Poster: SAPHO Syndrome: A « Case Report » (For Professionals)

Poster: SAPHO Syndrome: A « Case Report » (For Professionals)

• SAPHO syndrome is a rare clinical entity standing for:

1. S: synovitis
2. A: acne
3. P: pustulosis
4. H: hyperostosis
5. O: osteitis

• It was described by KAHN in 1994 and the following criteria are each sufficient for the diagnosis:

1. chronic multifocal ostéomyelitis with or without skin lesions.
2. sterile acute or chronic joint inflammation associated with either pustular psoriasis, palmo-plantar pustulosis, acne or hidradentitis.
3. sterile osteitis associated with on of the forementioned skin manifestations.

The authors describe the case of a 13 year old girl.

–clinically, she presented for two weeks with lower back pain (reduced mobility), anterior chest wall tenderness (painful on palpation) and low grade fever. Additionally, she had very severe acne.

-diagnosis was confirmed:

by laboratory tests:

• inflammation:

1. leucocytosis with neutrophilia, elevated CRP (C reactive protein) and sedimentation rate

• radiology:

1. radiographs normal
2. increased uptake on bone scintigraphy of L3 vertebra, sternum, sternoclavicular  joints and right mandible.
3. MRI showed abnormal signal at L1, L3, and the corresponding postvertebral soft tissue elements

• histology:

1. Bone marrow negative for malignancy
2. Osteitis

Empirical treatment of methylprednisone (additionally to isotretinoin and clindamycin for the acne) was introduced for 16 weeks, with back pain upon tapering of the steroid and improvement of inflammation but persistance of an abnormal MRI. Methotrexate was introduced for 17 months with full response and the patient remains in remission five years later.

Many dermatologists question the existence of this syndrome, one of the arguments beaing that acne and pustolosis are the same thing and that they can’t be present together. However, we believe dermatologists should all have this entity in mind because it is one of the few where the skin acts as the mirror to an osteoarticular problem.

Contributors:

Dr Christophe HSU – dermatologist. Geneva, Switzerland

Source of information: Poster (Kostouki I. E. et al.), 10th Congress of the ESPD – Lausanne, Switzerland