icd 10 code for sapho syndrome

Full Answer

What is a Sapho diagnosis?

SAPHO is an acronym for the combination of synovitis, acne, pustulosis, hyperostosis, and osteitis. Do I need to break it down into the individual Diagnosis?

What is synovitis (Sapho)?

The term SAPHO (an acronym for synovitis, acne, pustulosis, hyperostosis, osteitis) was coined in 1987 to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.

What is the ICD 10 code for Sayre syndrome?

Kearns-Sayre syndrome. H49.81 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail. The 2019 edition of ICD-10-CM H49.81 became effective on October 1, 2018.

What is the ICD 10 code for Kearns-Sayre syndrome?

Kearns-Sayre syndrome. H49.81 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail. The 2019 edition of ICD-10-CM H49.81 became effective on October 1, 2018. This is the American ICD-10-CM version of H49.81 - other international versions of ICD-10 H49.81 may differ.

What is SAPHO syndrome?

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized by synovitis, osteitis, hyperostosis, and enthesitis, typically with pain, swelling, and tenderness in affected areas.

How is sapho diagnosed?

How is the diagnosis made? SAPHO syndrome is suspected when a patient presents with a pustular skin disease in association with rheumatic pain. Magnetic resonance imaging (MRI scan) shows inflammation of the bone marrow or joints at characteristic sites such as the collar bone, breast bone, pelvis, heel, and lower jaw.

Is SAPHO syndrome a disability?

Despite its chronic and recurrent nature, SAPHO syndrome is very rarely the cause of major disability, even after several decades of evolution. Objective: We want to illustrate through three clinical cases, the importance of the anamnesis, the clinic, the diagnostic criteria in the diagnosis of SAPHO syndrome.

Is SAPHO syndrome serious?

In general, SAPHO syndrome should be suspected in patients with bone and joint symptoms consistent with inflammatory arthritis and/or osteitis, especially when it involves the anterior chest wall, sacroiliac joints, or spine, and particularly when occurring in association with a neutrophilic dermatosis or acneiform ...

What is the treatment for sapho syndrome?

Treatment options for SAPHO include NSAIDs, anti-rheumatic drugs, such as colchicine, corticosteroids and biphosphonates, and disease-modifying agents, such as methotrexate, sulfasalazine and infliximab [29].

What is chronic recurrent multifocal osteomyelitis?

Chronic recurrent multifocal osteomyelitis (CRMO) is a disease that causes pain and damage in bones due to inflammation. CRMO is a severe form of chronic nonbacterial osteomyelitis (CNO). It may affect 1 or more bones. Symptoms may come and go for years.

Does arthritis cause acne?

Is there a connection between rheumatoid arthritis and acne? There isn't much scientific evidence to show acne is related to RA. People who have RA and acne may be experiencing the two conditions independently. However, research has shown that RA is related to certain other skin conditions.

Is sapho hereditary?

The precise cause of SAPHO syndrome is not known. It is felt that the tendency toward developing SAPHO syndrome can be inherited. Genetic predisposition is suggested by the higher prevalence of HLA-B27, an inherited blood marker, in patients with SAPHO syndrome.

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Epidemiology

Although its incidence and prevalence are probably underestimated, SAPHO syndrome (acronym for Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis) is considered to be a rare disorder.

Clinical description

The age of onset ranges from adolescence to late adulthood, with a median age between 30 and 40 years. SAPHO syndrome encompasses a wide spectrum of anomalies characterized by variable combinations of osteoarticular and cutaneous manifestations of varying degrees of severity.

Etiology

The etiology is unknown. SAPHO syndrome seems to have a multifactorial origin with genetic, environmental, immunologic, and infectious components. Slow-growing bacteria, such as Propionibacterium acnes, could act as a triggering factor.

Diagnostic methods

Diagnosis, suspected upon clinical examination, must be confirmed with imaging procedures (X-rays, CT, MRI) showing a combination of osteolysis and osteosclerosis with secondary hyperostosis, bone marrow edema, endosteal-periosteal inflammation, perifocal myositis, and adjacent arthritis.

Differential diagnosis

Differential diagnosis includes infectious osteomyelitis or arthritis, Langerhans cell histiocytosis, and bone tumors such as Ewing sarcoma, osteoblastoma, and osteoid osteoma. Hypophosphatasia may mimic the bone phenotype of SAPHO syndrome.

Management and treatment

Treatment is mainly symptomatic and is based on non-steroidal anti-inflammatory drugs, sometimes replaced by corticosteroids (e.g. prednisone). In resistant cases, disease-modifying antirheumatic drugs (e.g. methotrexate) can also be used. Intravenous biphosphonates (pamidronate, zoledronate) can alleviate bone pain.

What is SAPHO syndrome?

Specialty. Rheumatology. SAPHO syndrome includes a variety of inflammatory bone disorders that may be associated with skin changes. These diseases share some clinical, radiologic, and pathologic characteristics. An entity initially known as chronic recurrent multifocal osteomyelitis was first described in 1972.

Which bones are affected by SAPHO syndrome?

In children, the SAPHO syndrome is most likely to affect the metaphysis of long bones in the legs (tibia, femur, fibula), followed by clavicles and spine.

What is the acronym for synovitis?

The term SAPHO (an acronym for synovitis, acne, pustulosis, hyperostosis, osteitis) was coined in 1987 to represent this spectrum of inflammatory bone disorders that may or may not be associated with dermatologic pathology.

What is the cause of SAPHO syndrome?

SAPHO syndrome involves any combination of: S ynovitis (inflammation of the joints), A cne, P ustulosis (thick yellow blisters containing pus) often on the palms and soles, H yperostosis (increase in bone substance) and O steitis (inflammation of the bones). The cause of SAPHO syndrome is unknown and treatment is focused on managing symptoms.

Can you treat SAPHO syndrome?

There is no specific treatment plan for SAPHO syndrome . It can be a chronic condition but sometimes eventually heals on its own. Joint pain may be managed with nonsteroidal anti-inflammatory drugs and prescription vitamin A is used to treat the acne.