vesiculobullous disorders Flashcards
Pemphigus Vulgaris
- A serious bullous autoimmune disease. IgG antibodies induce acantholysis, resulting in a loss of cell-to-cell adhesions
- Occurs in middle-aged adults. More common in Jewish or Mediterranean
PV lesions?
- Lesions usually begin in the oral mucosa
o Skin lesions occur 6-12 months later
o May be pain or burning, but NOT PRURITIS
o Weakness and malaise are common - Lesions are round vesicles or bullae that contain clear liquid and easily rupture
o Nikolsky’s sign occurs (lateral extension of the lesions when pushed)
o The lesions are discrete and randomly scattered
o Erosions and crusts occur because of the fragility of the blisters - Secondary infection as well as fluid and electrolytes imbalance are common causes of morbidity and mortality
pv VARIANTS
pemphigus vegetans, pemphigus foliaceous, Brazilian pemphigus (fogo selvagem), pemphigus erythematosus, drug-induced pemphigus, and paraneoplastic pemphigus
PV dx
o Immunofluorescence of serum or blister material highlights IgG
o Biopsy proves acantholysis
PV tx
o Systemic therapy is required
o Start with prednisone, then add immunosuppressive agents, azathioprine, and/or methotrexate as needed
o Dapsone, gold, or cyclophosphamide may help in refractory cases
o Supportive therapies include fluid and electrolyte replacement, cleansing baths, wet dressings, topical steroids, and antibiotics as needed
Bullous Pemphigoid
- Autoimmune disorder. Typically in patients in their sixth decade of life
- Autoantibodies, complement fixation, neutrophil, and eosinophils cause bullous formation
- There may be a prodrome of urticarial or papular lesions
BP CF
- Bullae are large, tense, oval, or round and contain serous or hemorrhagic fluid. They rupture less easily than in pemphigus
- Typically, bullae collapse and crust. At times, bleeding erosions occur
- Axillae, thighs, groin, abdomen are commonly affected
o Mucous membrane lesions are less severe and less painful than in pemphigus vulgaris
BP dx
bx and immunofluorescense
BP tx
o Systemic prednisone may be given in high doses until remission, and then at a lower dose for maintenance
o Azathioprine may be added
o Mild cases or localized occurrences are treated with topical steroids
Acne conglobata
sever, chronic, cystic acne that is highly inflammatory
Acne vulgaris work up
testosterone, FSH, LH, or dehydroepiandrosterone 5 levels can be measured if an endocrine disorder is suggested. However, majority of acnes cases are not endocrine based
Acne tx behavioral changes
no picking, no mechanical “exfoliation or harsh abrasives, mild gentle cleansing twice daily with fingertips (non-soap cleansers are best), role of diet, choose oil-free and non-comedogenic products (Cetaphil, CeraVe, Oil of Olay, Neutrogena, Aveeno, Purpose, Dove)
acne topical tx
retinoids, azelaic acid, glycolic and salicyclic acid, antibacterials
retinoids
increase cell turnover, prvent new comedones, chemically exfoliate
4-6 wks for full effect
retinoid ex
tretinoin, adapalene, tazarotene
retinoid adr
dry skin/ irritation, sun sensitivity
CI: pregnancy
azelaic acid
anti-keratininzing, antibacterial, antiinflammatory
salicyclic acid
caution with sun
gels vs creams
gels more effective, but wil dry out skin
antibacterial topicals
BPO, clindamycin, erythromycin, sulfur-containing prep, metronidaxole
BPO
“workhouse of acne”
2.5-10%
Clindamycin
CI: pts with hx for uclerative colitis, pseudomembranous colitis (can pair with BPO to decrease resistance)
Erythromycin topical
P. acnes resistance
Dapsone
new med, used to be oral problem with G6PD deficiency
use for inflammaory acne
Oral abx for acne
minocyclin, doxy, erythromycin, (top 3), others included clindamycin, ampicillin, cephalosporins, bactrim
most need 2-4 wks for full effect
minocyclin oral CI/ADR
• Contraindications : pregnancy, peds
• Adverse Effects : vertigo (usually dose related), bluish-gray discoloration on skin, mucosa, teeth & nails
o Uncommon : lupus like syndrome, hypersensitivity syndrome, serum sickness, hepatitis, pseudotumor cerebri
doxy ADR/CI
- Contraindications: pregnancy, peds
* Adverse Effects: photosensitivity, GI upset, vaginitis
erythromycin adr/ci
- Used less frequently due to emerging P. acnes resistance levels
- Good option for pregnancy / peds
ADr: GI upset, vaginitis
Isotretinoin indications
- Indicated for severe, nodular, cystic, inflammatory, recalcitrant acne REFERRAL!
- Tightly regulated prescribing by the FDA (iPledge System)
- TERATOGENICITY: detailed contraception counseling, monthly pregnancy tests
Isot. ADR
o Dry skin, cheilitis, epistaxis
o Headaches, myalgias, arthralgias, bone pain
o Osteopenia
o Premature closure of the long bones
o Mood changes / depression (suicidal thoughts)
o Elevated glucose, TG, hepatotoxicity (elevated hepatic enzymes), leukopenia
o Decreased night vision , hearing changes
why would you use oral hormonal tx for acne?
adult acne, hirsutism, PCOS, premenstrual flares)
oral estrogen MOA
- Suppress sebaceous gland uptake of testosterone
* Suppress peripheral metabolism of testosterone
oral estrogen ADR
hypertension, hypercoagulability, hyperkalemia in some brands
Sprionolactone MOA
- Androgen receptor blocker
- Dose 50-200mg daily in divided doses
- Decreases serum testosterone levels
- Results in decreased sebum secretion
Sprion. ADR
menstrual irregularities, hyperkalemia, breast tenderness
o CATEGORY X
Acne complications
scarring, keloids, psychological impact, pyogenic granulomas
tx algorithm
comedonal (mild)
topical retinoid
papular/pustular (mild-mod)
topical retinoid +/- BPO or BPO/abx
pap/pustular (mod)
topical retinoid + BPO or BPO/abx +/- oral abx
nodular (mod-severe)
topical retinoid + BPO or BPO/abx + oral abx
nodular (severe)
oral isotretinoin
