Rheumatism that will "rot the face", never forget it after reading it

* Only for medical professionals to read and refer to dig deep into the truth behind the "rotten face"!

Case profile

The patient, male, 15-year-old student, was admitted to the hospital
for "lower back pain for more than 3 months, and then worsened by 1 day".

The patient had no obvious cause of low back pain before 3 months, was severe at night, and had no obvious limitation of movement, morning stiffness, peripheral joint involvement, oral and genital ulcers, abdominal pain and diarrhea, fever and chills, and was not paid attention to and treated
.

One day ago, the patient began to have fever, the highest body temperature was 37.
8 ° C, the lumbosacral and chest pain were obvious, and he was hospitalized in our hospital again
.

History: The patient began to have severe acne on the face and face before 3 months, and had taken isotretinoin softgels and topical medication for treatment, and the acne improved
.

Physical examination: dark red papules on the face, small pustules on the top of the papules, partial post-inflammatory scars, no palmoplantar pustules, sternal stalk tenderness, bilateral 4-sign sign, negative straight leg elevation test, negative bilateral sacroiliac joint percussion
.

Laboratory tests:

(1) Blood routine + hypersensitivity CRP: WBC 9.

There are no special cases of liver and kidney function, creatine kinase, antinuclear antibody (ANA), rheumatoid factor (RF), HLA-B27, immunoglobulin, complement, infectious index, urine routine, etc.

Cytomegalovirus DNA, Epstein-Barr virus DNA, and tuberculosis T cells all tested negative
.

(2) Systemic bone imaging: sternum, bilateral collarbone, 11th thoracic vertebrae, right 6th anterior rib local bone metabolism is active
.

(3) PET-CT: bilateral sternal clavicle end, bilateral first rib, sternal stalk and sternal body margin, thoracic 11 vertebral anterior edge bone hardening: bilateral sternoclavicular joint, bilateral 1st thoracular rib joint and bilateral 2-5 rib cartilage at FDG metabolism increased to varying degrees, suggesting SAPHO syndrome
.

Finally, the doctor made the diagnosis: synovitis - acne - impetigo - osteopoea - osteopathy (SAPHO syndrome)

SAPHO syndrome refers to a syndrome that clinically presents with synovitis, acne, impetigo, osteopenia, and osteitis
.

It first came to the attention in 1961, and since then cases have been reported intermittently, but mainly in Europe
.

French doctor Chamot et al.
first proposed the concept
of SAPHO syndrome in 1987 after reviewing 85 cases.

The etiology of this disease is not yet clear, the lesions often involve the skin, bones and joints, the course of the disease is prolonged, clinically characterized by intermittent recurrence and remission, more likely to occur in young and middle-aged age, skin lesions are characteristic, including palmoplantar pustules, pustular psoriasis, cluster acne, fulminant acne and purulent hidradenitis
.

SAPHO syndrome is a spectrum of diseases that covers more than 50 disease names
.

Some patients have osteoarthropathy that occurs later than skin lesions, which is a great challenge
for clinical diagnosis.

Therefore, improving clinicians' understanding of the characteristic skin manifestations and imaging manifestations of SAPHO syndrome has great benefits
for the misdiagnosis and missed diagnosis of the disease.

1

The diagnosis
of SAPHO syndrome is characterized by skin lesions and osteoarthropathy and can generally occur
at any age.


1 Clinical manifestations • skin lesions: 20% to 60% of patients have skin lesions, palmoplantar pustules is one of the most common skin features of SAPHO syndrome, manifested
as chronic recurrent aseptic small pustules at the palmoplantar site, accompanied by keratinization and scaling
.

Acne and psoriasis lesions are also common skin manifestations
.

Other rare skin manifestations are Sweet syndrome and gangrene pyodermatitis lesions, which can occur before, during, or after
osteoarticular changes.


•Osteoarthropathy: mainly synovitis, bone hypertrophy, and osteitis, usually with insidious onset, mainly pain, swelling with or without morning stiffness, and occasionally osteolysis
may be seen early in the course of the disease.


▌ The site of onset is age-related
• Children and adolescents are mainly epiphyseal of the long shaft, followed by the spine and clavicle
.


•Adults primarily involve the anterior upper chest wall, followed by the spine and pelvis
.

The long course of the disease can lead to bone hypertrophy, fusion, and compression of neurovascular structures, causing pain and edema in the upper chest wall and upper extremities, that is, "chest syndrome", and bone hardening and bone hypertrophy in the thoracular and clavian areas are more characteristic
.


2 Laboratory tests
for SAPHO syndrome serology have no obvious specificities
.

Non-specific inflammatory indicators such as erythrocyte sedimentation rate and CRP are at normal levels or slightly elevated, and HLA-B27 and ANA are mostly negative
.


Imaging studies are specific and are an important basis for the detection of SAPHO syndrome with osteoarthropathy, including X-ray, ultrasound, CT, MRI, PET-CT, etc
.

The main radiographic manifestations of the disease are bone hypertrophy and osteitis, which are manifestations
of chronic inflammatory responses to the cortex and medullary cavity of the affected bone.

Bone hypertrophy is characterized by chronic periosteal reactions and cortical thickening leading to bone hypertrophy, and osteositis usually manifests as bone hardening, which is highly specific
.

ECT scans help to show abnormal metabolic activity in the bone, early detection of lesions, and the concentration of tracers in the thoracic rib-locked area is horn-like, called "bull's head sign", which is a highly characteristic manifestation
of the disease.


3 diagnostic criteria for SAPHO syndrome have been continuously modified since they were proposed, and the 2003 Kahn diagnostic criteria
of the American College of Rheumatology (ACR) are shown
below.

Even if the diagnostic criteria are clarified, the clinical diagnosis is still very difficult, and only about 1/4 of patients have osteoarticular lesions that occur
at the same time as the characteristics of dermatological diseases.

2

Characteristic manifestations
of SAPHO syndrome 1 Characteristic skin lesions Typical skin lesions are palmoplantar impetigo and acne, skin lesions can occur at the same time as or later than osteoarticular lesions for years or even decades, but only about 55% of patients with SAPHO syndrome have skin lesions

throughout the course of the disease.


1.
Palmoplantar impetigo is usually manifested as clustered acne, fulminant acne and purulent hidradenitis, and is manifested as multiple aseptic pustules of the palm mixed with yellow-brown spots
.


2.
Psoriasis
.


3.
Severe acne includes clustered acne, fulminant acne, purulent hidradenitis
.


A and B are palmoplantar impetigo; C: Psoriatic involvement of nails; D: Severe acne
2 characteristic bone lesions SAPHO syndrome bone lesions
are mainly anterior chest wall syndrome with thoracic-rib-collar-joint involvement, followed by spinal and sacroiliac joint involvement; Presents with symmetrical pain in the anterior upper chest wall, involvement of clavicle and shoulder joint movement, low back pain, or limited
lower back movement.


2% thoraccular-rib-collar-joint involvement: bone scan suggests bilateral sternoclavicular joint, bilateral first anterior rib, abnormal concentration of sternal tracers, and "bull's head sign" of the sternoclavicular joint (Figure 2b), typical imaging findings
of SAPHO syndrome.

a: Anterior and anterior radionuclide bone imaging suggests increased uptake of bilateral sternoclavicular joint, sternum, spine, sacral and bilateral sacroiliac joints;

b: increased uptake in both sternoclavicular joints and sternal stalks (minotaur sign)

2% of spinal involvement spine is the second most common site of SAPHO involvement, with the thoracic spine being the most common, followed by the lumbar spine and finally the cervical spine
.

Five characteristic manifestations are nonspecific discitis, vertebral angle lesions, osteolysis changes, paravertebral ossification, and vertebral osclerosis
.


▌ 2% of sacral and sacroiliac joint involvement
c: sacral (arrow) abnormal signal, suggesting bone marrow edema; d: abnormal signals around the bilateral sacroiliac joint, more severe iliac signals on the left joint (arrow), suggesting sacroiliitis and bone marrow edema

3

There are currently no guidelines for the treatment of SAPHO syndrome regarding the treatment of this disease, mainly empirical treatment

based on symptom improvement.

Some scholars believe that SAPHO syndrome can be attributed to seronegative spondyloarthritis, so the treatment of seronegative spondyloarthritis is mostly referred to in the treatment strategy
.


1.
Nonsteroidal anti-inflammatory drugs (NSAIDs): NSAIDs have a good effect on controlling osteoarticular symptoms and are a first-line choice for the treatment of this disease, but they are usually ineffective for most patients with chronic pain
.


2.
Glucocorticoids: fast onset of action, suitable for patients with
severe illness or intolerance to NSAIDs.

However, long-term use of hormones can worsen psoriasis lesions
.

Short-term use of the hormone
is recommended for patients with refractory disease.


3.
Antibiotics: Patients with positive biopsy culture at the site of the lesion can also be given antibiotics, such as azithromycin, sulfamethoxazole/trimethoprim, and clindamycin
.


4.
DMARDs can be added when traditional DMARDs are used but NSAIDs
are not effective.

Methotrexate is the first choice in DMARDs, others including sulfasalazine, azathioprine, cyclosporine, hydroxychloroquine, leflunomide, and derivatives of colchicine, calcitonin, interferon-α, and vitamin D have also been reported to be effective
.


5.
Intravenous bisphosphonate: bisphosphonates can promote long-term remission of some patients with refractory NSAIDs, and are suitable for patients with
spinal involvement.


6.
Biological agents: For cases of SAPHO that are ineffective or refractory to conventional drugs, TNF-α inhibitors such as infliximab, etanercept, and adalimumab
can be considered.


7.
Small molecule targeted drugs: If various drug treatments are ineffective, it can be considered for SAPHO syndrome
.


SUMMARY:
SAPHO syndrome is a relatively rare heterogeneous disease with a variety of clinical manifestations
.

Clinically, rheumatologists and dermatologists have difficulty definitively diagnosing the disease
.

Therefore, this paper improves the disease prognosis
of patients by improving clinicians' understanding of SAPHO syndrome and early diagnosis and early treatment.

Where to see more clinical cases of rheumatism?

Come to the "Doctor's Station" and take a look 👇

References:

[1] Li C,Zuo Y,Wu N,et al.
Synovitis,acne,pustulosis,hyperostosis and osteitis syndrome:a single centre study of a cohort of 164 patients[J].
Rheumatology(Oxford),2016,55(6):1023-1030.

[2] Himuro H,Kurata S,Nagata S,et al.
Imaging features in patients with SAPHO/CRMO:a pictorial review[J].
Jpn J Radiol,2020,38(7):622-629.

[3] Yang Q,Zhao Y,Li C,et al.
Case report:successful treatment of refractory SAPHO syndrome with the JAK inhibitor tofacitinib[J].
Medicine(Baltimore),2018,97(25):e11149.

Source of this article: Medical Rheumatism and Immunization Channel This article author: Mu Yi This article reviewed: Chen Xinpeng Deputy Chief Physician Responsible Editor: Tangerine

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