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Dermatology > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

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Dermatology Board Prep flashcards
1
Q

occurs in young adults, with women outnumbering men 2:1

usually confined to the skin

begin as dull red macules or indurated plaques that develop an adherent scale, and evolve with atrophy, scarring, and pigment changes

the hyperkeratosis extends to the hair follicles producing carpet tack-like spines on the undersurface of the scale (Carpet tacks or langue du chat or cat’s tongue”)

A

Discoid Lupus Erythematosus (DLE)

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2
Q

Acute lesions inflammation dermatitis

show and

patchy vacuolar

lymphoid interface

Lesions established for several months begin to show hyperkeratosis, basement membrane thickening, and dermal mucin

Chronic, inactive lesions show atrophy, with postinflammatory pigmentation and scarring throughout the dermis

A

Discoid Lupus Erythematosus (DLE)

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3
Q

positive in more than 75% of cases

confirm the diagnosis of DSLE

Get sample from a lesion that is > 2months duration

Early lesions usually have negative or nonspecific immunofluorescent findings,

Established lesions usually demonstrate strong continuous granular deposition of immunoglobulin and complement located at the dermoepidermal junction.

A

Direct immunofluorescence (DIF) testing

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4
Q

predominantly CD4+ lymphocytes

A

LE

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5
Q

does not show atrophy, alopecia, or dilated follicles, and has greasy, yellowish scale without follicular plugs

A

seborrheic dermatitis

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6
Q

Apple-jelly nodules (granulomas) are seen with diascopy

A

lupus vulgaris

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7
Q

absence of scarring and the presence of intensely edematous papules

plaques

and

DIF is generally negative or nonspecific in PMLE

A

polymorphous (PMLE)

light

eruption

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8
Q

composed largely of CD8+ lymphocytes

A

lymphocytic infiltration (Jessner)

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9
Q

Perifollicular erythema and the presence of easily extractable anagen hairs are signs of active disease
On the lips: patches are gray or red and hyperkeratotic. They may be eroded and surrounded by a narrow, red inflammatory zone

A

Localized Discoid Lupus Erythematosus

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10
Q

less common than localized DLE

usually superimposed on a localized DLE case

Most often the thorax and upper extremities are affected in addition to the head and neck

A

Generalized Discoid Lupus Erythematosus

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11
Q

Lack of female preponderance

Lower frequency of photosensitivity

50% progress to SLE

A

Childhood DLE

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12
Q

Non-pruritic papulonodular lesions may occur on the arms and hands, resembling keratoacanthoma or hypertrophic lichen planus (LP) on the arms and hands

A

Hypertrophic Lupus Erythematosus

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13
Q

lesions are usually large, atrophic, hypopigmented, red or pink patches and plaques with telangiectasia and scaling on the extensor aspects of the extremities and midline back

Prominent palmoplantar involvement is characteristic and tends to be the most troublesome feature for these patients.

Nail dystrophy and anonychia may occur

A

Lupus

erythematosus–lichen

planus

overlap

syndrome

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14
Q

can occasionally produce a lichenoid drug eruption in patients with LE.

A

Antimalarials

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15
Q

a chronic, unremitting form of LE with the fingertips, rims of ears, calves, and heels affected, especially in women

usually preceded by DLE on the face

due to cold

A

Chilblain lupus erythematosus (Hutchinson)

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16
Q

rare

edematous erythematous plaques, usually on the trunk the lesions demonstrate a patchy superficial and deep perivascular and periadnexal lymphoid infiltrate that frequently affects the eccrine coil

Dermal mucin deposition is typical and may be striking.

A

Tumid lupus erythematosus

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17
Q

Tumid lupus erythematosus

tx

A

Anti-malarials

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18
Q

Deep and derma subcutaneous nodules that are commonly firm, sharply defined, and nontender beneath the normal skin of the head, face, or upper arms or chest, buttocks and thighs

A

Lupus

erythematosus

panniculitis

(lupus

erythematosus profundus)

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19
Q

most often white women aged 15–40

Scaly papules, which evolve either into psoriasiform or polycyclic annular lesions

lesions vary from red to pink with faint violet tones

A

SUBACUTE CUTANEOUS LUPUS

ERYTHEMATOSUS

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20
Q

Shawl distribution

A

SUBACUTE CUTANEOUS LUPUS

ERYTHEMATOSUS

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21
Q

dustlike particulate deposition of IgG in epidermal nuclei of Ro-positive patients may be present and is a helpful diagnostic finding.

A

SUBACUTE CUTANEOUS LUPUS

ERYTHEMATOSUS

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22
Q

infant girls, born to mothers who carry the Ro/SSA antibody

A

Neonatal Lupus Erythematosus

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23
Q

Periocular involvement (raccoon eyes) may prominent

be

Telangiectasia or dermal mucinosis in an acral papular pattern may be the predominant findings

A

Neonatal Lupus Erythematosus

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24
Q

is composed of livedo reticularis and strokes related to a hyalinizing vasculopathy.

A

Sneddon syndrome

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25
Q

Leg ulcers, typically deeply punched out and with very little inflammation, may be seen on the pretibial or malleolar areas.

present with a livedoid pattern and many have an antiphospholipid antibody

A

Multiple eruptive dermatofibroma

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26
Q

Erythema multiforme-like lesions

A

Rowell’s Syndrome

27
Q

plaque-like or papulonodular depositions of mucin.

reddish-purple to skin-colored lesions are often present on the trunk and arms or head and neck

occur in patients with SLE, rheumatoid arthritis, or other immune complex-mediated disease

palisaded neutrophilic granulomatous dermatitis immune complex disease

a

A

Calcinosis cutis

28
Q

the most cardiac manifestation LUPUS

A

pericarditis

29
Q

Due to vasoconstriction of arteries

Triggered by exposure to cold & smoking

Initially presents pallor > cyanosis > red

A

Raynaud’s phenomenon

30
Q

occasionally the forerunner of SLE.

A

Idiopathic purpura

thrombocytopenic

31
Q

An inflammatory myositis characterized by prodromata, edema, dermatitis and muscular inflammation and degeneration

A

DERMATOMYOSITIS

32
Q

the drug of choice for bullous systemic LE, and may be effective in some cases of SCLE and DLE

A

Dapsone

33
Q

Polymyositis:muscle changes

involvement

without

skin

o

o

o

o

o

o

Gottron’s sign

With or without skin lesions, weakness of proximal muscle groups is characteristic

A

DERMATOMYOSITIS

34
Q

Begins with erythema and swelling of the face and upper eyelids; eyelids become swollen and pinkish violet (heliotrope), tender with minute telangiectasis

A

Heliotrope rash:

35
Q

Heliotrope rash:

A

DERMATOMYOSITIS

36
Q

pink to reddish-purple atrophic or scaling eruption over the knuckles, knees, and elbows

pathognomonic sign

A

Gottron’s sign

37
Q

Hyperkeratosis, scaling, fissuring, and hyperpigmentation over the fingertips, sides of the thumb, and fingers with occasional involvement of the palms

A

Mechanic’s hands

38
Q

Skin lesions / Eruption of Dermatomyositis precedes muscle symptoms by

A

2-3 months

39
Q

mainstay of acute treatment

dm

A

Prednisone

40
Q

Avoid in patients with pulmonary disease or anti-Jo-1 antibodies

A

Methotrexate

41
Q

Absence of myositis with skin changes

Muscle inflammation asymptomatic

A

amyotrophic

dermatomyositis

/

CUTANEOUS TYPES

Dermatomyositis sine myositis

42
Q

Neoplasia with Dermatomyositis

A

Ovarian cancer seen in more than 20% of women >40 years old with DM

43
Q

More common

Has a slow course, progressive weakness, calcinosis, and steroid responsiveness

A

Childhood Dermatomyositis

Brunsting type

44
Q

vasculitis of the gastrointestinal tract

muscles

rapid onset of severe weakness

steroid unresponsiveness

high death rate

A

Banker type

45
Q

characterized by the appearance of circumscribed or diffuse, hard, smooth, ivory-colored areas that are immobile and give the appearance of hidebound skin

A

SCLERODERMA

46
Q

Thibierge–Weissenbach syndrome / CREST syndrome

A

Systemic SCLERODERMA

47
Q

localized, generalized, profunda, atrophic, and pansclerotic types

A

morphea

48
Q

Rose or violaceous macules may appear first, followed by smooth, hard, somewhat depressed, yellowish-white or ivory lesions margins are surrounded by a light violaceous zone or telangiectases → elasticity is lost

A

Localized Morphea

49
Q

Localized Morphea Treatment

A

Calcipotriol

50
Q

multiple small, chalk-white, flat or slightly depressed macules occur over the chest, neck, shoulders, or upper back

lesions are not very firm

A

Guttate Morphea

51
Q

Widespread involvement by indurated plaques with pigmentary change

Muscle atrophy may be present, but there is no visceral involvement

2 Types:

Patients may lose their wrinkles as a result of the firmness and contraction of skin

A

Generalized Morphea

52
Q

brownish-gray, oval, round or irregular, smooth atrophic lesions depressed below the level of the skin with a welldemarcated, sharply sloping border

occurs mainly on the trunk of young individuals, predominately females

Linear atrophoderma of Moulin is condition that follows lines of Blaschko.

A

Atrophoderma of Pasini and Pierini

53
Q

manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, bone.

There is disabling limitation of motion of joints.

A

Pansclerotic morphea

54
Q

Sclerosis of dermis, panniculitis, fascia, muscle and bone

A

Morphea profunda

55
Q

progressive hemifacial exophthalmos, and alopecia

A

Parry–Romberg syndrome

56
Q

linear lesions may extend the length of the arm or leg, and may follow lines of Blaschko

begins during the first decade of life.

may also occur parasagittally on the frontal scalp and extend part way down the forehead (en coup de sabre)

A

Linear Scleroderma

57
Q

has the most favorable prognosis, owing to the usually limited systemic involvement

A

CREST Syndrome

58
Q

A generalized disorder of connective tissue in which there is thickening of dermal collagen bundles, and fibrosis and vascular abnormalities in internal organs.

Raynaud phenomenon is the first manifestation

A

Progressive systemic sclerosis

59
Q

Other patients present with “woody edema” of the hands.

The heart, lungs, gastrointestinal tract, kidney, and other organs are frequently involved.

A

Progressive systemic sclerosis

60
Q

Classic Criteria include either proximal sclerosis or two or all of the following:

CREST Syndrome

o

o

o

sclerodactyly

May be limited associated with

to

the

hands

digital pitting scars of the fingertips or loss of substance of the distal finger pad

bilateral basilar pulmonary fibrosis.

A

Progressive systemic sclerosis

61
Q

ridging and tightening of the neck on extension; (+) in 90%

A

neck sign”

62
Q

The most frequent internal organ involved is the

sclerosis

A

GI tract, followed by the lungs, cardiovascular and renal systems

63
Q

True speckled or anticentromere patterns is sensitive and specific for

A

CREST

64
Q

Anti-single stranded DNA antibodies are common in

A

linear scleroderma

Dermatology (6 decks)

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