13 - Autoimmune & HIV Flashcards
Characteristics of autoimmune diseases
- Dermatological manifestations can be the first manifestation of an autoimmune disease
- Immune system loses ability to recognize self from non-self
- Relevant immune phenomena can be identified
- Immunosuppressive therapy can be helpful
- Overlap phenomena between different autoimmune diseases is common
- They are often chronic and can be fatal
- Often a genetic predisposition is recognizable
Autoimmune diseases
- Dermatomyositis
- Rheumatoid Arthritis
- Systemic Sclerosis (Scleroderma)
- Systemic Lupus Erythematosus (SLE)
- Bullous (Blistering) Autoimmune Diseases
- Sjogren’s Syndrome
- Reiter’s Syndrome
- Polyarteritis nodosa
Dermatomyositis
- Inflammatory muscle disease, resulting from immune-mediated intramuscular vessel injury
- Typically affects children or adults over age 40
- Associated conditions include collagen vascular disease and internal malignancy
- Proximal muscle weakness is common with distal muscle strength intact
o Affected muscles are weak and sore
o Polyarthalgia may be present
Dermatomyositis lesions
Helitrope erythema of the eyelids
–Violaceous discoloration around the eyes
Gottron’s sign or papules
- -Smooth, flat-topped, violaceous to red lesions on knuckles - -Pathognomonic for dermatomyositis
Photosensitive violaceous erythema over joints and legs
–Patchy, diffuse becomes confluent
Dystrophic cuticles
Poikiloderma
- -Mottled white areas adjacent to brown pigmentation, telangiectasia & atrophy - -Occurs late in the disease coarse
Periungal telangiectasia
–Red linear streaks most prominent at posterior nail fold
Dermatomyositis treatment
You will refer on if you recognize these (what we can do is steroid treatment)
o Oral corticosteroids (Methotrexate or azathioprine if fail)
o Topical corticosteroids for erythematous lesions
o Minimal sunlight exposure
o Lab eval of muscle enzymes
o Bed rest for active muscle disease and an active and passive physical therapy program
o Evaluation for internal malignancy
Rheumatoid arthritis
VERY COMMON IN PODIATRY PRACTICE
- Chronic, systemic inflammatory condition secondary to autoimmune reaction of unknown origin
- Primarily affects the joints: producing a proliferative synovitis leading to
o Joint destruction
o Deformity
o Pain
- Blood vessels, heart, lungs and muscle also affected
- Multiple associated cutaneous lesions
Cutaneous manifestations of rheumatoid arthritis
- Subcutaneous nodules*
- 20% of patients common
- Most common dermal lesion in RA
- Usually present over joints
- May or may not be symptomatic
Others
- Erythematous, swollen joints
- Vasculitis
- Bywater’s lesions (Periungal, necrotic lesion in the hand or foot, 1 mm black spot adjacent to the nail)
- Leg ulcers (associated with stasis dermatitis and stasis ulcers)
Systemic sclerosis (scleroderma)
- General - Microvascular disease
o Excessive deposition of collagen (fibrosis) secondary to abnormal activation of the immune system due to unknown causes
o Skin is most commonly involved organ – tightening
o Gastrointestinal tract, kidneys, heart, muscles, and lungs frequently involved - Two major categories
o Diffuse – widespread skin involvement with early visceral involvement
o Localized – limited skin involvement, confined to fingers, forearms, and face, late visceral involvement
Syndrome associated with systemic sclerosis
- CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia)
Generalized Scleroderma
don’t need to know details
Raynaud’s phenomenon common
Edema of the hands and feet: “puffiness”
Narrowing of the fingers and toes
Localized Scleroderma (Morphea)
don’t need to know details
Non-specific erythema and pain that expands
Central portion transforms to firm, thickened and ivory in color with peripheral hyperpigmentation that is purple
Hair loss and absence of sweat are characteristic
Treatment is usually observation and spontaneous resolution.
Oral and topical corticosteroids may be used in some instances
Linear Scleroderma
don’t need to know details
More common on the lower extremities
Band-like induration possibly affecting subcutaneous and muscle tissue
Hypopigmented and hyperpigmented areas
When crosses a joint may cause contracture and stiffness
Systemic Lupus Erythematosus (SLE)
- Multi organ system autoimmune disease of unknown origin
- Antinuclear antibodies (ANA) particularly involved (not specific)
o Sm antigen and antibodies against double stranded DNA - Clinical features
o Acute or insidious onset
o Chronic, remitting and relapsing illness
o Principally affects the skin, joints, kidney, and serosal membranes
o 1/2500 Prevalence, 9:1 female to male
Typically arises in the 20’s and 30’s
American black women most common and severe
o Skin is involved in the majority of patients- 80-90%
Diagnosis for SLE (need 4/11)
o Malar Rash, Discoid Rash, Photosensitivity, Oral Ulcers
o Arthritis, Serositis
o Renal Disorder, Neurological Disorder
o Hematological Disorder, Immunological Disorder
o Antinuclear Antibody
Derm manifestations of SLE
o Characteristic “Butterfly” rash in 50% of patients
Erythema affects bridge of nose and cheeks
Similar rash is seen in extremities and trunk
Sunlight incites or accentuates the erythema
o Cutaneous lesions are widespread and annular
o Sun exposed areas, typically chest trunk
o Rarely scar
o Lesions are erythematous and scaly
o Nail folds typically show telangiectasis
Chronic Discoid Lupus Erythematosus
Don’t need to know details
Skin involvement predominates
Systemic conditions rare
Skin plaques with varying degrees of edema, erythema, scaling, follicular plugging and atrophy
Face and scalp usually affected
Subacute cutaneous lupus erythematous
Dont’ need to know details
Skin involvement predominates, not as intense as discoid LE
Systemic conditions rare
Widespread superficial, non-scarring lesions
SLE treatmetn
o Oral and topical corticosteroids, NSAIDS o Avoiding sunlight o Use of sunscreens and photoprotective clothing o Patient education Rest and energy conservation o Immunosuppressant agents Antimalarials, Methotrexate o IV Immunoglobulin
Bullous (blistering) Diseases
- Blisters are the primary and most distinctive skin lesion
- Occur at multiple levels of the skin
o Pemphigus group
o Pemphigoid group
o Dermatitis herpetiformis
Pemphigus group – Bullous diseases
Pemphigus Vulgaris is the most common type
o Affects scalp, face, axilla, groin, trunk, and pressure points
o Affects middle aged individuals (40-60 years)
o Characterized by widespread painful erosions of the skin & mucous membranes
o Primary lesion are superficial vesicles and bulla that readily rupture leaving weeping crusting, erosions that can become secondarily infected
Diagnosis - KNOW THIS
o ***+ Nikolsky’s Sign (ability to produce blister by rubbing skin adjacent to a natural blister)
o + Acantholysis (loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes)
Treatment
o Medical stabilization and local wound and burn care
o Can be life threatening if not treated
Pemphigoid group – Bullous disease
Acquired blistering diseases that cause separation at the basement membrane
o Disease of older individuals 60-80
o Firm stable blisters generally arise or erythematous skin, preceded by urticarial lesions
o Results in crusts & erosions
o Skin typically heals quickly without scarring
Diagnosis –> - Nikolsky’s sign ** KNOW THIS **
o This is what distinguishes the pemphigoid group from the pemphigus group
- Treatment
o Oral corticosteroids and immunosuppressive agents, local wound care
Dermatitis herpetiform group – Bullous disease
- AKA Duhring’s Disease
o Characterized by urticaria and vesicles
o Grouped blisters appear on erythematous base distinguishing characteristic
o Typically on the elbows, knees, back of neck, and sacral region
o Presents as excoriations, extremely pruritic
o Blisters are in a rosette pattern - NOTE Associated with celiac disease
- Treatment Local wound care and gluten free diet
Dermatological manifestations of HIV
these conditions are more common, more severe in HIV
- Patients with HIV often have several simultaneous or sequential cutaneous conditions with a progressively more aggressive course
o A KEY TO SUSPECTING UNDERLYING HIV INFECTION - HIV infected individuals commonly have cutaneous abnormalities; prevalence approaches 100%
- Three categories of dermatological presentation
o Infectious conditions
o Inflammatory conditions
o Neoplastic disorders
Staph aureus
- Most common cutaneous bacterial infection in the HIV infected individual
- Morphologic patterns: Bullous Impetigo, Ecthyma, Folliculitis
o Bullous impetigo presents as a very superficial blister or erosion
o Ecthyma Presents as an eroded or superficially ulcerated lesion with an adherent crust. Purulent material presents under the crust
o Folliculitis Presents as follicular pustules. Lesion of the trunk are intensely pruritic.
