Dermis & SubQ Flashcards
Ehlers-Danlos Syndrome (cause, 2 types, sxs, tx)
Genetic: Mutations in collagen or post-translational modifications of collagen
Type 1 (Classic) - hypermobile joints, extensive skin stretch, fragile skin, atrophic or “fish mouth” scars. Normal recoil.
Type IV - may involve blood vessels causing bruising and GI / arterial rupture. No tx, just minimize trauma.
Pseudoxanthoma Elasticum (inheritance, onset, sxs)
Autosomal recessive. Appears in first or second decade of life.
Clumped elastic fibers.
Yellow papules of neck, axillae, decreased vision,
CV: claudication (pain in legs w/ walking), high BP, angina, MI
Marfan Syndrome (inheritance, cause, sxs)
Autosomal dominant mutation in fibrillin
Skeletal: tall, long limbs / digits, scoliosis, pectus excavatum, flexible joints
Stretch marks on skin.
CV: aortic dilation / dissection, mitral valve prolapse
Eyes: myopia (near-sighted), dystopia lentis (lens displaced), and detached retina
Livedo Reticularis (cause, sxs, associated disorders)
Due to hypo perfusion of blood vessels
Reticulate ("net-like") macular erythema
Accentuates w/ cold weather
Benign
Associated w/ autoimmune or coagulation disordersHow does vasculitis affect the skin? (4 sxs)
Palpable purpura (Firm red bump that is non-blanching) Necrotizing livedo reticularis Ulceration Leukocytoclastic vasculitis (LCV)
Leukocytoclastic vasculitis (LCV) (mechanism, triggers, sxs, tx)
Inflammation of small cutaneous vessels Palpable purpura, most common on legs Triggers: medications, infection, CT disease, autoimmune disease May affect renal vasculature Tx - treat or remove trigger
3 phases of wound healing
Inflammatory
Proliferative
Maturation
Keloid (presentation, cause, tx)
Firm scar extending beyond borders of wound
Caused by injury, inflammation, or acne
Tx: avoid injury, intralesional steroids, NOT excision (gets bigger)
Local factors that delay wound healing
Vascular insufficiency Tissue edema Infection (due to biofilm) Dry wound bed Excess granulation tissue Chronic radiation injury
Systemic factors that delay wound healing
Old age, malnutrition, diabetes, CT disease
Morphea (sxs)
Affects trunk (mainly), scalp, and extremities
Expanding, erythematous, depressed plaque. Slowly becomes indurated (firm). Central portion becomes hypopigmented w/ lilac-colored rim.
Atrophy and firm scar-like changes
May be linear and associated w/ hair loss or permanent scarring
Inactive lesions are often hyper pigmented
Limited Systemic Scleroderma (sxs)
Affects face and distal extremities w/ tight, thickened skin
CREST Syndrome: Calcinosis cutis (Ca deposition in digits), Raynaud’s Syndrome (Hypersensitivity to cold weather:
Skin becomes white —> blue —> red), Esophageal dysmotility (choke on liquids), Scleroderma, Telangiectasias (dilated capillaries)
Scleroderma (sxs and types)
Fibrosis / thickening of dermis.
Localized includes morphea and linear scleroderma
Systemic includes limited and diffuse scleroderma
Morphea (sxs)
Affects scalp, trunk, and extremities
Hypopigmented, depressed plaque w/ lilac-colored rim
Atrophy and firm scar-like changes
May be linear and associated w/ hair loss or permanent scarring
Inactive lesions are often hyper pigmented
Diffuse Systemic Scleroderma (location and sxs)
Affects trunk and proximal extremities Cutaneous fibrosis Raynaud's syndrome Systemic: Pulmonary fibrosis, renal insufficiency, cardiac disease Poor prognosis
