed genes (DEGs) in peripheral blood neutrophils of SAPHO patients. Intriguingly, NIK/NF-kappaB-, MyD88-dependent toll-like receptor-, and MAPK pathway were included in the enrichment results of network pharmacology and RNA-seq. Moreover, we verified that the core components of WBT have good affinity with the core targets of NIK/NF-kappaB-, MyD88-dependent toll-like receptor-, and MAPK pathway by molecular docking. CONCLUSIONS: This study illustrated that the possible mechanisms of WBT against SAPHO syndrome may be related to NIK/NF-kappaB-, MyD88-dependent toll-like receptor-, and MAPK pathway, and further experiments are needed to prove these predictions.

Biologic Therapy for Hidradenitis Suppurativa Plus Conglobate Acne Associated with SAPHO Syndrome: A Case Report.

is missing (Short Communication).

Acne fulminans.

Acne fulminans (AF) is a rare and severe form of inflammatory acne presenting clinically with an abrupt outburst of painful, hemorrhagic pustules and ulceration, that may or may not be associated with systemic symptoms, such as fever, polyarthritis, and laboratory abnormalities. It typically affects male teenagers with a pre-existing acne. Although the pathogenetic mechanism has not been established yet, a role of genetic, abnormal immunologic response, drugs intake, hormonal imbalance and viral infection, as causal factors, has been identified. AF may occur as a single disease or may be associated with other disorders. Traditionally, AF has been classified, on the basis of the presence of systemic involvement, in "acne fulminans" and acne fulminans "sine fulminans," when no systemic involvement is present. Recently, four clinical variants have been proposed: acne fulminans with systemic symptoms (AF-SS), acne fulminans without systemic symptoms (AF-WOSS), isotretinoin-induced acne fulminans with systemic symptoms (IIAF-SS), isotretinoin-induced acne fulminans without systemic symptoms (IIAF-WOSS). The diagnosis of AF is usually based on clinical history and physical examination. No specific laboratory abnormalities are generally found. In selected cases, biopsy and/or radiologic imaging are helpful for a correct diagnosis. The treatment significantly differs from severe acne according to severity of clinical presentation and possible systemic involvement. Currently, systemic corticosteroids (prednisolone) and retinoids (isotretinoin) represent the first choice of treatment. Dapsone, cyclosporine A, methotrexate, azathioprine, levamisole, and biological agents such as anakinra, infliximab, adalimumab may be considered as alternative therapies in selected cases. Adjunctive topical and physical therapies may also be considered.

Síndrome SAPHO: a propósito de un caso / No disponible

No disponible

Is primary chronic osteomyelitis a uniform disease? Proposal of a classification based on a retrospective analysis of patients treated in the past 30 years.

INTRODUCTION: Primary chronic osteomyelitis of the jaw is a rare, non-suppurative, chronic inflammatory disease of unknown aetiology. To date, classification is confusing due to a non-uniform terminology. The aim of this study was to establish a simple (clinical) classification based on patient data from our clinic. METHODS: Retrospective analysis revealed 30 cases of which clinical course, radiology, pathology, therapy and outcome were analysed. RESULTS: Both sexes were equally represented. The mean age at onset of disease was 35 years (range 5-76 years). Onset of disease revealed two peaks of incidence, one in adolescence and one after age 50 years. While clinical symptoms were similar in all cases, an increased intensity of these symptoms was noted in younger individuals as well as in the early stages of the disease. Five adults and one adolescent presented with additional non facial bone, joint and skin manifestations consistent with the diagnosis of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome, or chronic recurrent multifocal osteomyelitis. Radiology demonstrated sclerosis, osteolysis and periosteal reaction in variable stages in all cases. However, findings were more extensive in younger patients. Histology revealed different stages of chronic inflammation in all cases. Microabscess formation was noted in 11 cases, six of which were children/adolescents. Therapy consisted mainly of surgery, antibiotics and hyperbaric oxygen therapy. At the end of the follow up period, 11 patients demonstrated complete remission, while in 14 cases amelioration and in 5 no significant improvement was noted. CONCLUSION: Based on differences in age at presentation, clinical appearance and course, radiology and histology, a subclassification into early and adult onset primary chronic osteomyelitis has been established. Cases with purely mandibular involvement should further be distinguished from cases associated with other syndromes.