Connective tissue disorders

Question 1

Minor form of LE

• Few lesions present • Erythematous alopetic lesion (thick scale in scalp)

Answer 1

CCLE

Question 2

Almost always confined to the skin, no
systemic involvement (internal organs not
involved).

Answer 2

Localized CCLE

Question 3

Auspitz sign in CCLE/CDLE is

Answer 3

negative

Question 4

in SYSTEMIC OR GENERALIZED CCLE/ CDLE lesions are located where

Answer 4

above and below the neck

Question 5

Erythematous swollen plaque onthe malar area that extends to the bridge of thenose

Answer 5

Butterfly Rash:

Question 6

Involves the deep dermis and subcutaneous layer

Answer 6

LE/ CDLE PROFUNDUS

Question 7

Lesions are verrucous, with a rough surface(appear like warts resembling surface of cauliflower or broccoli) /
hyperkeratotic

Answer 7

CCLE/ CDLE HYPERTROPIC

Question 8

histologic features of CCLE Hypertropic

Answer 8

mixture of features of LE and lichen planus

Question 9

Photosensitivity is more pronounced

Answer 9

SUBACUTE LUPUS ERYTHEMATOSUS

Question 10

Widespread, non-scarring;
There can be anemia and defects in renal function but
not of serious nature

Answer 10

SUBACUTE LUPUS ERYTHEMATOSUS

Question 11

Diffuse non-scarring alopecia may occur

Answer 11

SUBACUTE LUPUS ERYTHEMATOSUS

Question 12

subacute LE may mitaken for nummular eczema, what is the difference?

Answer 12

lesion in subacute LE, is not pruritic compared to nummular eczema.

Question 13

common in systemic disease and

is often an indicator of disease activity

Answer 13

diffuse alopecia

Question 14

usually present on the face and other sun-exposed areas.

Answer 14

diffuse blotchy erythema

Question 15

Frequently involves the skin and joints (usual complain is joint pain).

Answer 15

SYSTEMIC LUPUS ERYTHEMATOSUS

Question 16

sudden exposure to cold (put ice on patient’s palm) will result to immediate pallor (sudden vasoconstriction), followed by cyanosis due to reduced blood supply, then erythema (sudden or rebound vasodilation) and
pain on the tips of the extremities

Answer 16

Raynaud’s phenomenon:

Question 17

painful red plaques or lesions on
the base of the fingers in association with exposure to
cold weather

Answer 17

SLE Chilblain Lupus

Question 18

blistering may occasionallyoccur in response to photosensitivity, rarely in childhood. Vesicles and bullae can be seen in exposed areas

Answer 18

SLE Bullous of Childhood

Question 19

When biopsy is done in these lesions (SLE Bullou in childhood),
immunofluorescence studies will show

Answer 19

deposition on immunoglobulins (IgG) on the

dermo-epidermal junctions of the skin.

Question 20

Inflammation of the striated muscle results to progressive proximal muscle weakness

Answer 20

DERMATOMYOSITIS (DM)

Question 21

in radiography of DM what can be seen in the affected proximal muscles?

Answer 21

calcium deposits

Question 22

Eruption may spread onto the cheeks and may simulate systemic LE. However, ____ involvement is
characteristic

Answer 22

eyelid

Question 23

pathognomonic of the disease DM

Answer 23

Heliotrope sign

Gottron’s Sign

Question 24

Specific/distinctive erythema over knuckles w/c may be papular

Answer 24

Gottron’s sign

Question 25

T or F both distal and muscles are affected in DM

Answer 25

F only the proximal muscles

Question 26

Treatment of DM

Answer 26

corticosteroid therapy

Question 27

test to confirm diagnoisis of DM and what i the result

Answer 27

muscle biopsy, presence of nodules on the affected area

Question 28

A fibrotic skin disease/excessive deposition of collagen and other connective tissue components leading to dermal fibrosis

Answer 28

scleroderma

Question 29

Subset of scleroderma

Answer 29

Calcinosis, Reynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasia)

Question 30

type of scleroderma where Involvement is not only limited to the skin but also to other organs

Answer 30

progressive systemic sclerosis

Question 31

Type of scleroderma: Thickening and induration of the skin in association with eosinophilia and an inflammatory infiltrate (mostly eosinophils) in the subcutaneous fascia and muscles

Answer 31

EOSINOPHILIC FASCITIS

Question 32

Only the fingers and toe digits may present with the dermal changes. There would be difficulty in flexing the digits. Occurs in poorly controlled juvenile diabetics.

Answer 32

Syndrome of Digital Sclerosis and Joint Contractures:

Question 33

clinical features of scleroderma

Answer 33

``` reynaud's P acrosclerosis/sclerodactyly calcinosis oral aperture becomes smaller sausage shaped fingers ```

Question 34

Chronic non-pitting edema | of fingers/tapering of fingers and thumbs due to atrophy of the pulp of the tips

Answer 34

Acroslerosis/ sclerodatyly

Question 35

how can digital ischemia lead to autoamputation

Answer 35

it can lead to cutaenous infarction/ulceration/gangrene of fingertips which may lead to autoamputation/

Question 36

worst prognosis of scleroderma

Answer 36

lung involvement: pulmo dse w/c may result to terminal fibrosis

Question 37

Sclerodermatous process involving the extremities, sparing the hands and feet

Answer 37

EOSINOPHILIC FASCIITIS

Question 38

what is present in the subQ fascia of the muscles in eosinophilic fascitis

Answer 38

eosiniphilic infiltrates

Question 39

what distinguishes EF from scleroderma

Answer 39

(-) RP and visceral involvement

Question 40

Induration of the skin particularly of the upper back • Occurs suddenly after a prodrome, particularly in diabetics (some patients develop upper respiratory tract infection, viral or strep illness)

Answer 40

SCLERODERMA OF BUSHKE

Question 41

what happens to the sub Q tissue and skin in Scleroderma of bushke

Answer 41

SQ is replaced by Collagenous T, kin has a wooden like consistency, shiny app

Question 42

A disorder with a characteristic lichenoid eruption, mucin deposition and paraproteinemia • Small, dome shaped lichenoid papules arranged in a linear manner

Answer 42

LICHEN MYXOEDEMATOSUS (PAPULAR MUCINOSIS)

Question 43

Localized form of scleroderma

Answer 43

morphoea

Question 44

T or F morphoea resolves in 1 year

Answer 44

F no definite no. of yrs

Question 45

common sites of linear form of morphoea

Answer 45

butt and upper part of legs, limb head trunk

Question 46

indented, vertical, colorless, line of skin on the forehead, looking like a sword has struck the area. May be associated with other neurologic conditions such as seizures

Answer 46

En coup de sabre

Question 47

type of morphoea which involves the skin only, appears as shiny and sclerotic throughout

Answer 47

GENERALIZED FORM

Question 48

Usually patients present with very itchy lesion. It is located in the delicate areas. In females, located in the genital or labial area

Answer 48

LICHEN SCLEROSUS ET ATROPHICUS

Question 49

In males, ivory white papules, atrophy, telangiectasia, purpura on foreskin and glans penis

Answer 49

BALANITIS SEROTICA OBLITERANS

Question 50

Autoimmune episodic inflammation of cartilaginous tissue, especially on the pinna; recurrences would eventually lead to fibrosis, destruction, and deformity

Answer 50

RELAPSING POLYCHONDRITIS

Question 51

why relapsing polychondritis is fatal

Answer 51

if trachea is involved

Question 52

relapsing Polychondritis is an autoimmune reaction to

Answer 52

native collagen