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👩🏼‍⚕️ ANDREWS' DISEASES OF THE SKIN 13TH EDITION > 8- Connective Tissue Diseases > Flashcards

8- Connective Tissue Diseases Flashcards

1
Q

Young adults
Dull red macules/indurated plaques with adherent scale—evolve with atrophy, scarring, pigment changes

Usually above the neck (scalp, nose, malar, lower lip ears)
Generalized- thorax, upper extremities, head and neck, may have alopecia
95% will remain confined to skin

A

Discoid LE

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2
Q

[Chronic cutaneous LE]
Large lesions, atrophic, hypopigmented, red or pink patches and plaques

Fine telangiectasia and scaling
Extensor/midline back usually affected
Prominent palmoplantar involvement

A

Lupus erythematosus-lichen planus overlap

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3
Q

Evolve as polycyclic annular lesions or psoriasiform plaques
Scale is thin and easily detached
Follicles not involved
Transient/migratory; no scarring
Tend to occur in sun exposed areas-face and neck, chest and back
3/4 have arthritis (+) anti Ro/SSA

A

Subacute cutaneous LE (SCLE)

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4
Q

Drug induced SCLE is most often related to what drug

A

Hydrochlorothiazide

Also: ACE inhibitors, CCB, interferon, anticonvulsant

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5
Q

SLE must have how many of the criteria to diagnose

A

4/11
At least 1 clinical and 1 immunologic

or must have biopsy proven lupus nephritis in the presence of ANA or anti-dsDNA antibodies

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6
Q

SLE criteria

SOAP BRAIN MD

A

Serositis (pleuritis/pericarditis)
Oral ulcers
Arthritis
Photosensitivity

Blood low (leuko, thrombo, anemia)
Renal (protein >0.5g/day or casts)
ANA positive 
Immunologic- dsDNA, anti-Sm
Neurologic- seizures, psychosis

Malar rash
Discoid rash

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7
Q

Difference between bullous lesions of lupus erythematosus (BLE) and epidermolysis bullosa acquisita

A

Dapsone effective in BLE and not in EBA

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8
Q

Earliest changes that may be noted in SLE

May remain the sole symptom for some time

A

Transitory or migratory arthralgia

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9
Q

Most common cause of death in SLE DURING the first 5 years

A

Inflammatory lesions and infections

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10
Q

Most common cause of death in SLE AFTER the first 5 years

A

Thromboses

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11
Q

Most frequent cardiac manifestation in SLE

A

Pericarditis

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12
Q

This sign is a marker for SLE patients at risk for CNS lesions (Sneddon Syndrome)

A

Livedo reticularis

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13
Q

Implicated causes of drig induced LE

HIP SPAM

A

Hydralazine
Isoniazid
Procainamide

Sulfonamides
Penicillin
Anticonvulsants
Minocycline

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14
Q

This test is positive in 95% of cases of SLE

A

ANA

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15
Q

This is specific but not sensitive to SLE

Indicates high risk of renal disease

A

Anti-dsDNA

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16
Q

Immunologic test for SLE that has 10% sensitivity but specificity is very high

A

Anti-Sm

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17
Q

Immunologic test that is common in SCLE and Sjögren

A

Anti-La

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18
Q

(Immunologic test)

More commonly positive in SCLE, neonatal LE, late onset and Asian LE

A

Anti-Ro

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19
Q

Single most effective local treatment in SLE

A

Triamcinolone acetonide 2.5-10mg/mL at 4-6 weeks interval

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20
Q

Safest class of systemic agent for SLE

A

Antimalarials

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21
Q

Drug of choice for bullous systemic LE

A

Dapsone

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22
Q

First line systemic therapy in most forms of cutaneous LE

A

Hydroxychloroquine 6.5mg/kg/day

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23
Q

WOF this side effect of hydroxychloroquine

A

Ocular toxicity

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24
Q

When should Ophthalmology consult be done when administering hydroxychloroquine?

A

Before
After 4mos
After 6mos

25
Q

Side effect of Quinacrine in SLE tx

A

Yellow discoloration-skin and conjunctivae

Blue black discoloration- hard palate, nail bed, ears, sclerae

26
Q

Second line and third line agents for SLE

A

Retinoids

Systemic immunosuppresion

27
Q

Inflammatory myositis and skin disease

Erythema and edema of face and eyelids, upper face—evolve to reticulated white scarring
Extensor surfaces may be similar to psoriasis
Photosensitivity

A

Dermatomyositis

28
Q

Muscle group involved in dermatomyositis

A

Proximal muscle groups

29
Q

Erythema and scale over shoulder area in dermatomyositis is called

A

Shawl sign

30
Q

Erythema and scale over hip area in dermatomyositis is called

A

Holster sign

31
Q

Less common lesions but highly characteristic of dermatomyositis

A

Gottron’s papules

32
Q

In dermatomyositis, there is symmetrical muscle weakness, most commonly seen in

A

Shoulder girdle and pelvic region

Hands

33
Q

Antibody positive in dermatomyositis

A

Anti-Jo-1

34
Q

Diagnostic criteria for dermatomyositis must include:

How many

A

1 skin lesion + 4/8 other criteria

Polymyositis- 4/8 criteria, no skin lesions

35
Q

Criteria for diagnosis of dermatomyositis

A

Skin lesions- heliotrope rash, gottrons papules

Other: 4/8
Proximal muscle weakness 
Elevated CK/ aldolase
Muscle pain 
EMG changes
Positive anti-Jo-1
Arthritis 
Systemic signs (fever, elevated CRP/ESR)
Patho findings of myositis
36
Q

Mainstay of acute treatment for dermatomyositis

A

Prednisone 1mg/kg/day

37
Q

Circumscribed or diffuse, hard, smooth, ivory colored areas that are immobile and give appearance of hidebound skin

Face expressionless, mouth constricted, claw like hands

A

Scleroderma

38
Q

This form of scleroderma is more common in women
Macules or plaques, bands or guttate lesions
Rosy or violaceous — yellowish white

More common on trunk, margins surrounded by lilac border or telangiectases- skin elasticity lost

A

Morphea

39
Q

Linear lesions that extend the length of the arm or leg and may follow lines of Blaschko

Begins during 1st decade of life

A

Linear scleroderma

40
Q

Lesion in scleroderma that occur on the frontal scalp and extend partly down the forehead

A

En coup de sabre

41
Q

Variant of scleroderma that has the most favorable prognosis

A

CREST syndrome

42
Q

CREST syndrome acronym meaning

A 
Calcinosis cutis
Raynaud phenomenon
Eesophageal dysmotility
Sclerodactyly
Telangiectasia
43
Q

First manifestation of progressive systemic sclerosis

A

Raynaud phenomenon

44
Q

Generalized disorder of connective tissue- thickening of dermal collagen bundles, fibrosis and vascular abnormalities in internal organs

“Woody edema” of the hands
3rd-5th decade

A

Progressive systemic sclerosis (PSS)

45
Q

Criteria for progressive systemic sclerosis

A

Proximal sclerosis and 2 or all of ff:

  • sclerodactyly
  • digital pitting scars/ loss of substance of distal finger pad
  • bilateral basilar pulmonary fibrosis
46
Q

90% of PSS patients have this symptom

A

Esophageal involvement

-distal 2/3: dysphagia and reflux esophagitis

47
Q

Mainstay of medical treatment for Raynaud phenomenon

A

Vasodilators- CCB, ARB, topical nitrates and prostanoids

48
Q

First line therapy for Raynaud phenomenon

A

Nifedipine 30-60mg/day

49
Q 
Defined by presence of:
Raynaud phenomenon 
Arthralgia 
Swollen joints
Esophageal dysfunction 
Muscle weakness
Sausage like fingers
A

Mixed Connective Tissue Disease

MCTD

50
Q

Antibodies present in MCTD

A

High anti RNP
Absent anti-Sm

ANA generally persists through periods of remission

51
Q

Acute treatment for MCTD

A

Prednisone 1mg/kg/day

52
Q

Chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands (salivary and lacrimal)

A

Sjögren syndrome

53
Q

Vasculitis
Xerosis
Pruritus
Annular erythema

Decreased sweating

A

Sjögren syndrome

54
Q

Most definitive test for Sjögren syndrome

A

Labial salivary gland biopsy

55
Q

Classic criteria for Sjögren syndrome

A

2/3

Xerophthalmia
Xerostomia
Associated autoimmune, rheumatic or lymhoproliferative disorder

56
Q

Antibodies present in Sjögren syndrome

A

80% anti-Ro/SSA

40% anti-La/SSB

57
Q

Patients with Sjögren syndrome are predisposed to the development of this malignancy

A

Lymphoreticular- Non Hodgkin B cell lymphoma

58
Q

Pharma agents for Sjögren that help stimulate salivation

A

Pilocarpine

Cevimeline

👩🏼‍⚕️ ANDREWS' DISEASES OF THE SKIN 13TH EDITION (32 decks)

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