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Multisystems > CM- Cutaneous Clues to Systemic Disease > Flashcards

CM- Cutaneous Clues to Systemic Disease Flashcards

1
Q

Dermatomyositis is associated with a higher incidence of what?

A

Carcinoma

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2
Q

What underlying disease is pyoderma gangrenosum associated with?

A

IBD

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3
Q

What are the 3 “lupus specific” skin signs?

A
  1. discoid lupus erythematosus [DLE]
  2. subacute cutaneous lupus erythematosus [SCLE]
  3. Acute cutaneous lupus erythematosus [ACLE]
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4
Q

A patient presents with disc-shaped plaques on the head and neck. They were red and scaly initially, but now they have progressed to plaques with hyperpigmented borders and pink atrophic, scarred centers.
What type of lesion is this?
What percent of patients with these lesions will develop systemic disease?

A

This description is of discoid lupus erythematosus [DLE].

Only 5% will progress to systemic lupus. The remaining 95% will just have skin disease.

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5
Q

Describe what you would see on dermatologic exam of someone with generalized DLE.
What is the implications of generalized DLE as compared to localized DLE?

A

disc-shaped plaques that were originally red/scaly but now are plaques with hyperpigmented edges and pink, scarred centers. Instead of just being on the head and neck like DLE, it will also involve the trunk and arms.

It has an increased risk of SLE compared to head and neck.

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6
Q

What are the 2 variants of subacute cutaneous lupus erythematosus?
How do both appear?

A
  1. annular
  2. psoriasiform

Both appear as erythematous, scaly plaques on sun-damaged skin

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7
Q

SCLE is associated with the development of what antibody?

A

Ro/SS-A autoantibodies

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8
Q

On dermatology exam, you note a patient with erythematous scaly plaques on sun-damaged skin. It is in an inverted triangle pattern on the chest, It demonstrates photosensitivity.
It is polycyclic, spares the knuckles [but involves the area between knuckles on the fingers] and involves the side of the face.
There are NO periungual changes [around the fingernails].

What are these findings characteristic for?
What symptom can also be seen in another disease related to this one?
What percent of patients that present with this will develop systemic disease?

A

These findings are characteristic for SCLE.

ACLE has photosensitivity too, but all the other symptoms were specific to SCLE.

50-60% of SCLE will develop SLE, but only 10% will be severe

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9
Q

A patient presents with a malar rash [macular erythema on cheeks, nose, forehead and chin but sparing nasolabial folds. It is photo-sensitive.
What does this person have?

A

ACLE [acute cutaneous lupus erythematosus]

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10
Q

What is the average ANA antibody titer for patients with:

  1. DLE
  2. SCLE
  3. ACLE
A 
DLE = 5% 
SCLE = 60-80% with ANA, but can have a positive Ro/SSA with negative ANA
ACLE = 100% with ANA titer
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11
Q

What 2 signs are pathognomonic for dermatomyositis?

A
  1. Gottron’s papules- violaceous papules over the knuckles of the hand
  2. Gottron’s sign- violaceous macular erythema over any bony prominence
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12
Q

You are examining a patient and notice violaceous papules over the knuckles. He has macular erythema around the eyes on on the shoulders/upper back.
His hands show tendon streaking with violaceous macular erythema and he has ragged nail cuticles with periungual telangiectasias.

What disease does this man have?
Identify which 2 signs are pathognomonic.
What are the “characteristic” but not necessarily pathognomonic skin signs?

A

Dermatomyositis

Pathognomonic:
1. Gottron’s papules and sign

Characteristic:

  1. Heliotrope rash = macular erythema around the eyes
  2. “shawl sign” = macular erythema on shoulder’s/upper back
  3. tendon streaking on hands
  4. ragged nail cuticles
  5. periungual telangiectasia [differentiates dermatomyostitis from SCLE]
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13
Q

What are the 2 main systemic findings associated with dermatomyositis?

A
  1. prox. extremity weakness [increased CPK]

2. interstitial pneumonitis [Jo-1 Ab, PC-1 Ab]

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14
Q

What skin disease is the Mechanic’s Hands Skin Lesion associated with?
What underlying disease does it correlate with?

A

Mechanics Hand Skin Lesion is associated with dermatomyositis [rough, scaly, fissured fingers].

The presence of this correlates with pulmonary dermatomyositis.

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15
Q

What % of cases of dermatomyositis in adults are associated with internal malignancy?
What malignancy is there a disproportionate increased risk of?

A

25% of cases are associated with internal malignancy [ovarian, breast, lung, colon, gastric, uterine]

Disproportionate risk of ovarian carcinoma in women.

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16
Q

If a patient has dermatomyositis, what screening tests must be run yearly?

A
  1. Hx & PE
  2. mammography
  3. colonoscopy
  4. chest x-ray
  5. PAP and pelvic exam
  6. CT of ab and pelvis
  7. prostate exam/PSA
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17
Q

What is morphea?

A

skin-only scleroderma [hard sclerotic skin] without systemic involvement.
It presents with hard-indurated plaques with active red/violaceous borders

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18
Q

What are the 2 subtypes of systemic sclerosis?

A
  1. diffuse

2. limited [formerly CREST syndrome]

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19
Q

A patient presents with a beak-like nose, proximal scleroderma, sclerodactyly, digital pitted scars, “salt-and-pepper changes”, decreased oral aperture, nail fold capillary dilation, Reynaud’s, telangiectasias, and calcinosis cutis.
What are these the cutaneous signs for?
What organs are involved in systemic disease?
What Ab is in 30% of cases?

A

This is diffuse systemic sclerosis.

Organ systems involved:

  1. cardio
  2. GI
  3. Renal [oliguric renal failure]
  4. Joints
  5. Lungs [bibasilar pulm fibrosis, interstitial lung disease]

Antitopoisomerase-1 [Scl-70] Ab in 30% of cases

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20
Q

What are the symptoms of limited cutaneous systemic sclerosis?

A 
CREST Syndrome
Calcinosis cutis
Reynaud's 
Esophageal dysmotility
Sclerodactyly 
Telangiectasias
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21
Q

Patient A presents with mat-like sclerosis over her face, upper chest and extremities. She lacks proximal sclerosis. She has trouble swallowing. She also has calcium deposits on her skin, as long as sclerodactyly and telangiectasias. When she gets cold, her extremities go white, blue, then red due to constriction of blood flow.
What does this woman have?
What Ab is it associated with 70-80% of the time?
What percent will have pulmonary hypertension [with or without interstitial lung disease]?

A

She has limited cutaneous systemic sclerosis.
Ab = anticentromere
10-15% will have pulmonary HTN

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22
Q

What yeast infection is most common in diabetics? What 3 sites are most common for cutaneous infection?

A

Candida-

  1. axillae
  2. scrotum
  3. skin folds
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23
Q

What 2 fungal infections are diabetics most at risk for?

What are the negative effects of each?

A
  1. Tinea pedis/unguium - negative effect is that is serves as an entry point for serious bacterial infections and can lead to gangrene
  2. Mucormycosis- potentially fatal
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24
Q

What 4 bacterial infections are diabetics predisposed to?

A
  1. Staphylococcal [folliculitis, furuncle, carbuncle]
  2. pyoderma
  3. impetigo
  4. cellulitis
25
Q

What is a furuncle and carbuncle?

What bacterial infection leads to this?

A

Staph leads to furuncles and carbuncles.
Furuncle = boil = deep nodular infection around a hair follicle
Carbuncle = more than one interconnected and draining follicle

26
Q

Describe diabetic dermopathy.

In what percent of patients does is present?

A

It is hyperpigmented, slightly atrophic plaques primarily over the shins

30-60% of diabetics

27
Q

Describe necrobiosis lipoidica diabeticorum.

A

erythematous, indurated plaques that progress to yellow atrophic plaques with telangiectasias on anterior and lateral distal legs.

*may or may not ulcerate

28
Q

A diabetic patient presents with dermal ring-shaped papules or plaques on their distal extremities.
What does this describe and what would you see on microscopy?

A

Granuloma annulare - collections of tissue macrophages surrounding a pocket of mucin

29
Q

A type 2 diabetic presents with hyperpigmented, velvety plaques on their neck, axillae and knuckles. What is this?
What is it a sign of?
What are the 3 main associations with is?

A

Acanthosis Nigricans
- sign of insulin resistance

Associated with:

  1. diabetes
  2. obesity
  3. autoimmune/malignancy
30
Q

Where do diabetic bullae tend to form?

How long does it take for resolution?

A

Acrally [head, neck, extremities]

2-6 weeks for spontaneous resolution with no scarring

31
Q

What are two signs seen in diabetics that show vascular insufficiency involving the lower extremities?
What is the main sign that peripheral neuropathy is present?

A

Vascular insufficiency:

  1. cool skin
  2. sparse hair

Neuropathy:
1. cutaneous ulcerations on the feet

32
Q

A patient presents with fine thin hair and fine velvety skin. She is warm and moist to touch and is sweating. She has palmar erythema. The free edge of her fingernails curve upwards. What does this woman have?

A

hyperthyroidism

33
Q

What patients present with Plummer’s nails?

What does it look like?

A

Hyperthyroidism patients present with Plummer’s nails. The free edge of the nail curves upward

34
Q

What is the “classic triad” for Grave’s disease specific skin changes? What are these changes due to?

A
  1. ophthalmopathy- exophtkalmos, proptosis
  2. pretibial myxedema- shiny red plaques and nodules on the anterior shins
  3. thyroid acropachy- clubbing, soft tissue swelling of hands and feet, new bone formation

These are due to mucopolysaccharide deposition

35
Q

A patient presents with cold, pale, dry skin. Her skin has an orange tint [carotenemia]. Her hair is dull and coarse and she has lost the lateral 1/3 of her eyebrows. She has generalized myxedema, broad nose, puffy face, thick lips, large tongue and droopy eyelids. What does this patient likely have?

A

Hypothyroidism

36
Q

What is vitiligo?
How does it present?
What areas of the body are favored?

A

Autoimmune disease where melanocytes are targeted.

It presents as de-pigmented macules [white flat spots]. It favors the hands and face.

37
Q

What 2 diseases do people with vitiligo develop?

What disease can lead to the development of vitiligo?

A

Vitiligo can develop into:

  1. hypothyroidism [40%]
  2. Addison’s [2%]

Diabetics can develop vitiligo

38
Q

What is a reaction pattern?

A

Dermatosis [skin reaction] with distinct clinicopathology as a result of:

  1. systemic illness
  2. hypersensitivity
  3. infection
39
Q

What is erythema nodosum?
How does it present?
What do you see histologically?

A

It is panniculitis [inflammation of the fat] that presents as tender, red nodules over BILATERAL shins. It can be associated with fever, arthralgias, malaise.

Histologically:
Septal panniculitis with histiocytes and lymphocytes

40
Q

What 2 enteropathies present with erythema nodosum?

A
  1. Crohn’s

2. Ulcerative colitis

41
Q

What autoimmune conditions present with erythema nodosum?

A
  1. Sarcoidosis [also uveitis, arthritis]

2. Bechet’s syndrome

42
Q

What infections are associated with erythema nodosum?

3 bacterial, 2 fungal, 2 viral

A
  1. Strep
  2. yersinia,salmonella, mycoplasma pneumo, leprosy, leptospirosis, tularemia
  3. TB
  4. Deep Fungal infections
  5. dermatophytosis
  6. mono
  7. Hep B
43
Q

What 3 drugs are associated with erythema nodosum?

A
  1. OCPs
  2. sulfonamides
  3. bromides
44
Q

What malignancies are associated with erythema nodosum?

A
  1. hematologic [lymphoma, leukemia, myelodysplastic]

2. carcinomas

45
Q

A patient presents with an ulcer with violaceous , undermined border and a purulent dirty base on his lower extremities. The doctor thinks it is an infection and sends him into surgery for debridement of the ulcer. But it gets much worse!
What is the most likely cause of the ulcer?
What precipicates these lesions?
What is the treatment?

A

Pyoderma gangrenosum- lesion is usually precipitated by trauma.

PG usually responds to immunosuppressives, but make sure it is not an infection first!!

46
Q

What are 5 causes of pyoderma gangrenosum?

A
  1. Crohns
  2. Ulcerative colitis
  3. RA
  4. Monoclonal IgA gammopathy
  5. hematologic malignancy
47
Q

A patient presents with red/purple papules and nodules that form a mamillated surface [mountain range appearance]. It is circular, annular or arcuate.
The lesions are tender to touch, but will resolve without scarring. It affects the upper extremities, face and neck.
When she presented she had fever, myalgia and leukocytosis.

What is the syndrome?
What are the ocular manifestations?
What is the histology?

A

Sweet’s syndrome
- iritis, episcleritis, conjunctivitis

Histology will show:

  1. neutrophils infiltrating the dermal layer
  2. papillary dermal edema [hence the mamillated appearance]
48
Q

What are the 6 major causes of Sweet’s syndrome?

A
  1. Enteropathies [Crohns, UC]
  2. Medications [granulocyte colony stimulating factor]
  3. Infections [Strep, Yersinia]
  4. Solid tumors
  5. Hematologic malignancies [AML]
  6. Pregnancy
49
Q

What is leukocytoclastic vasculitis?
What is seen on histology?
What 2 things is it associated with?

A

It is when immune complexes deposit around blood vessels and then neutrophils infiltrate the blood vessels. Damage from inflammation leads to palpable purpura.

Histology:

  1. neutrophils in the vessel walls
  2. fibrin in vessel walls
  3. thrombus in vessel lumina
  4. neutrophil fragments [leukocytoclasis] around the vessel

Associated with:

  1. Cryoglobinemic vasculitis
  2. Henoch Schonlein Purpura
50
Q

What is a cryoglobin? What conditions is it seen with?

A

It is when Igs precipitate in the cold.

  1. RA
  2. malignancy
  3. Hep C
51
Q

How does cryoglobinemia vasculitis present in patients with Hep C?

A
  1. palpable purpura of lower extremities [leukocytoclastic vasculitis]
  2. arthritis
  3. glomerulonephritis
52
Q

What are the 4 presenting symptoms of a patient with Henoch Schonlein purpura?

A
  1. IgA leukocytoclastic vasculitis
  2. arthritis
  3. abdominal pain
  4. nephritis
53
Q

What 3 autoimmune connective tissue disease are associated with Henoch Schonlein purpura?

A
  1. RA
  2. SLE
  3. Sjogrens
54
Q

What 2 systemic vasculitides are associated with henoch schonlein purpura?

A
  1. Wegener’s

3. polyarteritis nodosa

55
Q

What 4 infections are associated with Henoch Schonlein purpura?

A
  1. strep
  2. endocarditis
  3. mycoplasma
  4. influenza
56
Q

What drugs are associated with HSP?

A

Beta Lactam antibiotics

57
Q

What is the most common cause of leukocytoclastic vasculitis?

A

Beta Lactam antibiotics

58
Q

What malignancies are associated with HSP?

A
  1. Hematologic [lymphoma, leukemia, monoclonal gammopathies]

2. solid tumor

Multisystems (29 decks)

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