SAPHO SYNDROME

TYPES 

Synovitis is often referred to as inflammation of the sheet covering the joints to cushion them from deep impact, thus preventing them from crush injuries.

Acne is the superficial skin manifestation that appears either as a white or blackheads, pimples, and other skin impurities brought about by an excessive buildup of germs on the outlet of the pore.

Pustulosis refers to the formation of a large indurated area that is filled with pus (whitish to yellowish inflammatory fluid collection), commonly found among female patients. Some manifest with exfoliative dermatitis (skin peels off from the dermis or the second layer of the skin), causing redness or weeping appearance.

Hyperostosis and Osteitis refer to the excessive accumulation of materials that comprises the bone, and osteitis is the inflammation of these.

SYMPTOMS

SAPHO syndrome involves mainly the joints, which are essential for moving around. One of the following symptoms can be seen in the patients:

• Pain especially on the area involved
• Limitation of motion ( flexion and extension of the affected extremity)
• The difference in the size of the affected area (maybe more significant than the healthy side)
• Warm skin when touched
• Presence of lesions on the palmar and plantar surface of the hands and the feet
• Difficulty in walking around and carrying heavy loads
• Presence of pimples and blackheads anywhere in the body

DIAGNOSIS

SAPHO syndrome is suspected in a patient present with arthritis coupled with dermal findings.  In 1997, Kahn developed criteria for the diagnosis of SAPHO syndrome that includes:

• Presence of inflammation of the bones in more than two areas
• Recurrent swelling of the joints with or without  skin manifestations (acne)
• Inflammation of the bony components of the body with either psoriasis (an autoimmune disorder that presents as silvery scales over the affected areas of the body) or acne.

Recent researches, however, have proven that detection of HLA B27 in the blood that supports the diagnosis of the syndrome.

TREATMENT

Symptomatic treatment is used in SAPHO syndrome, depending on their manifestations.

• To facilitate a reduction of the inflammation, the patient is given a non steroidal anti-inflammatory drug such as Ibuprofen, naproxen, and aspirin or a cyclo oxygenase drug such as Celecoxib and etoricoxib
• Use of steroids- This drug type shows a significant reduction in the inflammation. It can either be in the form of cream, intravenous drugs or per orem
• Disease-modifying antirheumatic drugs(DMARD’s) in the form of sulfasalazine and methotrexate can help in improving the mobility of the affected patient.
• New biologicals that include infliximab shows promise in the treatment of the manifestations of the syndrome