ICD-10: M33.00

Juvenile dermatomyositis (JDM), classified under ICD-10 code M33.0, is an idiopathic inflammatory myopathy that primarily affects children. The clinical presentation, signs, symptoms, and patient characteristics associated with this condition include:

Clinical Presentation

• Age of Onset: Typically occurs in children, often between the ages of 5 and 15 years.
• Gender: There is a higher prevalence in females, with studies indicating that approximately 68.5% of patients are women [13].

Signs and Symptoms

• Skin Manifestations:
• Gottron's Papules: These are erythematous papules that appear over the dorsal surfaces of the joints, observed in about 80% of patients [10].
• Heliotrope Rash: A violaceous rash with edema, particularly around the eyes, seen in approximately 78.5% of cases [10].

• Poikiloderma: Characterized by a combination of hyperpigmentation, hypopigmentation, and telangiectasia, present in about 75.7% of patients [10].

• Muscle Weakness:

• Patients typically present with symmetrical proximal muscle weakness, which can affect daily activities such as climbing stairs or lifting objects.

• Systemic Symptoms:

• Fatigue, fever, and weight loss may also be reported, indicating systemic involvement.

Patient Characteristics

• Clinical Signs: The most frequent clinical signs include:
• Gottron's papules (80%)
• Heliotrope erythema with edema (78.5%)

• Poikiloderma (75.7%) [10][13].

• Muscle Involvement: JDM is characterized by muscle inflammation, which can lead to significant weakness and disability if not treated promptly.

• Variability: The disease is heterogeneous, meaning that symptoms and severity can vary widely among patients. Some may experience more severe muscle involvement, while others may have predominantly skin symptoms [15].

Organ Involvement

• While the ICD-10 code M33.0 specifies "organ involvement unspecified," it is important to note that JDM can potentially affect other organs, including the lungs and gastrointestinal tract, although these manifestations are less common.

In summary, juvenile dermatomyositis is marked by distinctive skin rashes, muscle weakness, and a higher incidence in females. Early recognition and treatment are crucial to managing symptoms and preventing complications.