ANSWER TO FELLOW’S CHALLENGE –
NOVEMBER-DECEMBER 2004
A 15 year old male
has had a rash on his chest for 10 months. The rash has responded poorly to
topical creams. He has also experienced low grade fevers intermittently and
severe joint pains. He has had no other rash, no swollen joints, no high
fevers, no weight loss, no mouth ulcers, no alopecia, no Raynaud’s, no
muscle weakness, no color changes to his extremities, or hyperesthesia.
Physical
exam reveals a healthy appearing adolescent male in no distress. He has a
prominent raised, red rash on his chest (Figure 1). His knees and elbows are
tender and painful on range of motion. There is no joint swelling or limitation
of motion. He has no muscle weakness. He has no enlarged lymph nodes, no enlarged
liver or spleen, no heart or lung findings, and no mouth sores or palatal rash.
The rest of his exam is normal.
Lab
tests reveal hemoglobin of 10 g%, a WBC of 6500 cells/mm3. The platelet count is 480,000 cells/mm3. The ESR is 118 mm/hour. The chemistries
and urinalysis is normal. The ANA and RF are normal. Blood cultures are
negative. The bone scan is positive for increased uptake in multiple extremity
bones.
A. Your diagnosis is: _____SAPHO__________________
1.
Tuberculosis
2.
Psoriatic arthritis
3.
Chronic multicentric osteomyelitis
4.
SAPHO syndrome
5.
Systemic onset JIA
6.
Reactive arthritis
B. The teen improves dramatically over the
next year on which medication (ESR decreases to 13 mm/hour)?
1.
Naproxen
2.
Solumedrol pulses
3.
Cyclophosphamide
4.
Thalidomide
5.
Methotrexate
6.
Colchicine
We are grateful to Sujata
Sawhney of
Discussion
SAPHO, a term first
coined in 1987 [1], is a clinical syndrome of various combinations of synovitis, acne, pustulosis,
hyperostosis, and osteitis. Included under this
umbrella term are a variety of other entities (such as chronic recurrent multifocal osteomyelitis, acne
arthritis, arthritis associated with hidradenitis suppurativa) which manifest reactive osteitis
with or without the presence of cutaneous pustular lesions.
SAPHO is described primarily in young adults, but occurs in children and
older adults. It has a fairly equal sex
distribution. Most case reports are from
The pathogenesis is
unknown. It is thought to be a reactive
process, possibly to an infectious trigger.
Although there are a few case reports of positive bone and synovial tissue cultures for Propionibacterium
acnes [2], the vast majority of osseous and joint cultures are sterile. There are some similarities between SAPHO and
the spondyloarthropathies. These include an involvement of both
peripheral and axial joints (including sacroiliitis
and spondlyodiscitis), a suspected infectious trigger
and a slightly increased incidence of HLA-B27 (13%) [3]. Also, SAPHO is reported in patients
with inflammatory bowel disease [4].
There are several osteo-articular manifestations of SAPHO. The most common lesion is osteosclerotic
hyperostosis, especially of the anterior chest wall, involving sternoclavicular, sternocostal
and/or sternomanubrial joints. Commonly reported bony sites of involvement
include the clavicle, vertebrae, sacrum, ilium,
public symphysis and mandible. Peripheral osteitis
is rare. Axial arthritis is seen more
commonly (91.9%) than peripheral (36%) arthritis [3]. Biopsies of early bony lesions show acute
inflammation identical to that seen in infectious osteomyelitis,
while biopsy specimens of older lesions display sclerotic bone and only mild
chronic inflammation [5].
The skin
manifestations can be varied and impressive. The skin rashes are aseptic and
filled with neutrophils. These lesions include palmoplantar pustosis, non-palmoplantar pustulosis,
psoriatic vulgaris, severe acne, and rarely Sweet’s
syndrome or pyoderma gangrenosum. The skin lesions may present years before, or
after, the skeletal manifestations, or they may present simultaneously.
Treatment is still
empirical with no evidence-based recommendations. The majority of patients are given a trial of
non-steroidal anti-inflammatory medications.
Corticosteroids are often tried in patients who do not respond well to NSAIDs. Methotrexate has produced promising results in some
patients [3]. Pamidronate
is another alternative [6]. A recent
report described an excellent response in two patients to treatment with infliximab [7].
The teenager in
this case report demonstrated systemic inflammation, skeletal involvement and
severe acne, consistent with a diagnosis of SAPHO. The patient’s physician chose to treat him
with methotrexate to which he responded
dramatically. Methotrexate
is a reasonable choice in this clinical setting; it is a medication which may
provide good results without major side effects.
Charles H. Spencer, MD
Linda Wagner-Weiner, MD
References
1. Chamot AM, Benhamou CL, Kahn MF,
et al. Le syndrome acné pustulose hyperostose ostéite (SAPHO): resultats d’une
enquête nationale. 85 observations.
Rev
Rhum Mal Osteoartic. 1987;54:187-96.
2. Kotilainen P, Merilahti-Palo R, Lehtonen OP, et al. Propionibacterium acnes isolated from sternal
osteitis in a patient with SAPHO syndrome.
J Rheumatol.
1996;23:1302-4.
3. Hayem G, Bouchaud-Chabot A, Benali K, et al.
SAPHO syndrome: A long-term follow-up study of 120 cases. Seminars Arthritis Rheum.
1999:29:159-171.
4. Kotilainen PM, Laxen FO, Manner
IK et al. An aseptic inflammation of the
clavicle in a patient with Crohn’s disease: a
potential manifestation
of the SAPHO syndrome.
Scand J Rheumatol 1996;25:112-4.
5.
Reith JD, Bauer TW, Schils JP. Osseous manifestations of SAPHO (synovitis,
acne, pustulosis, hyperostosis, osteitis)
syndrome.
Am J Surg Path. 1996;20:1368-1377.
6. Amital H, Applbaum YH, Aamar S, et al.
SAPHO syndrome treated with pamidronate: an
open-label study of 10 patients.
Rheumatology. 2004;43:658-61.
7. Olivieri I, Padula A, Ciancio G, et al. Successful treatment of SAPHO syndrome with
infliximab: report of two cases.
Ann
Rheum Dis. 2002;61:375-376.
The majority of fellow responses correctly
identified SAPHO as the diagnosis. The
first response was received by:
Dr. Esra Ozer