childhood type dermatomyositis

Childhood Type Dermatomyositis: A Rare Autoimmune Disorder

Childhood type dermatomyositis, also known as juvenile dermatomyositis (JDM), is a rare autoimmune disorder that affects children and young adults. It is characterized by muscle inflammation and a distinctive skin rash.

Muscle Involvement

The childhood type of dermatomyositis shows a specific constellation of pathologic changes in muscle [5][6]. Capillary necrosis leads to capillary loss, generally starting on the periphery of muscle fascicles. Electron microscopy discloses undulating tubules in endothelial cells, lymphocytes, pericytes, and other cellular components.

Skin Rash

The disease is also marked by a distinctive skin rash, which can include:

• Heliotrope dermatitis: a purple-colored rash on the face
• Gottron papules: raised, pink patches on the knuckles and elbows

Other Symptoms

Children with JDM may experience other symptoms, including:

• Muscle weakness and fatigue [1]
• Difficulty swallowing or voice changes
• Joint pain and swelling
• Gastrointestinal problems
• Heart, lung, kidney, and eye involvement in some cases

Diagnosis and Prognosis

Diagnosis of JDM is suspected clinically based on the presence of characteristic skin signs and muscle weakness. Investigations, including blood tests and imaging studies, can confirm the diagnosis and help predict prognosis [3].

Treatment and Management

While there is no cure for JDM, treatment options are available to manage symptoms and improve quality of life. These may include medications to reduce inflammation, physical therapy to maintain muscle strength, and other supportive measures.

References:

[1] Children with JDM often complain of weakness, tiredness, and shortness of breath [1]. [2] Factors such as achieving a healthy weight and eating balanced meals can help lessen the likelihood of severe symptoms [2]. [3] Diagnosis is suspected clinically based on skin signs and muscle weakness, confirmed by investigations [3]. [5][6] The childhood type of dermatomyositis shows specific pathologic changes in muscle, including capillary necrosis and undulating tubules [5][6]. [10] Stirling Carpenter published a series of six children with childhood-type dermatomyositis, highlighting the disease's distinctive features [10].

Common Signs and Symptoms of Childhood Dermatomyositis

Childhood dermatomyositis, also known as juvenile dermatomyositis (JDM), is a rare autoimmune disease that affects children. The condition causes inflammation in the muscles and skin, leading to various symptoms. Here are some common signs and symptoms of childhood dermatomyositis:

• Skin Rash: A distinctive red or purplish rash appears around the eyes and cheeks, as well as on the elbows, knees, chest, and back [9][10].
• Muscle Weakness: Muscles in the upper arms, upper legs, neck, and belly slowly get weaker over time, making it difficult to perform daily activities like climbing stairs or getting in and out of a car [4][14].
• Fever: Children with JDM may experience fever as one of their symptoms [5][6].
• Rash around the Eyelids, Knuckles, or Finger Joints: A rash can appear on these areas, which is a classic sign of JDM [6][10].
• Gottron Papules: These are small, raised patches that can appear on the skin, particularly on the elbows and knees [7][8].
• Heliotrope Rash: This is a type of rash that appears as a purple or red discoloration around the eyelids and mouth [7][8].

Other Symptoms

In addition to these common signs and symptoms, children with JDM may also experience:

• Muscle pain and stiffness
• Calcium deposits under the skin (calcinosis cutis)
• Weakness in the muscles of the face and neck

It's essential for parents or caregivers to be aware of these symptoms and seek medical attention if they suspect that their child has childhood dermatomyositis.

References: [4] - Context result 4 [5] - Context result 5 [6] - Context result 6 [7] - Context result 7 [8] - Context result 8 [9] - Context result 9 [10] - Context result 10 [14] - Context result 14

Treatment Options for Childhood Dermatomyositis

Childhood dermatomyositis, also known as juvenile dermatomyositis (JDM), is a rare autoimmune disease that affects children and adolescents. The primary goal of treatment is to reduce inflammation, prevent muscle damage, and manage symptoms.

Medications Used in Treatment

The following medications are commonly used to treat JDM:

• Corticosteroids: These powerful anti-inflammatory drugs are often the first line of treatment for JDM. They can be administered orally or intravenously.
  • Oral prednisone is a common corticosteroid used to treat JDM [3][5].
  • High doses of daily oral prednisone have been reported to lead to faster reduction in symptoms [5].
• Immunosuppressive therapy: This type of treatment is often used in conjunction with corticosteroids to reduce inflammation and prevent muscle damage.
  • Methotrexate is a commonly prescribed immunosuppressive medication for JDM [8][9].
  • Hydroxychloroquine is another immunosuppressive medication that may be used to treat JDM [8].
• Other medications: In some cases, other medications such as azathioprine or cyclosporine may be used to treat JDM.

Treatment Approach

The treatment approach for JDM typically involves a combination of medications and physical therapy. The goal is to reduce inflammation, prevent muscle damage, and manage symptoms.

• Medications: Corticosteroids are often the first line of treatment, followed by immunosuppressive therapy if necessary.
• Physical therapy: Physical therapy can help improve muscle strength and mobility.
• Speech therapy: In some cases, speech therapy may be necessary to address any speech or swallowing difficulties related to JDM.

Remission Rates

The good news is that many children with JDM experience long periods of remission after treatment. According to one study, 57% of patients achieved drug-free remission [14].

It's essential to note that every child with JDM is unique, and the best course of treatment will depend on individual factors such as age, severity of symptoms, and response to treatment.

References:

[1] Systemic corticosteroids are the mainstay of treatment for juvenile dermatomyositis (JDM) [2]. [3] Oral prednisone has been the mainstay of therapy in JDM, and prednisone was reduced faster in patients diagnosed after 1997 [3]. [4] Treatment involves medication, physical therapy, and speech therapy, depending on your child's symptoms [4]. [5] High doses of daily oral prednisone have been reported to lead to faster reduction in symptoms [5]. [6] Methotrexate is a commonly prescribed immunosuppressive medication for JDM [8][9]. [7] Hydroxychloroquine is another immunosuppressive medication that may be used to treat JDM [8]. [8] In contrast to adults with dermatomyositis, children with JDM are more likely to have complications that are thought to indicate a vasculopathic process [14].

Diagnosis of Juvenile Dermatomyositis

Juvenile dermatomyositis (JDM) is a rare autoimmune inflammatory myopathy that affects children, characterized by symmetric, proximal muscle weakness and distinct rashes. The diagnosis of JDM is suspected clinically in a child presenting with the classic skin signs of heliotrope rash, Gottron papules/sign, and myositis.

Investigations for Diagnosis

The diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine kinase (CK), and lactate dehydrogenase (LDH). A biopsy may be performed to definitively diagnose JDM if there is not presence of other typical features, such as rash.

Differential Diagnosis

The differential diagnosis for JDM includes various conditions that present with similar symptoms, such as lupus erythematosus, graft-versus-host disease (GVHD), lichen myxedematosus, lichen planus, and multicentric reticulohistiocytosis. A punch biopsy and knowledge of rare diseases improve the diagnostic ability and therapy.

Diagnostic Criteria

The Bohan Peters diagnostic criteria are used to diagnose JDM, which includes clinical findings such as heliotrope rash, Gottron papules/sign, and myositis. Investigations may include some of the following: Raynaud phenomenon, lupus erythematosus (systemic, discoid, subacute cutaneous), juvenile idiopathic arthritis, scleroderma, morphea, psoriasis.

References

• The diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine kinase (CK), and lactate dehydrogenase (LDH) [3].
• A biopsy may be performed to definitively diagnose JDM if there is not presence of other typical features, such as rash [8].
• The differential diagnosis for JDM includes various conditions that present with similar symptoms, such as lupus erythematosus, graft-versus-host disease (GVHD), lichen myxedematosus, lichen planus, and multicentric reticulohistiocytosis [5].
• The Bohan Peters diagnostic criteria are used to diagnose JDM, which includes clinical findings such as heliotrope rash, Gottron papules/sign, and myositis [10].

Note: The references are based on the provided text and may not be a comprehensive list of all relevant studies or sources.