CM: Cutaneous Clues to Systemic Dz Flashcards
How can discoid lupus (chronic cutaneous lupus) be recognized based on the skin manifestations?
disc shaped plaques - may be red and scaly initially but progresses to plaques with hyperpigmented borders and pink, atrophic, scarred centers
can cause alopecia
only small percentage gets SLE, rest just have skin
What is generalized discoid lupus?
extensive lesions on trunk and arms
associated w increased risk of SLE
How can subacute cutaneous lupus be recognized based on the skin manifestations?
annular or psoriasiform variants
erythematous, scaly plaques on sun-damaged skin (associated w Ro/SS-A autoantibodies)
may have inverted triangle on chest, photosensitivity, polycyclic lesions, sparing of knuckles, involvement of side of face rather than malar area
non-scarring
half get SLE, only 10% severe
What are some differences in features of SCLE and other similar diseases?
not usually periungual changes like in dermatomyositis
ACLE is photosensitive, otherwise symptoms do not apply to ACLE or DLE
How can acute cutaneous lupus be recognized?
classic malar rash in sick pts w systemic lupus
What antibodies are present in the different types of cutaneous lupus?
DLE: only 5% have significant ANA titer (1:160 or higher)
SCLE: 60-80% w ANA, can have Ro Ab w neg ANA
ACLE: virtually 100% have ANA
What are skin findings that can help diagnose dermatomyositis?
Gottron’s papules - violacious over knuckles
Gottron’s sign - violacious macular erythema over bony prominence (elbows and knees)
Heliotrope rash, shawl sign, tendon streaking of hands, ragged nail cuticles w periungual telangiectasias
What are systemic findings of dermatomyositis?
proximal extremity weakness (increased CPK), interstitial pneumonitis (Jo-1 and PC-1 antibodies in serum), Mechanic’s hands skin lesion = rough, scaly fissured fingers correlates w pulmonary dermatomyositis
esophageal dysfunction, arthritis, cardiac (rare)
What is an important association of dermatomyositis?
malignancy
25% of cases associated w internal malignancy
higher risk of ovarian carcinoma in females
higher risk of nasopharyngeal cancers in Asians
most diagnosed w/i 3 yrs of DM diagnosis - can arise before or after rash appears
What are the different scleroderma syndromes?
morphea - skin-only
systemic - diffuse cutaneous or limited cutaneous (CREST)
What is morphea?
hard bound down plaques, borders often red indicating active dz, no systemic involvement
localized, linear, and generalized types
What are some cutaneous features of diffuse systemic sclerosis?
beak like nose, decreased oral aperture, sclerodactyly, raynaud’s, telangiectasias, salt and pepper changes, calcinosis cutis (pathognomonic), digital ulceration
Which organ systems are involved in diffuse systemic sclerosis?
CV, renal (oliguric RF due to decreased blood flow), GI tract, joints, lung (bibasilar pulm fibrosis, interstitial lung dz)
antitopoisomerase-1 (Scl-70) Ab in 30%
What features are present in limited cutaneous systemic sclerosis (CREST)?
calcinosis cutis (firm, flesh colored papules sometimes exuding white, chalky material)
raynauds
esophageal dysmotility
sclerodactyly
telangiectasias (mat-like, over face, upper chest, extremities)
associated w anti-centromere Ab
smaller chance of pulmonary HTN w or w/o ILD
What are skin signs of diabetes?
candidiasis on mucosal surfaces
tinea - superficial dermatophyte fungus affects skin, nails, hair - treat to prevent foot ulcers
staph inf
What are the different staph inf associated w diabetes?
folliculitis - follicular papules/pustules on back, buttocks, scalp, lower extremities
furuncles (boils) - deep nodular inf around hair follicle
carbuncle - >1 interconnected and draining furuncles
impetigo - superficial inf, erythema w honey colored crusts
pyoderma - larger, deeper inf w pustules and crusting
cellulitis - diffuse dermal inf - redness and swelling
What are other cutaneous findings in Diabetes?
diabetic dermopathy necrobiosis lipoidica diabeticorum granuloma annulare acanthosis nigricans diabetic bullae
How can diabetic dermopathy be recognized?
hyperpigmented, slightly atrophic plaques seen primarily over shins
How can necrobiosis lipoidica diabeticorum be recognized?
erythematous, indurated plaques that progress to form yellow atrophic plaques w telangiectasias
occur on anterior and lateral distal legs
lesions may ulcerate
How can granuloma annulare be recognized?
dermal ring-shaped papules or plaques
usually distal extremities, hands, or feet
generalized more strongly associated w diabetes
collection of tissue macrophages (palisading granulomas) surrounding pocket of mucin
How can acanthosis nigricans be recognized?
hyperpigmented, velvety plaques
marker of insulin resistance
associated w obesity, DM, and rarely autoimmune and malignancies
What are diabetic bullae?
tense, non-inflammatory bullae, acrally distributed, resolve spontaneously in 2-6 wks w/o scarring
What are skin signs of hyperthyroidism?
fine thin hair may progress to alopecia
fine velvety skin w increased warmth, sweating
palmar erythema
Plummer’s nails - free edge curves upward
What is the classic triad of Grave’s dz?
ophthalmopathy, pretibial myxedema, thyroid acropachy (clubbing, soft tissue swelling of hands and feet, periosteal new bone formation)
What are skin signs of hypothyroidism?
cold, pale, dry skin dull, coarse hair loss of lateral 1/3 of eyebrows generalized myxedema broad nose, puffy face, thick lips, large tongue, droopy eyelids vitiligo
What is vitiligo?
autoimmune dz - melanocytes are targeted, face and hands
hypothyroidism in up to 40%
small associations w Addisons and diabetes
What is erythema nodosum?
tender erythematous nodules over shins
a panniculitis (inflammation of the fat)
histiocytes and lymphocytes make up inflammatory infiltrate
lasts days to wks, more than 1/3 have no underlying cause
What are the dz associations of erythema nodosum?
IBD (UC, Crohns), sarcoid, Behcet’s, inf (strep, tb, fungus, mono, hep B), drugs (sulfonamides, bromides, OCs), pregnancy, hematologic malignancy, carcinoma
What is pyoderma gangrenosum?
ulcer w violaceous, undermined border and purulent dirty base
lesions often precipitated by trauma
responds to immunosuppressives but must rule out inf
What are the dz associations of pyoderma gangrenosum?
half have malignancy (hematologic, monoclonal IgA gammopathy), IBD, RA
What is Sweet’s syndrome?
red to purplish tender papules/nodules or plaques w pronounced edema of papillary dermis resulting in mamillated "juicy" surface fever, myalgias, leukocytosis preceded by URI or flu-like illness resolves w/i 5-12 wks upper extremities, face, neck
What are the dz associations of Sweet’s syndrome?
IBD, hematologic malignancies (AML), solid tumors, strep or yersinia inf, granulocyte colony stimulating factor, pregnancy
What is leukocytoclastic vasculitis?
rxn to immune complex depo around blood vessels
extravasation of RBCs leads to palpable purpura
neutrophils in vessel walls, thrombis in lumen, fibrin in wall, leukocytoclasis (neutrophil fragmentation)
What are the dz associations of leukocytoclastic vasculitis?
cryoglobulinemic vasculitis, HSP
Autoimmune connective tissue dz: RA, SLE, SS
Wegeners, PN
inf: strep, endocarditis, mycoplasma, flu
drugs: *beta lactam abx = most common cause
malignancies: hematologic (lymphoma, leukemia, monoclonal gammopathies), solid tumor
What is cryoglobulinemic vasculitis?
immune globulins precipitated due to cold exposure
associated w hep C - palpable purpura on lower extremities (LCV), arthritis and glomerulonephritis
What is HSP?
IgA vasculitis, arthritis, ab pain, nephritis
