Neonatal lupus syndrome occurs when a newborn or a mother with clinical or serological evidence of SLE, rheumatoid arthritis (RA), MCTD or Sjogren's syndrome has:

• Congenital complete heart block
• Low platelet count (thrombocytopenia)
• Rash (erythema annulare)
• Hepatitis

Vasculitis can be both a primary childhood disease and a feature of other syndromes, including dermatomyositis (JDMS) and systemic lupus erythematosus (SLE)

• One of the most common forms of vasculitis is children in Henoch-Schonlein purpura (HSP). This syndrome consists of a rash, arthritis, abdominal pain and renal dysfunction. Signs and symptoms may occur for several days or weeks. The rash progresses from red to purple to brown and most commonly is located on the buttocks and lower extremities.
• Polyarteritis nodosa is a vasculitis of small and medium-sized blood vessels. Clinical manifestations are similar to those that adults experience. It can affect any organ system of the body, but most frequently involves the skin, peripheral nerves, kidneys, intestinal tract, and joints.
• Kawasaki disease symptoms include fever and rash, which include bright red, swollen lips that crack and bleed. Heart involvement is the most serious complication of this form of vasculitis, which predominantly occurs in the very young child (boys more often than girls).
• Some other forms of juvenile vasculitis include cutaneous polyarteritis, Wegener's granulomatosis, giant cell arteritis (Takayasu's arteritis and temporal arteritis), and Beçhet's syndrome.

Juvenile scleroderma is rare in children but includes both localized and systemic disease. The skin, muscles, tendons, joints and bones are affected -- often resulting in severe growth abnormalities. Localized scleroderma includes several types of skin lesions:

• Morphea has at least one plaque of thickened skin, excluding post-traumatic skin scarring;
• Linear scleroderma has at least one band-like tight lesion involving skin and subcutaneous tissues.

Children may have other overlapping diseases with features of chronic arthritis, scleroderma, dermatomyositis (JDMS), lupus (SLE) and Sjogren's syndrome.

Other Connective Tissue-Related Diagnoses

• Raynaud's Phenomenon -- Sudden, reversible "dead-white" uniform coloring of the extremities caused by exposure to cold and occurring in the absence of another recognized connective tissue disease. Raynaud's phenomenon is often a symptom of connective tissue diseases, especially scleroderma.
• Primary Sjogren's Syndrome -- Symptoms of dry eyes, dry mouth, and parotid gland swelling in the absence of another defined connective tissue disease. Sjogren's syndrome is often called Sicca syndrome if it is a symptom of connective tissue diseases.
• Panniculitis -- Generalized painful subcutaneous nodules. Diagnosis is made by skin biopsy with evidence of inflammatory cells in the subcutaneous tissue.
• Erythema Nodosum -- Painful, erythematous nodules over the upper surfaces of the legs and sometimes the arms.

Juvenile Non-Inflammatory Disorders

• Benign Hypermobility Syndromes -- Generalized hypermobility of the joints is associated with musculoskeletal pain but not with underlying connective tissue disease. Hypermobility syndrome represents extreme variation of the usual range of joint motion. Girls are more often affected than boys, and frequently there is a family history of hypermobility.
• Pain Syndromes -- Non-inflammatory disorders are important causes of chronic or recurrent pain in children. They arise from a variety of causes -- some congenital and others acquired possibly as the result of injury and others of unknown cause. These include:
  • Growing Pains -- The cause of growing pains is unknown. They occur equally among boys and girls. They cause deep aching, bilateral cramping pain in the thigh or calf usually in the evening or during the night, which responds to massage and analgesia. Physical and laboratory exams are negative.
  • Primary Fibromyalgia Syndrome (diffuse idiopathic pain syndrome) -- Fibromyalgia is characterized by diffuse, often ill-defined musculoskeletal aching and stiffness. The child may have constant fatigue, disturbed sleep patterns, anxiety and depression. Diagnosis is based on history and demonstration of multiple tender points (at least three) at characteristic locations. Arthritis does not occur, and the child may appear otherwise healthy. The pain is very real, but not caused by a serious underlying disease. Treatment consists of a combination of exercise and pain medication.
  • Reflex Sympathetic Dystrophy (RSD) (localized idiopathic pain syndrome) -- Children and adolescents with RSD have a history of constant pain and increasing disability in the distal part of an extremity that is exacerbated by even mild activity. The involved extremity may have diffuse swelling, tenderness, coolness, and mottling. Even slight touch may cause severe discomfort, and attempts to move the limb are extremely painful. Treatment includes pain control to allow physical therapy of the affected limb. A psychologist may be needed to assist the child in pain control or desensitization.
  • Overuse Syndromes -- Syndromes related to overuse of joints occur with considerable frequency in children and adolescents. The growing child is particularly susceptible to overuse injuries.