Review of the cutaneous manifestations of autoimmune connective tissue diseases in pediatric patients

Table 1 Cutaneous findings, diagnostic criteria, and treatments for juvenile systemic connective tissue disease

Disease	Cutaneous findings	Diagnostic criteria		Treatment
Lupus erythematosus	Acute cutaneous lupus Malar rash Generalized erythematous macules and papules, sparing the knuckles on dorsal hands Mucosal ulcerations Bullous lupus Subacute cutaneous lupus Photodistributed annular eczematous or psoriasiform plaques Chronic cutaneous lupus Discoid lupus Lupus panniculitis Chilblain lupus Tumid lupus	Systemic lupus erythematous requires fulfillment of ≥ 4 criteria with at least 1 criterion from either category:		Mild skin-limited disease Photoprotection Topical corticosteroids Calcineurin inhibitors Hydroxychloroquine Refractory skin disease and systemic disease Hydroxychloroquine Systemic corticosteroids Methotrexate Dapsone (bullous lupus) Other steroid sparing immune modulators
		Clinical criteria Acute cutaneous lupus Chronic cutaneous lupus Oral ulcers Nonscarring alopecia Synovitis Serositis Renal disease Neurologic disease Hemolytic anemia Leukopenia Thrombocytopenia	Immunologic criteria ANA Anti-dsDNA Anti-Smith Anti-phospholipid antibody Low complement Direct Coombs’ test
Systemic juvenile idiopathic arthritis	Transient salmon-pink macules and edematous papules Flagellate erythema	Onset before 16 yr of age, ≥ 6 wk of arthritis, ≥ 2 wk of fever At least one of the following: Evanescent eruption Generalized lymphadenopathy Splenomegaly Serositis		Systemic corticosteroids Methotrexate Biologic agents Supportive symptomatic measures for skin manifestations
Juvenile dermatomyositis	Gottron’s papules–lichenoid papules overlying phalangeal joints, elbows, knees Heliotrope rash of eyelids Poikiloderma of upper back and chest Hyperkeratosis and fissuring of fingertips Mucosal ulcers Gingival telangiectasia Calcinosis cutis	Fulfillment of all five criteria: Symmetric proximal muscle weakness Elevated skeletal muscle enzymes Electromyography changes1 Muscle biopsy abnormalities1 Characteristic skin findings		Photoprotection Hydroxychloroquine Systemic corticosteroids Methotrexate IVIG Other steroid sparing immune modulators
Juvenile onset systemic sclerosis	Limited Sclerosis limited to distal extremities and face CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasias) Diffuse Diffuse sclerosis	Skin sclerosis and ≥ 2 of the following:		Avoidance of vasospasm triggers Methotrexate Physiotherapy Other immune modulators
		Sclerodactyly Raynaud’s phenomenon Nailfold capillary abnormalities Digital tip ulcers Dysphagia Gastroesophageal reflux Neuropathy Carpal tunnel syndrome Tendon friction rubs Arthritis Myositis	Arrhythmias Heart failure Renal crisis New-onset arterial hypertension Pulmonary fibrosis Decreased DLCO Pulmonary arterial hypertension Antinuclear antibodies SSc-selective autoantibodies
Morphea	Linear Linear erythematous indurated plaques En coup de sabre–indurates plaques affecting forehead and scalp Parry Romberg–indurated plaques of the face with ipsilateral facial atrophy Circumscribed Isolated indurated plaques Generalized 4 or more circumscribed type plaques that are larger than 3 cm in diameter Involving at least 2/7 anatomic sites: head- neck, right upper extremity, left upper extremity, right lower extremity, left lower extremity, anterior trunk or posterior trunk Pansclerotic Diffuse involvement of the limbs, trunk, face and scalp with fixation of underlying structures Mixed 2 or more different types of morphea present	No sclerosis of internal organs, except for rare involvement of underlying fascia, muscle and bone		Topical corticosteroids Calcipotriene UVA1 phototherapy Severe or critical location of lesions Systemic corticosteroids Methotrexate

¹These tests are typically not done in children and have been clinically replaced with MRI evidence of muscle inflammation and autoantibodies compatible with juvenile dermatomyositis.