**6. Clinic**

A bone densitometry was performed, showing a T-score of −2.6 in the lumbar spine (L1–L4), compatible with osteoporosis. There was also a study in her neck, trochanter and total femur

Before the clinical suspicion, a bone scan [12] was requested. It showed a pathological focus on the right sternoclavicular joint, compatible with SAPHO syndrome, with hyperostosis of

Treatment was established with colchicine 0.5 mg 1/24 h. Methotrexate 15 mg IM/week (the Mantoux-Booster test was previously requested and serologies for HBV, HCV, and HIV that were negative): folic acid one tablet 1 day after the administration of methotrexate and alendronate one tablet/week. Treatment with oral bisphosphonates was decided [13] despite the scarce records in the literature (agreed with the patient who did not want intravenous treatment and opted for oral treatment). This dose and frequency of administration were decided

At 6 weeks, the patient was reevaluated and presented an almost total decrease in sternocla-

**Figure 1.** Hyperostosis on the right sternoclavicular joint, compatible with SAPHO syndrome, can be seen.

since it is the one used in the treatment of postmenopausal osteoporosis.

vicular pain and a marked improvement in the palmoplantar lesions.

110 Anatomy, Posture, Prevalence, Pain, Treatment and Interventions of Musculoskeletal Disorders

compatible with osteopenia.

the joint (**Figure 1**).

SAPHO syndrome is an entity that associates musculoskeletal disorders with dermatological alterations (**Table 3**).

The most characteristic clinical manifestation of the SAPHO syndrome is pain in the anterior chest wall, due to the involvement of the sternoclavicular and costochondral joints. Less commonly, the sacroiliac, intervertebral, or peripheral joints are affected. It could also affect the jaw. It is usually presented symmetrically, bilaterally, and in outbreaks. In adults, disease predominates in the sternocostoclavicular region (65–90% of patients). All the components of the anterior chest wall might be affected. The second affected region is the spine (33% of cases), mostly at the dorsal level. Nonspecific spondylitis, osteosclerosis of one or more vertebral bodies, and paravertebral ossifications could be observed. Ninety-two percent of patients have arthritis with involvement their knees, hips, ankles, feet, and hands.


**Table 3.** Diagnostic criteria: one of the three presentations is enough for diagnosis.

**Figure 2.** Inflammatory pustules with erythema that affect palms of hands.

symmetric, and sterile, with erythema and desquamation, which affect palms of the hands (**Figure 2**) and soles of the feet (**Figure 3**). The pathogenesis of the disease remains unknown, and there is still a debate about whether palmoplantar pustulosis is a variant of psoriasis or

SAPHO Syndrome

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http://dx.doi.org/10.5772/intechopen.75351

The SAPHO syndrome could present several clinical manifestations and therefore originate different differential diagnoses (**Table 4**). This is very important in neoplastic disease context, since it is necessary to avoid errors in the diagnosis that lead to aggressive treatment in a dis-

The diagnosis is basically clinical and is complemented by imaging tests. Due to its low prevalence, it is necessary to have a high index of suspicion. The analytical data are usually nonspecific but have value in excluding other pathologies. Mild leukocytosis, anemia, or an increase in inflammatory markers might occur. A simple radiography, bone scan with technetium 99 m, and CT are useful. Simple X-ray is of little use since it is usually normal. Bone scintigraphy provides much information, and the pattern of symmetrical sternoclavicular hypercaptation in "bull's head" (where the sternal manubrium represents the skull and the sternoclavicular joints and the clavicles correspond to the horns) is very typical. CT provides us with a lot of information as it is the technique that best visualizes the joints of the anterior

Biopsy and cultures of the affected joints are reserved for doubtful cases and to rule out other diagnostic possibilities. Imaging allows differential diagnosis with other processes (osteomyelitis, Paget's disease, bone metastases, Tietze syndrome, other spondyloarthropathies).

In some patients, cutaneous manifestations might appear after years of nonspecific joint

SAPHO syndrome is a chronic disease that develops in form of outbreaks and remissions but in which radiological progression is slow [14]. Prognosis is usually good, but there are cases in which the pain that is produced is very intense and difficult to control it. Sometimes the onset of the disease could be acute and crippling. It has been observed that those factors such as female gender, anterior chest wall involvement, peripheral arthritis, skin lesions and elevation of acute phase reactants at onset of disease are related to the chronicity of the disease.

In a minority of patients, disease heals spontaneously or follows a chronically indolent course. Complications are rare. Peripheral arthritis could become erosive in a minority of cases. Venous thrombosis could be observed due to an important disseminated inflammation from bones or joints to the adjacent tissue (especially as a result of clavicular hypertrophy), inflammatory masses in the anterior mediastinum, thoracic gorge syndrome that the swelling is

a distinct condition.

**7. Diagnosis**

**8. Evolution**

ease that it usually develops benign.

thoracic wall, showing a hyperostosis of this zone.

symptoms, making diagnosis even more difficult.

**Figure 3.** Pustular lesions, symmetric, with desquamation that affects the soles of the feet.


**Table 4.** Differential diagnosis of SAPHO syndrome.

In children it usually affects to the long bones such as the tibia and femur, clavicle, and lumbar spine.

Skin involvement is more variable and includes palmoplantar pustulosis, acne conglobata or fulminating, suppurative hidradenitis, dissecting cellulitis, or pustular psoriasis. The most predominant is palmoplantar pustulosis, which is characterized by inflammatory pustules, symmetric, and sterile, with erythema and desquamation, which affect palms of the hands (**Figure 2**) and soles of the feet (**Figure 3**). The pathogenesis of the disease remains unknown, and there is still a debate about whether palmoplantar pustulosis is a variant of psoriasis or a distinct condition.

The SAPHO syndrome could present several clinical manifestations and therefore originate different differential diagnoses (**Table 4**). This is very important in neoplastic disease context, since it is necessary to avoid errors in the diagnosis that lead to aggressive treatment in a disease that it usually develops benign.
