13 - Autoimmune & HIV Flashcards

1
Q

Characteristics of autoimmune diseases

A
  • 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
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2
Q

Autoimmune diseases

A
  • Dermatomyositis
  • Rheumatoid Arthritis
  • Systemic Sclerosis (Scleroderma)
  • Systemic Lupus Erythematosus (SLE)
  • Bullous (Blistering) Autoimmune Diseases
  • Sjogren’s Syndrome
  • Reiter’s Syndrome
  • Polyarteritis nodosa
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3
Q

Dermatomyositis

A
  • 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
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4
Q

Dermatomyositis lesions

A

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

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5
Q

Dermatomyositis treatment

A

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

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6
Q

Rheumatoid arthritis

A

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

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7
Q

Cutaneous manifestations of rheumatoid arthritis

A
  • 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)
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8
Q

Systemic sclerosis (scleroderma)

A
  • 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
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9
Q

Syndrome associated with systemic sclerosis

A
  • CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia)
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10
Q

Generalized Scleroderma

don’t need to know details

A

 Raynaud’s phenomenon common
 Edema of the hands and feet: “puffiness”
 Narrowing of the fingers and toes

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11
Q

Localized Scleroderma (Morphea)

don’t need to know details

A

 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

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12
Q

Linear Scleroderma

don’t need to know details

A

 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

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13
Q

Systemic Lupus Erythematosus (SLE)

A
  • 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%
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14
Q

Diagnosis for SLE (need 4/11)

A

o Malar Rash, Discoid Rash, Photosensitivity, Oral Ulcers
o Arthritis, Serositis
o Renal Disorder, Neurological Disorder
o Hematological Disorder, Immunological Disorder
o Antinuclear Antibody

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15
Q

Derm manifestations of SLE

A

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

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16
Q

Chronic Discoid Lupus Erythematosus

Don’t need to know details

A

 Skin involvement predominates
 Systemic conditions rare
 Skin plaques with varying degrees of edema, erythema, scaling, follicular plugging and atrophy
 Face and scalp usually affected

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17
Q

Subacute cutaneous lupus erythematous

Dont’ need to know details

A

 Skin involvement predominates, not as intense as discoid LE
 Systemic conditions rare
 Widespread superficial, non-scarring lesions

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18
Q

SLE treatmetn

A
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
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19
Q

Bullous (blistering) Diseases

A
  • 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
20
Q

Pemphigus group – Bullous diseases

A

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

21
Q

Pemphigoid group – Bullous disease

A

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

22
Q

Dermatitis herpetiform group – Bullous disease

A
  • 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
23
Q

Dermatological manifestations of HIV

these conditions are more common, more severe in HIV

A
  • 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
24
Q

Staph aureus

A
  • 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.
25
Q

Bacillary angiomatosis

A
  • Causative Organism: BARTONELLA
  • An opportunistic infection associated with cat scratch disease
  • Cutaneous lesions resemble pyogenic granulomas (which is benign, so don’t just assume)
    o Fleshy, friable, protuberant papules-nodules, bleeds easily
  • Deep cellulitic plaques and nodules may occur
  • Systemic findings:
    o Fever, night sweat, weight loss, and anemia
    o Patients also present with abdominal pain, hepatospleenomegaly
  • Bone pain is also present which is demonstrated on radiographs as a lytic lesion
26
Q

Herpes simplex virus

A
  • Lesions many appear as grouped blister that rupture, crust, and heal in 7-10 days.
  • Once severely immunosuppressed, (HIV infected) the individual will experience a more chronic lesion with progression to an erosion
  • Lesions tend to be quite painful
27
Q

Varicella zoster

A
  • Initial presentation is usually herpes zoster, which often preceded thrush and oral hairy leukoplakia
  • Dermatomal eruptions may be particularly bullous, hemorrhagic, necrotic, and painful in HIV-infected persons
  • Dissemination is uncommon, but more frequent in the HIV-infected patient
28
Q

Molluscum contagiosum

A
  • Superficial cutaneous viral infection
    o Manifested as 2 - 3mm flesh colored hemispheric papules
     Faint whitish core, some may be umbilicated
  • Usually seen in immunocompromised or in children
  • In adults, it is usually sexually transferred
  • Occurs in approximately 5-18% of HIV-infected individuals
  • Early in the infection remains localized to the groin and face
  • Once CD-4 counts
29
Q

Human papilloma virus (HPV) warts

A
  • Occurs with increased frequency
  • Presentation is the same as in the non-immunosuppressed
  • Cervical cancer and anorectal cancer can be secondary to HPV
    o Higher frequency of cancer in immunocompromised
30
Q

Fungal and yeast infections

A
  • Superficial infections
  • Candida of the nail
  • Tinea of hands, feet, nials
  • Deep systemic fungal infectiosn
31
Q

Superficial infections

A

Thrush, intertriginous infections, nail, paronychial and foot infections
o Thrush: Most common form of yeast infection
o Intertriginous infections: Candida or tinea

32
Q

Candida infection of the nails

A

o Candida and tinea may infect the nails (Candida almost always affects the fingernails as paronychia)
o Onycholysis many occur
o Does not involve the nail plate

33
Q

Tinea infection of the nails, feet and hands

A

o Very common, but not a marker for HIV
o If you see the infection proximally in the nail (not distally, which is normal, ask more questions
o Nail plate is usually infected (4x more common than in general population)
 Trichophyton rubrum
 PSO- most commonly seen in HIV vs non HIV
 However, DLSO and TDO most common
o Tinea of the skin tends to be characterized by:
 Chronic maceration
 Scaling
 Blistering
 Thickening of the skin

34
Q

Deep systemic fungal infections

A

o Tend to have a higher incidence in the immunocompromised patient
o Aspergillus can occur as a primary or secondary infection
o Lesions appear as erythematous indurations overlying pustules, hemorrhagic ulcers, or molluscum contagiosum-like lesions

35
Q

Syphilis

A
  • Cutaneous presentation is the same as in the non-HIV patient
  • May be associated with palmoplantar keratoderma
  • Patient may develop CNS signs
36
Q

INFLAMMATORY CONDITIONS OF HIV - general

A
  • Papulosquamous diseases: Xerosis and ichthyosis
  • HIV Patients commonly complain of increasing dryness of the skin (xerosis)
  • Most prominent on the anterior leg
  • Frequent bathing is a precipitating factor and should be avoided
37
Q

Seborrheic dermatitis

A
  • Presents as faint pink patches with mild to profuse fine, loose waxy scales
    o Usual location is the face, scalp, axilla, and groin
  • Severe cases present with extensive involvement of the intertriginous areas
  • HIV Infected have a higher prevalence
38
Q

Psoriasis

A
  • Patients w/ HIV-associated psoriasis have higher prevalence of HLA-B27, suggesting genetic link
  • Associated with exacerbation of long-standing stable psoriasis, may worsen as HIV progresses
  • The incidence of severe involvement of the axilla is groin in increased
  • Pruritus may be serious
    o With scratching, secondary infection of excoriated plaques with staph aureus may occur
39
Q

Reiter’s syndrome

A
  • Reiter’s may be increased in the presence of HIV
    o Or it may merely permit the expression or exacerbation of features
  • Palms and soles develop superficial pustules
    o Dry, forming keratotic papules
    o Lesions will coalesce until the soles are thickened and scaled
     Keratoderma blennorrhagicum
40
Q

Eosinophilic folliculitis

A
  • Folliculitis commonly reveals eosinophils on biopsy

- Usually occurs in HIV-infected persons with T-helper cells

41
Q

NEOPLASIC DISORDERS OF HIV

A

Kaposi’s sarcoma*** = Common boards test question

42
Q

Kaposi’s sarcoma***

A
  • A tumor/neoplasm of mesenchymal cells involving the skin and other organs at times
    o Viral-HHV8
    o Usually transmitted through saliva
     Organ transplantation
     Blood transfusion
  • Initially appears as red to brown flat macules, papules, nodules, or tumors
43
Q

4 subtypes

A

o Classic KS (middle aged men of Mediterranean descent)
o African KS
o KS in iatrogenically immunosuppressed
o AIDS-related KS

44
Q

Prognosis

A
  • Natural history of KS is not uniform, but the prognosis historically was poor
    o 10% 5 yr relative survival rate early in AIDS epidemic
    o 68% 5 yr relative survival rate based on most recent stats  high increase in survival
45
Q

Diagnosis

A
  • Biopsies used for diagnosis  Early treatment is VERY important***
46
Q

Treatment

A
  • Treatment is geared towards controlling symptoms, disease may go into remission/be palliated, but not cured.
    o Cutaneous lesions:
     Cryotherapy, injections, radiation, excision, topical immunotherapy
    o Severe cutaneous lesions/Systemic
     IV chemo or immunotherapy
     Symptomatic
     May need to d/c immunosuppressive therapy
47
Q

Focus on

A
  • Pathognomonic conditions
  • Unique characteristics of each disease
  • Remember H & P
  • Don’t be afraid to biopsy
  • Can present on foot, but be a sign of an underlying disorder
  • Ask good questions and dig deeper  main point of this lecture