Connective Tissue Disorders

Question 1

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”)

Answer 1

Discoid Lupus Erythematosus (DLE)

Question 2

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

Answer 2

Discoid Lupus Erythematosus (DLE)

Question 3

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.

Answer 3

Direct immunofluorescence (DIF) testing

Question 4

predominantly CD4+ lymphocytes

Answer 4

LE

Question 5

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

Answer 5

seborrheic dermatitis

Question 6

Apple-jelly nodules (granulomas) are seen with diascopy

Answer 6

lupus vulgaris

Question 7

absence of scarring and the presence of intensely edematous papules

plaques

and

DIF is generally negative or nonspecific in PMLE

Answer 7

polymorphous (PMLE)

light

eruption

Question 8

composed largely of CD8+ lymphocytes

Answer 8

lymphocytic infiltration (Jessner)

Question 9

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

Answer 9

Localized Discoid Lupus Erythematosus

Question 10

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

Answer 10

Generalized Discoid Lupus Erythematosus

Question 11

Lack of female preponderance

Lower frequency of photosensitivity

50% progress to SLE

Answer 11

Childhood DLE

Question 12

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

Answer 12

Hypertrophic Lupus Erythematosus

Question 13

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

Answer 13

Lupus

erythematosus–lichen

planus

overlap

syndrome

Question 14

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

Answer 14

Antimalarials

Question 15

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

Answer 15

Chilblain lupus erythematosus (Hutchinson)

Question 16

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.

Answer 16

Tumid lupus erythematosus

Question 17

Tumid lupus erythematosus

tx

Answer 17

Anti-malarials

Question 18

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

Answer 18

Lupus

erythematosus

panniculitis

(lupus

erythematosus profundus)

Question 19

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

Answer 19

SUBACUTE CUTANEOUS LUPUS

ERYTHEMATOSUS

Question 20

Shawl distribution

Answer 20

SUBACUTE CUTANEOUS LUPUS

ERYTHEMATOSUS

Question 21

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

Answer 21

SUBACUTE CUTANEOUS LUPUS

ERYTHEMATOSUS

Question 22

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

Answer 22

Neonatal Lupus Erythematosus

Question 23

Periocular involvement (raccoon eyes) may prominent

be

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

Answer 23

Neonatal Lupus Erythematosus

Question 24

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

Answer 24

Sneddon syndrome

Question 25

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

Answer 25

Multiple eruptive dermatofibroma

Question 26

Erythema multiforme-like lesions

Answer 26

Rowell’s Syndrome

Question 27

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

Answer 27

Calcinosis cutis

Question 28

the most cardiac manifestation LUPUS

Answer 28

pericarditis

Question 29

Due to vasoconstriction of arteries Triggered by exposure to cold & smoking Initially presents pallor > cyanosis > red

Answer 29

Raynaud’s phenomenon

Question 30

occasionally the forerunner of SLE.

Answer 30

Idiopathic purpura thrombocytopenic

Question 31

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

Answer 31

DERMATOMYOSITIS

Question 32

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

Answer 32

Dapsone

Question 33

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

Answer 33

DERMATOMYOSITIS

Question 34

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

Answer 34

Heliotrope rash:

Question 35

Heliotrope rash:

Answer 35

DERMATOMYOSITIS

Question 36

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

Answer 36

Gottron’s sign

Question 37

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

Answer 37

Mechanic’s hands

Question 38

Skin lesions / Eruption of Dermatomyositis precedes muscle symptoms by

Answer 38

2-3 months

Question 39

mainstay of acute treatment | dm

Answer 39

Prednisone

Question 40

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

Answer 40

Methotrexate

Question 41

Absence of myositis with skin changes Muscle inflammation asymptomatic

Answer 41

amyotrophic dermatomyositis / CUTANEOUS TYPES Dermatomyositis sine myositis

Question 42

Neoplasia with Dermatomyositis

Answer 42

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

Question 43

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

Answer 43

Childhood Dermatomyositis   Brunsting type

Question 44

vasculitis of the gastrointestinal tract muscles rapid onset of severe weakness steroid unresponsiveness high death rate

Answer 44

Banker type

Question 45

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

Answer 45

SCLERODERMA

Question 46

Thibierge–Weissenbach syndrome / CREST syndrome

Answer 46

Systemic SCLERODERMA

Question 47

localized, generalized, profunda, atrophic, and pansclerotic types

Answer 47

morphea

Question 48

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

Answer 48

Localized Morphea

Question 49

Localized Morphea Treatment

Answer 49

Calcipotriol

Question 50

multiple small, chalk-white, flat or slightly depressed macules occur over the chest, neck, shoulders, or upper back lesions are not very firm

Answer 50

Guttate Morphea

Question 51

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

Answer 51

Generalized Morphea

Question 52

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.

Answer 52

Atrophoderma of Pasini and Pierini

Question 53

manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, bone. There is disabling limitation of motion of joints.

Answer 53

Pansclerotic morphea

Question 54

Sclerosis of dermis, panniculitis, fascia, muscle and bone

Answer 54

Morphea profunda

Question 55

progressive hemifacial exophthalmos, and alopecia

Answer 55

Parry–Romberg syndrome

Question 56

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)

Answer 56

Linear Scleroderma

Question 57

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

Answer 57

CREST Syndrome

Question 58

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

Answer 58

Progressive systemic sclerosis

Question 59

Other patients present with “woody edema” of the hands. The heart, lungs, gastrointestinal tract, kidney, and other organs are frequently involved.

Answer 59

Progressive systemic sclerosis

Question 60

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.

Answer 60

Progressive systemic sclerosis

Question 61

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

Answer 61

neck sign”

Question 62

The most frequent internal organ involved is the sclerosis

Answer 62

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

Question 63

True speckled or anticentromere patterns is sensitive and specific for

Answer 63

CREST

Question 64

Anti-single stranded DNA antibodies are common in

Answer 64

linear scleroderma