**5. Clinical case**

**2. Etiology**

– Synovitis – Acne

– Hyperostosis – Osteitis

– Palmoplantar pustulosis

patient has not been proven.

**Table 1.** Types of SAPHO syndrome.

ally affects to the female gender.

reports in the medical literature).

– Arthro-osteitis with palmoplantar pustulosis – Manubrium-sternal arthritis and pustular psoriasis – Symmetric multifocal chronic osteomyelitis

– Chronic sclerosing osteitis

– Pustular palmoplantar arthritis – Hyperostosis of the sternal manubrium – Recurrent hyperostosis of the mandible – Bone lesions in palmoplantar pustulosis – Sternocostoclavicular hyperostosis – Sternocostoclavicular arthro-osteitis

**Table 2.** Synonyms of the SAPHO syndrome.

– Pseudoseptic acute arthritis and palmoplantar pustulosis

– Chronic multifocal osteomyelitis of unknown etiology – Skeletal muscle syndromes associated with acne

– Bilateral clavicular osteomyelitis with palmar and plantar pustulosis

**3. Epidemiology**

The etiology of SAPHO syndrome is unclear. It is thought that it may have a multifactorial origin where genetic, environmental, immunological [1], and infectious causes intervene. Some bacteria, such as the *Bacillus Propionibacterium acnes*, could act as a triggering factor [2]. However, the possible pathogenic role of this or other germs in a genetically predisposed

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

Its prevalence is unknown, and it can be described in different ways (**Table 2**). A few cases have been reported in Spain to date. It usually occurs in childhood and adolescence and usu-

A patient with SAPHO syndrome who had a good response to oral alendronate has been described, although this is not the first choice recommended for treatment (there are a few

The case of a 45-year-old woman who came for consultation due to costal pain and in the right renal fossa is presented. The pain was continuous, did not give in to rest, and did not increase with exercise; it was not related to any trigger, and she had it for 3–4 months. Any micturition syndrome or fever was not reported. She had not had any weight loss. The patient was a smoker [11] and had a history of hypercholesterolemia, renal lithiasis, osteoporosis in treatment with calcium/vitamin D, and palmoplantar psoriasis on treatment with acitretin.

On the physical examination, pustule-erythematous, confluent lesions were found in the palms of both hands and on both soles of the feet. In addition, although she described it as costal, she presented localized pain in the sternoclavicular and chondrosternal region that increased to acupressure. She also had pain in the right renal fossa with a positive percussion fist.

Before these findings, various tests such as hemogram, biochemistry, coagulation, systematic urine, and chest X-ray were requested, with normal results. SVS, autoimmunity, and HLA-B27 were negative. Chest and abdomen CT were requested with the following report: probable areas of atelectasis/fibrous tracts in both lung bases; small bilateral renal cortical cysts; nonobstructive right renal lithiasis; arteriosclerosis of the aortoiliac axis; apparent increase of density of the subcutaneous at the level of the interlabial cleft, to be assessed in the clinical context of the patient; and degenerative alterations in the axial skeleton.

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 compatible with osteopenia.

**6. Clinic**

alterations (**Table 3**).

1. Chronic multifocal relapsing osteomyelitis

2. Acute/subacute/chronic arthritis in addition to:

3. Sterile osteitis in any localization in addition to

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

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

 With/without coccyx affection With/without skin affection

 Palmoplantar pustulosis Pustular psoriasis Severe acne

 Palmoplantar pustulosis Pustular psoriasis Vulgar psoriasis Severe acne

Generally sterile

SAPHO syndrome is an entity that associates musculoskeletal disorders with dermatological

SAPHO Syndrome

111

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

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.

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 the joint (**Figure 1**).

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

At 6 weeks, the patient was reevaluated and presented an almost total decrease in sternoclavicular pain and a marked improvement in the palmoplantar lesions.

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