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A form of polyostotic fibrous dysplasia characterized by multiple unilateral bone lesions, endocrine anomalies (i.e. precocious puberty) and unilateral pigmented skin lesions |
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Strata (surface to base): Corneum, Lucidum, Granulosum, Spinosum, Basalis |
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Type I vs. Type II osteoperosis |
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Type I: Post-menopausal caused by decreased estrogen Type II: Senile variant affects men and women over 70 |
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Osteoclast malfunction leads to dense bones. Less marrow space- thrombocytopenia, anemia, infection. |
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Hyperparathyroidism. "Brown Tumors" High: Serum Ca++, Alk. Phos. Low: Serum Phosphorus |
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Abnormal bone architecture 2ndary to increased osteoblast/osteoclast activity. Elevated Alk. Phos. "Chalk Stick" fractures |
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progressive proximal symmetrical muscle weakness cause by CD8+ Tcell induced injury to myofibers. Biopsy is diagnostic. Dermomyositis is similar, but involes skin rash + malignancy risk |
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Triad: Dry mouth, arthritis, dry eyes. Usually women 40-60. Assoc. w/RA. |
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Immunoglobulins, Malar rash, Discoid rash, Antinuclear antibodies, Mucositis, Neurological problems, Serositis, Hematologic anomalies, Arthritis, Renal problems, Photosensitivity. |
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Gammaglobulinemia, RA, ACE increase, Interstitial fibrosis, Noncaseating gramulomas. |
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Triad: Urethritis, Conjunctivitis/uveitis, Arthritis. Assoc. w/HLA-B27. Occurs post-GI/chlamydia infection. |
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Excessive fibrosis/collagen deposition throughout body. 75% female. Two categories. 1. Diffuse scleroderma: rapid, widespread skin involvement + viscera, anti-Scl-70 ab 2. CREST syndrome (more benign): Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasia |
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Atopic dermatitis (eczema) |
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Definition
pruritis eruptions on flexur surfaces |
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Epidermal hyperplasia, w/parakeratotic scaling. Knees & elbows. Auspitz sign. More spinosum, less granulosum. |
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Pruritic papules/vesicles; IgA deposits; Assoc. w/ celiac disease. |
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Pruritic, purple, polygonal, papules. Lymphocytes infiltrate dermoepidermal junction. |
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This response to sulfa drugs/anticonvulsants is the more common type of erythema multiform. Many rash types, including target lesions. |
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Benign flat pigmented squamous proliferation/keratin cysts |
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Wegeners minus granulomas. P-ANCA instead of C-ANCA |
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Premalignant small, rough red/brown papules assoc. w/sun exposure. |
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Anti-desmosome Abs creates bullae in mucosa + skin, acantholysis (breakdown of cell/cell junctions). Potentially fatal. |
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"Pulseless disease"- granulomatous thickening of great vessels. Mostly asian women. Elevated ESR Fever, Arthritis, Night sweats, Myalgia, Skin nodules, Ocular disturbance, Weak pulse (upper ext.) |
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Most common vasculitis affecting medium/small arteries. Elevated ESR. Treat w/ steroids. Older women. |
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Necrotizing immune complexes in renal/visceral vessels. Fever, Malaise, Headache, Myalgia, Weight loss, etc. Treat w/ corticosteroids, cyclophosphamide. |
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Triad: focal necrotizing vasculitis, upper airway/lung necrotizing granulomas, necrotizing glomerulonephritis. Also: perforation of nasal septum, otitis media, sinusitis, mastoiditis, caugh, dyspnea, hematuria, hemoptysis, aneurysms. C-ANCA is a strong marker. |
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