CM: Cutaneous Clues to Systemic Dz

Question 1

How can discoid lupus (chronic cutaneous lupus) be recognized based on the skin manifestations?

Answer 1

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

Question 2

What is generalized discoid lupus?

Answer 2

extensive lesions on trunk and arms

associated w increased risk of SLE

Question 3

How can subacute cutaneous lupus be recognized based on the skin manifestations?

Answer 3

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

Question 4

What are some differences in features of SCLE and other similar diseases?

Answer 4

not usually periungual changes like in dermatomyositis

ACLE is photosensitive, otherwise symptoms do not apply to ACLE or DLE

Question 5

How can acute cutaneous lupus be recognized?

Answer 5

classic malar rash in sick pts w systemic lupus

Question 6

What antibodies are present in the different types of cutaneous lupus?

Answer 6

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

Question 7

What are skin findings that can help diagnose dermatomyositis?

Answer 7

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

Question 8

What are systemic findings of dermatomyositis?

Answer 8

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)

Question 9

What is an important association of dermatomyositis?

Answer 9

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

Question 10

What are the different scleroderma syndromes?

Answer 10

morphea - skin-only

systemic - diffuse cutaneous or limited cutaneous (CREST)

Question 11

What is morphea?

Answer 11

hard bound down plaques, borders often red indicating active dz, no systemic involvement
localized, linear, and generalized types

Question 12

What are some cutaneous features of diffuse systemic sclerosis?

Answer 12

beak like nose, decreased oral aperture, sclerodactyly, raynaud’s, telangiectasias, salt and pepper changes, calcinosis cutis (pathognomonic), digital ulceration

Question 13

Which organ systems are involved in diffuse systemic sclerosis?

Answer 13

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%

Question 14

What features are present in limited cutaneous systemic sclerosis (CREST)?

Answer 14

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

Question 15

What are skin signs of diabetes?

Answer 15

candidiasis on mucosal surfaces
tinea - superficial dermatophyte fungus affects skin, nails, hair - treat to prevent foot ulcers
staph inf

Question 16

What are the different staph inf associated w diabetes?

Answer 16

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

Question 17

What are other cutaneous findings in Diabetes?

Answer 17

diabetic dermopathy
necrobiosis lipoidica diabeticorum
granuloma annulare
acanthosis nigricans
diabetic bullae

Question 18

How can diabetic dermopathy be recognized?

Answer 18

hyperpigmented, slightly atrophic plaques seen primarily over shins

Question 19

How can necrobiosis lipoidica diabeticorum be recognized?

Answer 19

erythematous, indurated plaques that progress to form yellow atrophic plaques w telangiectasias
occur on anterior and lateral distal legs
lesions may ulcerate

Question 20

How can granuloma annulare be recognized?

Answer 20

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

Question 21

How can acanthosis nigricans be recognized?

Answer 21

hyperpigmented, velvety plaques
marker of insulin resistance
associated w obesity, DM, and rarely autoimmune and malignancies

Question 22

What are diabetic bullae?

Answer 22

tense, non-inflammatory bullae, acrally distributed, resolve spontaneously in 2-6 wks w/o scarring

Question 23

What are skin signs of hyperthyroidism?

Answer 23

fine thin hair may progress to alopecia
fine velvety skin w increased warmth, sweating
palmar erythema
Plummer’s nails - free edge curves upward

Question 24

What is the classic triad of Grave’s dz?

Answer 24

ophthalmopathy, pretibial myxedema, thyroid acropachy (clubbing, soft tissue swelling of hands and feet, periosteal new bone formation)

Question 25

What are skin signs of hypothyroidism?

Answer 25

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

Question 26

What is vitiligo?

Answer 26

autoimmune dz - melanocytes are targeted, face and hands
hypothyroidism in up to 40%
small associations w Addisons and diabetes

Question 27

What is erythema nodosum?

Answer 27

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

Question 28

What are the dz associations of erythema nodosum?

Answer 28

IBD (UC, Crohns), sarcoid, Behcet’s, inf (strep, tb, fungus, mono, hep B), drugs (sulfonamides, bromides, OCs), pregnancy, hematologic malignancy, carcinoma

Question 29

What is pyoderma gangrenosum?

Answer 29

ulcer w violaceous, undermined border and purulent dirty base
lesions often precipitated by trauma
responds to immunosuppressives but must rule out inf

Question 30

What are the dz associations of pyoderma gangrenosum?

Answer 30

half have malignancy (hematologic, monoclonal IgA gammopathy), IBD, RA

Question 31

What is Sweet’s syndrome?

Answer 31

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

Question 32

What are the dz associations of Sweet’s syndrome?

Answer 32

IBD, hematologic malignancies (AML), solid tumors, strep or yersinia inf, granulocyte colony stimulating factor, pregnancy

Question 33

What is leukocytoclastic vasculitis?

Answer 33

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)

Question 34

What are the dz associations of leukocytoclastic vasculitis?

Answer 34

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

Question 35

What is cryoglobulinemic vasculitis?

Answer 35

immune globulins precipitated due to cold exposure

associated w hep C - palpable purpura on lower extremities (LCV), arthritis and glomerulonephritis

Question 36

What is HSP?

Answer 36

IgA vasculitis, arthritis, ab pain, nephritis