Term
Dermal Induration Important Points:
Presents as ____ or ____ skin
Usually requires a ____ |
|
Definition
|
|
Term
| Scleroderma/Morphea Definition: |
|
Definition
| Increase in the number and activity of fibroblasts that produces excessive collagen |
|
|
Term
| _____ – scleroderma localized to the skin |
|
Definition
|
|
Term
________________________
More diffuse cutaneous fibrosis
Classified as an ‘autoimmune’ collagen vascular disease |
|
Definition
| Progressive systemic sclerosis |
|
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Term
Scleroderma/Morphea
•History
–PSS
•_______ phenomenon most commonly seen initially
–___>____>____
»____caused by vasoconstriction
•Skin tightening
•Contracture of the fingertips
•Difficulty swallowing
•Shortness of breath
•Joint pain |
|
Definition
Raynaud’s
White -> purple -> red
White |
|
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Term
Scleroderma/Morphea
•Physical Examination
–Sharply demarcated _____
–____ lesions – violaceous hue and whitish center
–____ lesions – brown or yellow brown
–Most common on ____
–Frontal linear scleroderma – _________ -> have to send them to opthamology b/c can affect the eye! Usually treated w/ methotrexate and prednisone
–_____– skin tightening that affects the hands and face
–____– sausage digits
–Skin hyperpigmented, telangiectatic
–Generalized thickening portends a worse prognosis
–CREST syndrome – .... |
|
Definition
plaque
Early
Mature
trunk
en coupe de sabre******
Acroscerlosis
Sclerodactyly
Calcinosis, Raynaud’s syndrome, Esophageal dysmotility, Sclerodactyly, and Telangiectasia |
|
|
Term
Scleroderma/Morphea
•Differential diagnosis
–_________
•Women get this in vaginal area
–_________
•Diabetics get this – hard plaque yellow on chin
•No good treatment – can try steroids
–Myxedema
•Hypothyroidism
•Skin feels more doughy than hard
–Scleromyxedema
•Mucin deposition in the skin
•Serum monoclonal immunoglobulin G (IgG) protein (7S)
–MCTD (mixed connective tissue disorder)
•Overlap syndrome with features of several collagen vascular diseases
•Anti-RNP antibodies
–Graft-versus-host disease
–PCT
–Bleomycin
–Nephrogenicfibrosingdermopathy
•Seen in patients with renal insufficiency that have had gadolinium dye exposure |
|
Definition
LSA (lichen sclerosis et atrophicus)
NLD (necrobiosis lipoidica)
"these are what she told us to know" |
|
|
Term
Scleroderma/Morphea
•Laboratory and Biopsy
–CBC, UA, renal function tests, CXR, pulmonary function tests, barium swallow
–_____ positive in 95% of patients with PSS
–_______antibodies – CREST
–_______ antibodies – diffuse slceroderma
–Biopsy
•Thickened dermis
•Increased fibroblasts
•Increased size and number of collagen bundles |
|
Definition
|
|
Term
Scleroderma/Morphea
•Therapy
–Topical, intralesional and systemic steroids
–PSS
•_______ – prevents cross linking of collagen
–DOC
–Requires monthly _____ & ______
»Aplastic anemia and renal insufficiency
•Prednisone, azathrioprine, methotrexate – immunosuppressants
•Physical therapy
–Raynaud’s
•_______ |
|
Definition
D-penicillamine
CBC and UA
Calcium channel blockers |
|
|
Term
Scleroderma/Morphea
•Course and complications
–_____ eventually softens but can take years so give them tx
•Residual hypo- and hyperpigmentation common
–____
•5-year survival rate of 50-90% depending on visceral involvement
•Renal failure and susbsequent HTN common cause of death
•Pumlonary fibrosis
•Malnutrition from esophageal complications
•Cutaneous ulcerations and ischemia |
|
Definition
|
|
Term
Scleroderma/Morphea
•Pathogenesis
–Immunologically mediated
–Possible link to __________
•Treated in Europe with some success
•American studies have not confirmed its role |
|
Definition
|
|
Term
Granuloma Annlare
•Defintion
–____ skin lesion
–Dermal papules forming annular plaques
–Most common on ____ & _____
–Center becomes ____ |
|
Definition
Asymptomatic
dorsal hands and feet
depressed |
|
|
Term
Granuloma Annlare
•Incidence
–Most common in ___ & ____
–
|
|
Definition
children and young adults
> |
|
|
Term
Granuloma Annlare
•History
–May be mistaken for ____ |
|
Definition
|
|
Term
Granuloma Annlare
•Physical Examination
–Shiny ___ papules and ___ plaques that are centrally depressed
–May be skin-colored, violaceous, or erythematous
–Other variants
•_____
•_____ |
|
Definition
dermal
annular
Subcutaneous
Perforating |
|
|
Term
Granuloma Annlare
•Differential diagnosis (3)
|
|
Definition
|
|
Term
Granuloma Annlare
•Laboratory
–____ associated with generalized GA
–Rare association with____ |
|
Definition
|
|
Term
Granuloma Annlare
•Biopsy
–Necrobiosis (degenerative collagen) in the dermis
–Histiocytic
and _______ cell infiltrate
|
|
Definition
|
|
Term
Granuloma Annlare
•Therapy
–Topical and intralesional ___
–____ every 4 weeks
–___ & ___
|
|
Definition
steroids
Liquid nitrogen
PUVA and hydroxychloroquine |
|
|
Term
Granuloma Annlare
•Course and complications
–75% of cases resolve after __years |
|
Definition
|
|
Term
Lichen sclerosis et atrophicus
•Definition
–___ inflammatory condition
–Sclerotic ___ plaques due to thickening of the superficial dermis with overlying, thin, ___ epidermis
–Major ____
–Now called ________ |
|
Definition
Chronic
white
wrinkled
pruritus
lichen sclerosis |
|
|
Term
Lichen sclerosis et atrophicus
•Incidence
–Females ___ males |
|
Definition
|
|
Term
Lichen sclerosis et atrophicus
•History
–Non-genital skin _____ but can be dry and pruritic
–Intractable pruritus with ____ involvement
–May appear as ____in young females |
|
Definition
asymptomatic
genital
bruises |
|
|
Term
Lichen sclerosis et atrophicus
•Physical examination
–Non-genital skin
•____ & ___
•Initial lesions are ___
–Genital skin
•Erythema evolves into ___ sclerotic plaque that may erode and scar
•Men - ____ |
|
Definition
Trunk and extremities
guttate - drop like
hypopigmented
phimosis |
|
|
Term
Lichen sclerosis et atrophicus
•Differential diagnosis
–Non-genital skin – ___
–Genital skin – ____
–____
|
|
Definition
morphea
sexual abuse
Early SCC (erythroplasia of Queyrat) |
|
|
Term
Lichen sclerosis et atrophicus
•Laboratory and Biopsy
–Not associated with ___ disease
–Smudgy collagen in the superficial dermis with flattened DE junction
|
|
Definition
|
|
Term
Lichen sclerosis et atrophicus
•Therapy
–____ for genital skin – must slowly taper
–____ for generalized disease |
|
Definition
Ultrapotent steroids
Acitretin |
|
|
Term
NecrobiosisLipoidica
•Definition
–Well-circumscribed, _____ plaques with a ____ center most common on pre-___ region
–Strong association with ___ |
|
Definition
yellowish
yellow
tibial
diabetes |
|
|
Term
NecrobiosisLipoidica
•Incidence
–Female:male ratio ___:___
–____
|
|
Definition
|
|
Term
NecrobiosisLipoidica
•History
–____ evolves
May rarely ulcerate and become painful
|
|
Definition
|
|
Term
NecrobiosisLipoidica
•Physical examination
–________ (early) papule that evolves into a plaque
–Center becomes atrophic and takes on a ____ hue
–Prominent ____
–_____ border
–Firm
–
|
|
Definition
Brownish-red
yellowish
telangiectasias
Elevated
symmetric |
|
|
Term
NecrobiosisLipoidica
•Differential diagnosis (2)
|
|
Definition
|
|
Term
NecrobiosisLipoidica
•Laboratory
–________ in 2/3 patients
|
|
Definition
|
|
Term
NecrobiosisLipoidica
•Biopsy
–Layered granulomatous inflammation in the dermis that is parallel to the dermis
–Connective tissue _____
–______
|
|
Definition
|
|
Term
NecrobiosisLipoidica
•Therapy
–____ cessation
–Topical and intralesional ___
–______ - slow response to this treatment
|
|
Definition
Smoking
steroids
Pentoxifylline (trental)–> "allows for the plateles to squeeze through things a little better – helps blood go to the area" |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Sarcoid - can mimic granuloma annlare |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Lichen sclerosis et atrophicus |
|
|
Term
|
Definition
| Lichen sclerosis et atrophicus - genital |
|
|
Term
|
Definition
|
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