Hidradenitis suppurativa (HS) is an autoinflammatory skin disorder of the terminal hair follicle, which can present in sporadic, familial, or syndromic form. A classification has been proposed for the latter, distinguishing cases associated with a known genetic condition, with follicular keratinization disorders or with autoinflammatory diseases. This review focuses on the clinical and genetic features of those entities (ie, pyoderma gangrenosum [PG], acne and HS; PG, acne, pyogenic arthritis and HS; psoriatic arthritis, PG, acne and HS; synovitis, acne, pustulosis, hyperostosis, osteitis; and so forth) for which the collective term HS-related autoinflammatory syndromes is proposed.

BACKGROUND: Planar hyperostotic meningiomas account for 2-9% of intracranial meningiomas. They are characterized by planar node following the contours of the inner surface of the skull. Hyperostosis is present in most cases. Timely diagnosis of skull base tumors is usually simple due to early involvement of the cranial nerves. However, convexity meningiomas en plaque usually reach large dimensions that complicates surgery and radiotherapy.
OBJECTIVE: To analyze the current state of diagnosis, molecular biology and surgical treatment of hyperostotic meningiomas en plaque.
METHODS: A systematic review was performed in accordance with the PRISMA guidelines. Searching for literature data included the following keywords: «planar meningioma», «hyperostotic meningioma», «meningioma en plaque», «infiltrative meningioma». We reviewed the PubMed and Google Scholar databases until May 2023 and enrolled only full-text Russian-, English- or French-language reports.
CONCLUSIONS: Among primary 332 reports, 35 references met the inclusion criteria. We found less severity or absence of focal neurological symptoms, comparable incidence of intracranial hypertension and no histological differences between planar and nodular meningiomas. Analysis of molecular biological features of planar meningiomas, including cell cultures, is feasible. There is no consensus regarding surgical treatment and radiotherapy. Most publications are case reports.
CONCLUSIONS: The results of treatment of planar hyperostotic meningiomas, especially large and giant ones, are unsatisfactory. There is no a generally accepted algorithm for treating patients in the literature. This problem requires further research.
Плоскостные («en plaque») гиперостотические менингиомы составляют от 2 до 9% внутричерепных менингиом. Они характеризуются наличием плоскостного узла, повторяющего контуры внутренней поверхности черепа, и в большинстве случаев формируют гиперостоз. Если при локализации в области основания черепа возможна своевременная диагностика по причине раннего вовлечения черепных нервов, плоскостные менингиомы свода черепа на момент постановки диагноза могут достигать значительных размеров, что затрудняет как хирургическое, так и лучевое лечение.
UNASSIGNED: Анализ современного состояния проблемы диагностики, молекулярной биологии и хирургического лечения плоскостных гиперостотических менингиом свода черепа по данным литературы.
UNASSIGNED: Систематический обзор выполнен в соответствии с рекомендациями PRISMA. Проведен поиск по ключевым словам: «planar meningioma», «hyperostotic meningioma», «meningioma en plaque», «infiltrative meningioma» — в базах PubMed и Google Scholar на всю глубину по май 2023 г., в исследование включены только полнотекстовые публикации на русском, английском или французском языках.
UNASSIGNED: Из 332 выявленных ссылок критериям включения соответствовали 35 источников. Отмечаются меньшая выраженность или отсутствие очаговых неврологических симптомов, сопоставимая частота внутричерепной гипертензии и отсутствие гистологических различий при плоскостных по сравнению с узловыми менингиомами. Подчеркивается целесообразность изучения молекулярно-биологических особенностей плоскостных менингиом, в том числе в клеточных культурах. Нет консенсуса в отношении тактики хирургического и лучевого лечения. Большинство публикаций является описанием клинических случаев.
UNASSIGNED: Результаты лечения плоскостных гиперостотических менингиом свода черепа, особенно больших и гигантских, являются неудовлетворительными. Общепризнанный алгоритм лечения пациентов в литературе не представлен. Проблема требует дальнейших исследований.

OBJECTIVE: Synovitis acne pustulosis hyperostosis osteitis (SAPHO) is a rare heterogeneous disease of unknown aetiopathology. Externally validated and internationally agreed diagnostic criteria or outcomes and, as a result, prospective randomised controlled trials in SAPHO are absent. Consequently, there is no agreed treatment standard. This study aimed to systematically collate and discuss treatment options in SAPHO.
METHODS: Following \'Preferred Reporting Items for Systematic Reviews and Meta-Analyses\' guidance, a systematic literature search was conducted using PubMed, Scopus and Web of Science databases. Prospective clinical studies and retrospective case collections discussing management and outcomes in SAPHO involving five or more participants were included. Articles not published in English, studies not reporting defined outcomes, and studies solely relying on patient-reported outcomes were excluded.
RESULTS: A total of 28 studies (20 observational, 8 open-label clinical studies) reporting 796 patients of predominantly European ethnicity were included. Reported therapies varied greatly, with many centres using multiple treatments in parallel. Most patients (37.1%) received non-steroidal anti-inflammatory drugs alone or in combination. Bisphosphonates (22.1%), conventional (21.7%) and biological (11.3%) disease-modifying antirheumatic drugs were the next most frequently reported treatments. Reported outcomes varied and delivered mixed results, which complicates comparisons. Bisphosphonates demonstrated the most consistent improvement of osteoarticular symptoms and were associated with transient influenza-like symptoms. Paradoxical skin reactions were reported in patients treated with TNF inhibitors, but no serious adverse events were recorded. Most treatments had limited or mixed effects on cutaneous involvement. A recent study investigating the Janus kinase inhibitor tofacitinib delivered promising results in relation to skin and nail involvement.
CONCLUSIONS: No single currently available treatment option sufficiently addresses all SAPHO-associated symptoms. Variable, sometimes descriptive outcomes and the use of treatment combinations complicate conclusions and treatment recommendations. Randomised clinical trials are necessary to generate reliable evidence.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare autoimmune inflammatory disease characterized by osteoarticular and dermatological manifestations. The most common osteoarticular manifestations involve the anterior chest wall, axial skeleton, and long bones. Cranial bone involvement is less reported in SAPHO syndrome. We herein present three cases of SAPHO syndrome with cranial bone involvement, and review the previous literature on similar manifestations. It was revealed that SAPHO syndrome could lead to cranial bone involvement, which could involve the dura mater, leading to hypertrophic pachymeningitis, but the outcome is usually good. Janus kinase inhibitors may be a potential treatment option.

We describe a 74-year-old male with intractable essential tremor (ET) and hyperostosis calvariae diffusa who was unsuccessfully treated with magnetic resonance-guided focused ultrasound (MRgFUS). A computed tomography performed prior to the procedure demonstrated a skull density ratio (SDR) of 0.37 and tricortical hyperostosis calvariae diffusa. No lesion was evident on post-MRgFUS MRI, and no improvement in the patient\'s hand tremor was noted clinically. We systematically reviewed the literature to understand outcomes for those patients with hyperostosis who have undergone MRgFUS. A comprehensive literature search using the PubMed, Cochrane, and Google Scholar databases identified 3 ET patients with hyperostosis who failed treatment with MRgFUS. Clinical findings, skull characteristics, treatment parameters, and outcomes were summarized, demonstrating different patterns/degrees of bicortical hyperostosis and variable SDRs (i.e., from 0.38 to ≥0.45). Although we have successfully treated patients with bicortical hyperostosis frontalis interna (n = 50), tricortical hyperostosis calvariae diffusa appears to be a contraindication for MRgFUS despite acceptable SDRs.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an umbrella term covering a constellation of bone lesions and skin manifestations, but has rarely been clarified in the clinic. We report a 28-year-old woman who had initial onset of SAPHO syndrome with involvement of the femur, and she experienced a tortuous diagnostic course. We also performed a literature review of SAPHO syndrome cases involving the femur and summarize several empirical conclusions by integrating previous findings with our case. Furthermore, we propose our perspective that ailment of the skin caused by infection of pathogens might be the first hit for triggering or perpetuating the activation of the immune system. As a result, musculoskeletal manifestations are probably the second hit by crosstalk of an autoimmune reaction. The skin manifestations preceding bone lesions can be well explained. Current interventions for SAPHO syndrome remain controversial, but drugs aiming at symptom relief could serve as the first preference for treatment. An accurate diagnosis and appropriate treatment can cure patients in a timely manner. Although the pathogenesis of SAPHO syndrome remains to be determined, physicians and surgeons still need to heighten awareness of this entity to avoid invasive procedures, such as frequent biopsies or nonessential ostectomy.

Child abuse is a major public health problem that can lead to critical consequences for the child and family. However, early identification of abuse may be difficult. An 8-month-old boy presented with extensive periosteal reaction in both upper and lower long bones. There was no specific history of injury. Caffey disease was initially considered as the diagnosis because the patient displayed fever and hyperostosis of multiple bones with elevated erythrocyte sedimentation rates and C-reactive protein and alkaline phosphatase levels. However, we suspected child abuse based on the clinical and radiological features. We eventually found out that the child had been injured through child abuse and were able to treat him. We report this case because child abuse cases may be confused with Caffey disease. This case report can, therefore, help distinguish between Caffey disease and child abuse.

Camurati-Engelmann disease (CED) is a rare, progressive diaphyseal dysplasia characterized as diaphyseal hyperostosis and sclerosis of the long bones. Corticosteroids, bisphosphonates, and losartan have been reported to be effective systemic medications used to reduce CED symptoms. There are no reports of osteoblastoma in patients with CED, and osteoblastoma in the distal radius is rare. We present a patient diagnosed with CED, based on radiological and histological examinations, at 11 years old. At 22 years old, she experienced severe pain in her right forearm and was treated with bisphosphonate, losartan, and prednisolone; however, the pain continued. An expansive and sclerotic lesion at the distal radius was observed on radiography. A follow-up plain radiograph indicated that the lesion was growing. Fluorodeoxyglucose positron emission tomography revealed solitary, intense radiotracer uptake, and a biopsy and surgical resection were performed due to suspected malignancy. Pathologic analysis showed anastomosing bony trabeculae rimmed by osteoblasts observed in a loose fibrovascular stroma. The lesion was diagnosed as an osteoblastoma. Following bone excision and artificial bone grafting, the patient\'s severe pain almost completely disappeared. At final follow-up, no evidence of osteoblastoma recurrence was noted. To our knowledge, this is the first case report of osteoblastoma arising in a patient with CED. Bone excision and artificial bone grafting may be a treatment option for local symptomatic osteoblastoma in patients with CED.

Diffuse idiopathic skeletal hyperostosis (DISH) also known as Forestier disease is a noninflammatory, systemic skeletal disease of unknown etiology. DISH is usually asymptomatic but may compress the posterior wall of the aero digestive tract and lead to dysphagia, globus, hoarseness, stridor, dyspnea, and neurological problems. Although dysphagia is not uncommon among the presenting symptoms of DISH but dysphonia and stridor are rarely reported. We report a 68-year-old man who presented with a history of progressive dysphagia over 1 year and recent dysphonia and stridor secondary to cervical osteophytes. We discuss the symptoms, radiological features, and management of this uncommon case of DISH in conjunction with review of literature.

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