**8. Evolution**

In children it usually affects to the long bones such as the tibia and femur, clavicle, and lum-

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

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

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,

bar spine.

– Acne-associated syndrome

– Follicular occlusion triad

– Isotretinoin side effect – Pustular psoriasis

– Majeed syndrome – Nonbacterial osteitis – Tuberculous spondylitis – Secondary syphilis – Primary bone tumors – Metastatic tumors

– Sneddon-Wilkinson disease – Pustulotic arthro-osteitis – Acquired hyperostosis syndrome

– Arthritis associated with hidradenitis suppurativa

– Minocycline-induced autoimmune syndromes

– Chronic recurrent multifocal osteomyelitis – Diffuse sclerosing osteomyelitis of the mandible

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

– PAPA syndrome – PASH syndrome

– Behçet disease

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 confused with a tumor (signs mainly observed at clavicular level), etc. Recurrent chronic multifocal osteomyelitis could cause permanent bone deformities, irregularities in limb length, and growth problems.

the disease was found. This is due to an action on bone remodeling that interferes with the

SAPHO Syndrome

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

Zoledronic acid is the most potent bisphosphonate and is usually used in hypercalcemias of tumoral origin. It has been used at a dose of 4 mg IV in a single dose with repetition at 6 months if there was no improvement [20], obtaining good results (decrease in pain and

In the treatment with biological therapy, anti-tumor necrosis factor (TNF) drugs have been

Although NSAIDs usually control pain and inflammation, it is often necessary to use diseasemodifying drugs to improve symptoms. In this regard, it seems that IV bisphosphonates have

Biological therapy also seems to show effectiveness [21]. More trials with these drugs would be needed to test their effectiveness against this disease and protocol the number of doses, the

[1] Rohekar G, Inman RD. Conundrums in nosology: Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome and spondylarthritis. Arthritis and Rheumatism. 2006;**55**:

[2] Assmann G, Kueck O, Kirchhoff T, Rosenthal H, Voswinkel J, Pfreundschuh M, et al. Efficacy of antibiotic therapy for SAPHO syndrome is lost after its discontinuation:

[3] Chamot AM, Benhamou CL, Kahn MF, Beraneck L, Kaplan G, Prost A. Acne-pustulosishyperostosis-osteitis syndrome. Results of national survey. 85 cases. Revue du Rhu-

[4] Kahn MF, Khan MA. The SAPHO syndrome. Baillière's Clinical Rheumatology. 1994;**8**:

Interventional study. Arthritis Research & Therapy. 2009;**11**:R140

matisme et des Maladies Ostéo-Articulaires. 1987;**54**:187-196

and Sara Alcántara Luna3

been the most effective, but the dose and interval have not been unified.

\*, Luis Geniz Rubio<sup>2</sup>

\*Address all correspondence to: jald99@hotmail.com

production of proinflammatory cytokines.

amount, and the interval between them.

1 UGC Molino de la Vega, Huelva, Spain

3 CPE Virgen de la Cinta, Huelva, Spain

2 UGC Adoratrices, Huelva, Spain

regression of disease).

**Author details**

**References**

665-669

333-362

José Ángel López Díaz<sup>1</sup>

used with promising results.
