Cutaneous Manifestations Of Systemic Disease Flashcards
What are the major forms of cutaneous lupus?
Acute cutaneous lupus (ACLE)- strongly associated w SLE, butterfly rash Subacute cutaneous (SCLE) Chronic cutaneous (CCLE)
What are the characteristics of subacute cutaneous lupus?
Associated with HLA-B8( strongest);HLA-DR3
Associated with complement deficiencies
Anti-RO/SS-A antibodies presence
80% are ANA positive
Located on lateral face, neck,v-chest, upper back, extremities
What are the characteristics of chronic cutaneous lupus?
Discoid lupus accounts for majority
Less likely to be ANA positive (25%)
What are the characteristics of SLE?
Affects more female than male
African Americans have 4 times more incidence
Criteria= MD SOAP BRAIN
99% ANA positive- sensitive but most specific
dsDNA positive- not sensitive but specific, used for monitoring disease
Anti smith antibodies
Associated with HLA-DR2, HLA-DR3
What are the characteristics of drug induced lupus?
95% are anti histone antibodies positive
Risk factors: procainamide, hydralazine
What are the characteristics of localized scleroderma?
Morphea is the most common
Positive anti topoisomerase 2 antibodies
Lack anti scl-70 and anti centromere antibodies
What are the characteristics of systemic sclerosis?
Limited cutaneous systemic sclerosis
CREST syndrome
Positive anti centromere antibodies
What are the characteristics of diffuse progressive systemic sclerosis?
Associated with more severe visceral disease and has a worse prognosis
Raynauds phenomenon might be 1st manifestation
Positive anti SCL-70 antibodies
What are the signs of dermatomyositis?
Heliotrop rash- Discoloration around eyes
Gottrons papules- erythematous papules over interphalanx joints
Samitz sign- dry, ragged cuticles
Shawl sign- Telangiectasia/poikiloderma,upper chest, shoulder
Symmetrical proximal muscle weakness
Mechanic’s hand
Associated with malignancy
What are the labs findings of dermatomyositis?
40% are ANA positive
CK more sensitive marker of muscle involvement (>1000)
Triceps muscle biopsy is gold standard
Anti Mi2- associated with shawl sign,cuticular changes and good prognosis
Anti Jo1 antibody specific for dermatomyositis
What are the characteristics of Peutz-Jeghers syndrome?
Pigmented mucocutaneous macules
GI polyps most common in jejunum and ileum leading to intussception (MC), GI bleed
93% develop cancer before the age of 65 (GI most common )
What are the characteristics of Gardner syndrome?
Aka familial colorectal polyposis (FCP)
Autosomal dominant due to mutation in APC gene ( tumor suppressor gene)
Premalignant polyposis throughout GI tract leads to increased risk of GI adenocarcinoma
Desmoid tumors are locally agressive but do not metastasize
Osteoma in skull,mandible, maxilla in 80% of patients
What are the characteristics of Pyoderma Gangrenosum?
Associated with IBD (MC), AML,CML, Hairy Cell Leukemia, Polycythemia Vera
What are the characteristics of Porphyria Cutanea Tarda?
Most common porphyria
Due to decreased hepatic uroporphyrinogen decarboxylase (UROD) activity
Triggers are alcohol abuse, hemochromatosis, hepatitis C
What are some diabetic skin manifestations?
Acanthosis Nigricans- indicate insulin resistance/ diabetes
Necrobiosis Lipoidica Diabeticorum (NLD)- atrophic yellow orange patches with red border and Telangiectasia: 30% of NLD have diabetes but only 0.3% of diabetics have NLD
Recurrent candidiasis
Bullous diabeticorum
