Connective Tissue Disorders Flashcards
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”)
Discoid Lupus Erythematosus (DLE)
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
Discoid Lupus Erythematosus (DLE)
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.
Direct immunofluorescence (DIF) testing
predominantly CD4+ lymphocytes
LE
does not show atrophy, alopecia, or dilated follicles, and has greasy, yellowish scale without follicular plugs
seborrheic dermatitis
Apple-jelly nodules (granulomas) are seen with diascopy
lupus vulgaris
absence of scarring and the presence of intensely edematous papules
plaques
and
DIF is generally negative or nonspecific in PMLE
polymorphous (PMLE)
light
eruption
composed largely of CD8+ lymphocytes
lymphocytic infiltration (Jessner)
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
Localized Discoid Lupus Erythematosus
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
Generalized Discoid Lupus Erythematosus
Lack of female preponderance
Lower frequency of photosensitivity
50% progress to SLE
Childhood DLE
Non-pruritic papulonodular lesions may occur on the arms and hands, resembling keratoacanthoma or hypertrophic lichen planus (LP) on the arms and hands
Hypertrophic Lupus Erythematosus
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
Lupus
erythematosus–lichen
planus
overlap
syndrome
can occasionally produce a lichenoid drug eruption in patients with LE.
Antimalarials
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
Chilblain lupus erythematosus (Hutchinson)
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.
Tumid lupus erythematosus
Tumid lupus erythematosus
tx
Anti-malarials
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
Lupus
erythematosus
panniculitis
(lupus
erythematosus profundus)
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
SUBACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS
Shawl distribution
SUBACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS
dustlike particulate deposition of IgG in epidermal nuclei of Ro-positive patients may be present and is a helpful diagnostic finding.
SUBACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS
infant girls, born to mothers who carry the Ro/SSA antibody
Neonatal Lupus Erythematosus
Periocular involvement (raccoon eyes) may prominent
be
Telangiectasia or dermal mucinosis in an acral papular pattern may be the predominant findings
Neonatal Lupus Erythematosus
is composed of livedo reticularis and strokes related to a hyalinizing vasculopathy.
Sneddon syndrome
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
Multiple eruptive dermatofibroma
Erythema multiforme-like lesions
Rowell’s Syndrome
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
Calcinosis cutis
the most cardiac manifestation LUPUS
pericarditis
Due to vasoconstriction of arteries
Triggered by exposure to cold & smoking
Initially presents pallor > cyanosis > red
Raynaud’s phenomenon
occasionally the forerunner of SLE.
Idiopathic purpura
thrombocytopenic
An inflammatory myositis characterized by prodromata, edema, dermatitis and muscular inflammation and degeneration
DERMATOMYOSITIS
the drug of choice for bullous systemic LE, and may be effective in some cases of SCLE and DLE
Dapsone
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
DERMATOMYOSITIS
Begins with erythema and swelling of the face and upper eyelids; eyelids become swollen and pinkish violet (heliotrope), tender with minute telangiectasis
Heliotrope rash:
Heliotrope rash:
DERMATOMYOSITIS
pink to reddish-purple atrophic or scaling eruption over the knuckles, knees, and elbows
pathognomonic sign
Gottron’s sign
Hyperkeratosis, scaling, fissuring, and hyperpigmentation over the fingertips, sides of the thumb, and fingers with occasional involvement of the palms
Mechanic’s hands
Skin lesions / Eruption of Dermatomyositis precedes muscle symptoms by
2-3 months
mainstay of acute treatment
dm
Prednisone
Avoid in patients with pulmonary disease or anti-Jo-1 antibodies
Methotrexate
Absence of myositis with skin changes
Muscle inflammation asymptomatic
amyotrophic
dermatomyositis
/
CUTANEOUS TYPES
Dermatomyositis sine myositis
Neoplasia with Dermatomyositis
Ovarian cancer seen in more than 20% of women >40 years old with DM
More common
Has a slow course, progressive weakness, calcinosis, and steroid responsiveness
Childhood Dermatomyositis
Brunsting type
vasculitis of the gastrointestinal tract
muscles
rapid onset of severe weakness
steroid unresponsiveness
high death rate
Banker type
characterized by the appearance of circumscribed or diffuse, hard, smooth, ivory-colored areas that are immobile and give the appearance of hidebound skin
SCLERODERMA
Thibierge–Weissenbach syndrome / CREST syndrome
Systemic SCLERODERMA
localized, generalized, profunda, atrophic, and pansclerotic types
morphea
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
Localized Morphea
Localized Morphea Treatment
Calcipotriol
multiple small, chalk-white, flat or slightly depressed macules occur over the chest, neck, shoulders, or upper back
lesions are not very firm
Guttate Morphea
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
Generalized Morphea
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.
Atrophoderma of Pasini and Pierini
manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, bone.
There is disabling limitation of motion of joints.
Pansclerotic morphea
Sclerosis of dermis, panniculitis, fascia, muscle and bone
Morphea profunda
progressive hemifacial exophthalmos, and alopecia
Parry–Romberg syndrome
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)
Linear Scleroderma
has the most favorable prognosis, owing to the usually limited systemic involvement
CREST Syndrome
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
Progressive systemic sclerosis
Other patients present with “woody edema” of the hands.
The heart, lungs, gastrointestinal tract, kidney, and other organs are frequently involved.
Progressive systemic sclerosis
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.
Progressive systemic sclerosis
ridging and tightening of the neck on extension; (+) in 90%
neck sign”
The most frequent internal organ involved is the
sclerosis
GI tract, followed by the lungs, cardiovascular and renal systems
True speckled or anticentromere patterns is sensitive and specific for
CREST
Anti-single stranded DNA antibodies are common in
linear scleroderma
