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Flashcards in Systemic derm Diseases Deck (84):
1

What is dermatomyositis?

-Rare inflammatory muscle and skin disease, causing proximal muscle weakness and a skin rash

2

Which gender and in what ages is dermatomyositis more common in?

Women
5-10 years and over 40

3

Adult dermatomyositis is associated with what?

Malignancy and collagen vascular disease

4

What is the heliotrope sign in dermatomyositis?

Red-purple edematous erythema on the upper palpebral

5

What is Gottron's sign found in dermatomyositis?

Red-purple, keratotic and atrophic erythema / macules on the extensor surface of finger joints

6

Where is the erythema usually seen with dermatomyositis?

Extensor surfaces of extremity joints, and slight raised red-purple erythema over the elbows or knees

7

What are the labs that can be elevated with dermatomyositis?

Creatinine kinase

8

What are the antibodies that can be found with dermatomyositis?

Anti-jo-1

9

Is the rash seen with dermatomyositis pruritic?

Yes--can awaken from sleep

10

What happens to the hair with dermatomyositis?

Localized Alopecia

11

What can happen to the lesions with dermatomyositis?

Calcinosis

12

What are the systemic s/sx of dermatomyositis?

Fever
Malaise
Weight loss

13

What are the GI findings of dermatomyositis? (3)

-Esophageal impairment / dysphagia
-GERD
-Ulcers and infections

14

What are the cardiac findings of dermatomyositis? (2)

AV defects
Tachyarrhythmias

15

What is the gait that is characteristic of dermatomyositis?

Tip-toe gait

16

What is the treatment for dermatomyositis?

-Steroids
-Immunosuppressants (MTX)
-Avoid sun + sunscreen

17

What is the prognosis for dermatomyositis?

Gradual disability in most, but 20% have spontaneous remission

18

True or false: there is an increased risk of malignancy in dermatomyositis

True

19

What are the psych / neuro s/sx of SLE?

Psychosis
Szs

20

What are the lung / CV findings of SLE?

Pleuritis and pericarditis

21

What is the natural h/o SLE?

Flares and remissions

22

Who is SLE more common in?

Black women of child -bearing ages

23

What is the discoid rash with SLE?

Erythematous, raised patches with adherent keratotic scaling and follicular plugging

24

What is the malar rash seen with SLE?

Fixed erythema (flat or raised) over the malar eminences

25

What are the oral findings of SLE?

Oral or nasopharyngeal ulceration that are usually painless

26

What are the hematological disorders found with SLE?

-hemolytic anemia
-Leukopenia

27

What are the abs that are found with SLE? (3)

-anti dsDNA
-anti Smith
-ANA

28

What infectious disease do patients with SLE test positive for?

Syphilis

29

True or false: the rash seen with SLE is photosensitive

True

30

What is discoid lupus? What areas of the body are particularly affected? What are the lesion characteristics?

A chronic skin condition of sores with inflammation and scarring favoring the face, ears, and scalp and at times on other body areas.

These lesions develop as a red, inflamed patch with a scaling and crusty appearance, that are well demarcated.

31

True or false: there is a high incidence of systemic disease in discoid lupus

False--low incidence

32

What are the drugs that can cause SLE?

HCTZ
CCBs
ACEIs

33

What antifungal drug can cause subacute cutaneous SLE?

Terbinafine

34

Who usually gets drug induced lupus?

White females

35

What is Subacute cutaneous lupus erythematosus? Who does it usually affect, what what are the characteristic skin lesions?

a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as polycyclic annular lesions or psoriasiform plaques

36

What is the treatment for cutaneous lupus?

-Sunscreens
-Topical steroids TID
-Tacrolimus

37

What are the systemic therapies for SLE?

-Antimalarials
-MTX
-Isotretinoin

38

What is scleroderma?

Systemic autoimmune disease characterized by sclerosing of the skin and sometimes internal organs

39

What does scleroderma present with, usually?

Thickening of the hand and raynaud's phenomenon

40

What are the three stages of the skin part of scleroderma?

edematous
Indurative
Atrophic

41

What are the components of CREST syndrome?

Calcinosis
Raynaud's
Esophageal dysfunction
Sclerodactyly
Telangiectasias

42

What is the order of color changes with Raynaud's?

White
Blue
Red

43

What is the morphea type of scleroderma? How does it start and progress?

Localized form of scleroderma, that usually starts with one to several circumscribed areas of purplish induration, that progresses to a raised, white sacr with liac ring

44

Raised white scar with lilac ring = ?

Morphea

45

What is the treatment for morphea?

Intralesional steroids or hydroxychloroquine

46

What is the linear scleroderma form of moephea?

Linear patch of sclerotic skin that crosses joint lines, and may lead to joint contractures

47

What is the en coup de sabre form of morphea?

a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp

48

What is Reiter's syndrome?

Reactive arthritis that usually occurs following an infection

Triad of:
-Inflammatory arthritis
-Conjuntivitis / uveitis
-Urethritis

"can't see, can't pee, can't climb a tree"

49

What is the HLA haplotype associated with Reiter's syndrome?

HLA B27

50

What are the skin findings of Reiter's syndrome?

Keratoderma blenorrhagicum

51

What is mycosis fungoides?

T cell lymphoma that presents with rash-like patches that are pruritic, similar to psoriasis

52

What is Paget's disease of the bone?

Chronic bone deforming disorder that results from excessive breakdown and formation of bone, causing affected bones to weaken, change shape, and produce pain.

53

What are the lab findings of Paget's disease of the bone?

Elevated alk phos

54

What are the major symptoms of Paget's disease?

bone pain
hearing loss

55

What is the characteristic histological finding of Paget's disease of the bone?

Jigsaw eosinophilia

56

What are the skin manifestations of Paget's disease of the bone?

None, but skull deformity may occur

57

What causes Paget's disease of the breast?

Ductal CA that migrates to the skin

58

What is extramammary Paget's disease?

a rare, slow-growing, usually noninvasive intraepithelial (in the skin) adenocarcinoma outside the mammary gland and includes Paget's disease of the vulva and the extremely rare Paget's disease of the penis.

59

What are the s/sx of extramammary Paget's disease?

itching, burning, and soreness

60

What is the treatment for Kaposi's sarcoma?

Excision or chemo

61

What is Sweet's syndrome (acute febrile neutrophilic dermatosis)? S/sx?

Skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.

62

What are the characteristic lab findings of Sweet's syndrome? (4)

ESR elevated
CRP elevation
Leukocytosis
Neutrophilia

63

What is the treatment for Sweet's syndrome?

Systemic corticosteroids

64

What is Sweet's syndrome associated with?

Underlying hematological or visceral malignancy or pregnancy

65

What are the skin findings with sweet's disease?

Tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.

66

What is the classic skin triad of hypothyroidism?

-Edematous eyelids
-Swollen lower lip
-Droopy facial appearance

67

What are the characteristics of diabetic dermopathy?

bruises in the lower extremities

68

What is the associated of granuloma annulare?

pre-DM

69

What is necrobiosis lipoidica diabeticorum?

Necrotizing skin condition in DM pts that is usually asymptomatic, but may become tender and ulcerate when injured

70

What is waterhouse friederichsen syndrome?

N meningitidis causes hemorrhage into adrenal glands, causing insufficiency

71

What are two diseases that can cause acanthosis nigricans?

DM II
Cushing's

72

What are the crystals found in gout?

Monosodium urate

73

What is pseudogout? S/sx?

-Accumulation of Ca pyrophosphate in CT, with the knee joint most commonly affected.
-Joint pain + cellulitis-like rash over the area

74

What is the difference in the hand findings of osteoarthritis vs RA?

OA = MCP
RA = DIP and PIPs

75

What is psoriatic arthritis? S/sx?

-Inflammatory arthritis that can develop in people with psoriasis
-Inflammatory arthralgias
-Dactylitis (sausage joint)

76

What are the nail findings of psoriatic arthritis?

Onycholysis, pitting and hyperkeratosis

77

What are the lesions like with psoriasis? What makes the pain better?

Large, scaly white-red Plaques that get better in the sun

78

What, generally, is psoriasis? S/sx?

Autoimmune disease characterized by an abnormally excessive and rapid growth of the epidermal layer of the skin

large, pruritic plaques

79

What is the treatment for psoriatic arthritis?

MTX or steroids

80

What causes the elevated Ca in sarcoidosis?

Granulomas of sarcoid can produce vit D

81

What is lupus pernio? What is it pathognomonic for?

a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis.

82

What are the mouth findings in Peutz-Jeghers syndrome?

Patches of hyperpigmentation in the mouth and on the hands and feet

83

What is osler-Weber-Rendu disease (Hereditary hemorrhagic telangiectasia)? S/sx?

-AD genetic disorder, that leads to abnormal blood vessel formation in the skin, mucous membranes, and organs
-Telangiectasis in the mouth, epistaxis, GI bleeds

84

What is the inheritance pattern of Peutz-jeghers syndrome?

AD