Acquired Hyperostosis Syndrome/surgery* ; Humans ; Tonsillectomy

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.
Acitretin ; Acne Vulgaris ; Acquired Hyperostosis Syndrome ; Female ; Hand ; Humans ; Hyperostosis ; Low Back Pain ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Metastasis ; Osteitis ; Osteosclerosis ; Pathology ; Physical Examination ; Positron-Emission Tomography ; Ribs ; Spine ; Thoracic Wall

SAPHO syndrome, characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis is rare compared to other spondyloarthropathies. It is also difficult to diagnose, and treatment methods have not yet been fully identified. Approximately 72% of patients are diagnosed with at least one other disease before a final diagnosis of SAPHO syndrome. In addition, SAPHO syndrome is subject to a delayed diagnosis period of 4.5 to 9.1 years. Medications such as non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, and tumor necrosis factor inhibitors are used in treatment of SAPHO syndrome. Bisphosphonate is also used for refractory SAPHO syndrome; however, most reports on this relate to intravenous injection of medication. The authors experienced and subsequently reported on a case involving a patient with SAPHO syndrome accompanied by fracture and infection of the left second finger who was treated with the oral biphosphonate, alendronate.
Acne Vulgaris ; Acquired Hyperostosis Syndrome* ; Alendronate* ; Antirheumatic Agents ; Delayed Diagnosis ; Diagnosis ; Fingers ; Humans ; Hyperostosis ; Injections, Intravenous ; Osteitis ; Spondylarthropathies ; Synovitis ; Tumor Necrosis Factor-alpha

SAPHO syndrome is a rare inflammatory, pseudoinfectious disease. Initially it was an acronym for Syndrome Acne Pustulosis Hyperostosis Osteitis, and the meaning of S was later changed to synovitis. It occurs predominantly in children and adults and is not common over 60 years. The most common clinical presentation is osteoarticular involvement at the anterior chest wall and skin manifestations may be evident, but it could occur years earlier or develop later. We report on two cases of mother and daughter. A 51-year-old female was diagnosed with SAPHO syndrome with costochondritis and palmoplantar pustulosis. Five years later, her 31-year-old daughter presented with similar skin manifestations of the hand and foot.
Acne Vulgaris ; Acquired Hyperostosis Syndrome* ; Adult ; Child ; Female ; Foot ; Hand ; Humans ; Hyperostosis ; Middle Aged ; Mothers ; Nuclear Family* ; Osteitis ; Psoriasis ; Skin Manifestations ; Synovitis ; Thoracic Wall

Acquired Hyperostosis Syndrome ; therapy ; Combined Modality Therapy ; Humans ; Integrative Medicine ; Male ; Middle Aged

A 66-year-old woman was transferred to our hospital due to her right breast cancer. Preoperative breast MRI shows 1.9 cm malignancy on her right breast (cT1N0M0) and incidentally found osteosclerotic change of left coststernoclavicular region. Bone scintigraphy showed hot uptake and the possibility of bone metastasis was not excluded. However, because the bone metastasis is not common in early stage cancer and the costosternoclavicular region is not common site, other possibility should be considered. SAPHO syndrome can be diagnosed even in the absence of dermatosis when there is an axial or appendicular osteitis and hyperostosis, especially in costosternoclavicular region. Though breast imaging specialists are not accustomed to this disease entity, awareness and diagnosis of the SAPHO syndrome can help differentiate bone metastasis.
Acquired Hyperostosis Syndrome* ; Aged ; Breast Neoplasms* ; Breast* ; Diagnosis ; Female ; Humans ; Hyperostosis ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Osteitis ; Radionuclide Imaging ; Skin Diseases ; Specialization

Synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome is a rare disease that involves the skin, bones and joints. It is thought to be caused by infection with low-toxicity bacteria and to be the result of reactive infectious osteitis. However, this hypothesis has not yet been clearly established. New SAPHO syndrome treatment methods are needed because the disease does not respond to treatment in many cases. In this paper, a case is reported of SAPHO syndrome with pain in the acromioclavicular joint and with squamous and pustular macules on the palms and soles. First, the patient was treated with aceclofenac, prednisolon and sulfasalazine for two weeks. However, the symptoms were not relieved, so methotrexate and pamidronate were added to the treatment. Since no improvement was seen after four weeks of treatment, adalimumab was prescribed. The skin lesions were relieved two weeks later, and the bone pain and arthralgia, four weeks later. No recurrence or adverse effects were observed at the 22-week follow-up.
Acne Vulgaris ; Acquired Hyperostosis Syndrome ; Acromioclavicular Joint ; Antibodies, Monoclonal, Humanized ; Arthralgia ; Bacteria ; Diclofenac ; Diphosphonates ; Follow-Up Studies ; Humans ; Hyperostosis ; Joints ; Methotrexate ; Osteitis ; Rare Diseases ; Recurrence ; Skin ; Sulfasalazine ; Synovitis ; Adalimumab

Psoriatic arthritis (PsA) is an autoimmune arthritis related to psoriasis and one of seronegative spondyloarthropathies. PsA provokes joint pain and morning stiffness more than 30 minutes, which is relieved by exercise. PsA usually affects distal small joints and exhibits asymmetry, which is one of the typical characteristics of PsA and gives clues to make a differential diagnosis between PsA and rheumatoid arthritis. Thirty to forty patients with PsA experience arthritis in one large joint or asymmetric multiple joints. Arthritis in distal joints and arthritis mutilans often develop concurrently and patterns of PsA change along with disease progression. Spondylitis is observed in 20-30% of PsA patients. In contrast to ankylosing spondylitis, spondylitis in PsA present with mild clinical symptoms despite radiological progression, inflammation limited to one spinal tract, cervical spine dominance, non-marginal syndesmophytosis. Enthesitis is also one of the typical characteristics of PsA and it frequently affects Achilles tendon, plantar fascia and tendons inserting pelvic bones. Tenosynovitis can develop accompanied by enthesitis. Typical dactylitis (sausage digit), pitting edema and nail lesions, including nail pits, onycholysis, hyperkeratosis and splinter hemorrhage, also contribute to a differential diagnosis of PsA. Anterior uveitis, SAPHO syndrome, amyloidosis and IgA nephropathy are well-known extra-articular manifestation of PsA. In 2006, a new classification-criterion for PsA was suggested by the CASPAR study. The CASPAR criteria included 5 categories with a certain number of points; 1) skin psoriasis, 2) nail lesions, 3) dactylitis, 4) negative RF and 5) bone formation around joints. The CASPAR criteria should be applied to PsA patients having at least one of three (peripheral arthritis, spondylitis and enthesitis).
Achilles Tendon ; Acquired Hyperostosis Syndrome ; Amyloidosis ; Arthralgia ; Arthritis ; Arthritis, Psoriatic ; Arthritis, Rheumatoid ; Diagnosis, Differential ; Disease Progression ; Edema ; Fascia ; Glomerulonephritis, IGA ; Hemorrhage ; Humans ; Inflammation ; Joints ; Nails ; Onycholysis ; Osteogenesis ; Pelvic Bones ; Psoriasis ; Skin ; Spine ; Spondylarthropathies ; Spondylitis ; Spondylitis, Ankylosing ; Tendons ; Tenosynovitis ; Uveitis, Anterior

SAPHO syndrome, which has different skin changes and osteoarticular inflammation, is an acronym that stands for synovitis, acne, pustulosis, hyperostosis, and osteitis. Treatment of SAPHO syndrome includes non-steroidal anti-inflammatory drugs (NSAIDs), anti-rheumatic drugs, such as colchicines, corticosteroids and bisphosphonates, and disease-modifying agents. However, the treatment of SAPHO syndrome is controversial because it is a new clinical entity with unclear etiopathogenesis and inadequate clinical studies. We report a case with refractory SAPHO syndrome, which was successfully treated with a tumor necrosis factor (TNF)-alpha blocker.
Acne Vulgaris ; Acquired Hyperostosis Syndrome ; Adrenal Cortex Hormones ; Antirheumatic Agents ; Diphosphonates ; Hyperostosis ; Immunoglobulin G ; Inflammation ; Osteitis ; Receptors, Tumor Necrosis Factor ; Skin ; Synovitis ; Tumor Necrosis Factor-alpha ; Etanercept

Acquired Hyperostosis Syndrome ; drug therapy ; Adult ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Phytotherapy