2/22/13 Elizabeth Parsons, MD

year old female presents with a 4 month history of rash and a nodule on her fingers. PCP diagnosed her with ganglion cyst. The patients symptoms worsened with development of more nodules involving her fingers and worsening of the rash. The rash began to involve her eyelids with associated burning feeling of her eyes. In the past month, she has noted muscle aches and weakness. She is no longer able to hold a loaf of bread and has difficulty walking stairs. She also has difficulty standing from a sitting position.

 Positives:

+ nodules, rash on face and fingers, muscle pains, muscle weakness, decreased ability to perform ADLs, burning eyes, 5-7 lb weight loss, photosensitivity NEGATIVES: fever, tinnitus, vertigo, vomiting, diarrhea, constipation, frequent urination, hematuria, hematochezia, syncope, frequent infections

 WT:

18.5 kg (58%) HT: 108.3 cm (55%) VITALS: T 35.8 HR 111 BP 100/56 GEN: WDWN, NAD EYES: Mild erythema of conjunctiva. Normal sclerae. Pupils equal and reactive. + photophobia OP: Normal mucosal surfaces. NECK: No LAD. Normal thyroid SKIN: + purple rash over her eyelids with swelling extending to cheeks bilaterally. + erythematous papules over MCP and PIP joints.

 RESP:

Normal respiratory effort CV: RRR. No murmurs. CRT < 2 seconds ABD: soft, ND, NT. + BS. No HSM MSK: 1+ swelling and warmth of wrists, trace swelling of PIP joints, + telangectasias under nail beds. Upper ext strength 3/5. Lower ext strength 5/5. + Gowers sign NEURO: No abnormalities

Muscular dystrophy Systemic Lupus Erythematosis Polymyositis Juvenile Dermatomyositis Drug induced myositis Viral myositis Eczema Psoriasis JIA Mixed Connective Tissue Disease Scleroderma Psoriasis Contact dermatitis Urticaria

CBC: WBC: 5.5 (nml diff) HCT: 41.1 Plt 492 CMP: Normal CRP: 0.2 C3/C4: normal Hep B, Hep C: negative CCP < 0.5 CK 49 Aldolase 10.5 (mildly elevated) LDH 796 Urine pr/cr = 0.1 Anti Smith = 0 DSDNA = 0 RF < 15

 Juvenile

Dermatomyositis

% of rheumatologic disease in childhood Presentation from age 4-10 (25% < age 5) Vasculopathy of the skin and skeletal muscle Para-neoplastic association in adults, but not in children Children have overall better prognosis than adults diagnosed with disease Girls predominate 2:1 No racial bias No family predisposition

 Painless

weakness: fatigue and symmetrical, progressive, proximal muscle weakness, particularly affecting the limb-girdle musculature (shoulders and hips), anterior neck flexors, and trunk muscles. I can no longer climb stairs Some patients have myalgias of patients have arthritis and arthralgias Dysphagia, dysphonia, dyspnea Cutaneous Manifestations

Malar

Heliotrope

Spares naso-labial folds

Diffuse, +eye swelling, does not spare nasolabial folds

Zitelli et al.

 Long

standing, untreated disease

 Muscle

disease- elevated CK, aldolase, ALT, AST, and LDH (can be used to monitor disease activity) Elevated vWF in severe vasculitis Imaging (xray, MRI) Muscle Biopsy EMG Labs to rule out other differential diagnoses (ANA, antiRNP, Smith, DS DNA, RF, etc)

Retinitis Hepatosplenomegaly Pancreatitis Vasculopathy of the gastrointestinal tract leading to ulceration, bleeding, or perforation Respiratory muscle weakness with a restrictive pattern on lung function studies, Intersitital lung disease Aspiration pneumonitis from pharyngeal muscle weakness Myocarditis, and pericarditis.

 Corticosteroids
Methotrexate Plaquenil Biologics Severe

vasculitis (cyclophosphamide, IVIG)