The pemphigoid group of diseases Flashcards
BP epidemiology
- usually occurs after age 80
- associated with neurologic disease such as CVA or MS which predate the pemphigoid
- can be precipitated by medications
BP clinical manifestations
Progression: localized erythema that becomes pruritic urticarial plaques-> EM like red/cyanotic plaques in 1-3 weeks-> tense vesicles and bullae on the surface
- MC sites are lower abdomen, groin, flexor arms and legs
Localized clinical variants of BP
Vesicular, vegetating, hyperkeratotic, erythrodermic
DDx of BP
Epidermolysis bullosa acquisita DH Pemphigus bullous SLE bullous drug eruption Others: bug bits, cellulitis, EM, contact derm
Course and prognosis of BP
If generalized, poor prognosis especially if BP180 autoantibody +
If localized-> may become generalized or regress
- no clinical/immunologic/immunogenic factors predict disease duration
Lab diagnosis of BP
- often see elevated IgE and eosinophilia
W/U of BP
Two biopsies (1. perilesional for DIF and 2. intact elsion for histopathology) Serum for BP180, 230 as well as desmoglein 1 and 3 autoantibodies (BP180 titers decrease with disease activity)
DIF findings in BP
- linear deposits of IgG +/ C3 along BMZ
- salt split skin would show auto-antibodies on the roof (unlike epidermolysis bullosa acquisita)
Treatment of BP (mild/localized)
Topical steroids to affected areas, low dose systemic steroids (0.3mg/kg/day), antiinflammatory antibiotics
Treatment of BP (moderate to severe)
systemic steroids (0.5-1mg/kg/day) with Calcium, VitD, and PPI protection
very potent topical steroids to whole skin surface
anti-inflammatory antibiotics
Treatment of BP (refractory)
- can add nicotinamide to anti-inflammatory antibiotics
- Imuran (be sure to look at TPMT activity)
- methotrexate (with folic acid)
- dapsone
- chlorambucil
- rituximab
Treatment of BP (exceptional cases)
- CellCept 0.5-1gm BID
- IVIG
- cyclophosphamide
- plasmaphoresis
Localized pemphigoid examples
- Cicatricial (Mucous membrane)
- localized childhood vulvar pemphigoid (use group III-V steroids)
- pretibial pemphigoid (non-scaring usually in women)
- chronic pemphigoid of Brunsting-Perry (crops of grouped blisters on the head and neck that heal with atrophic scars)
- dyshidrosiform pemphigoid (vesiculobullous hemorrhagic lesions of palms and soles)
- Pemphigoid vegetans (erosive and vegetating plaques)
MM Pemphigoid epidemiology
- patients older than 40 with female predominance
MM Pemphigoid etiology
autoantibodies to BMZ antigens (lamina lucida proteins involved in keratinocyte adhesion to extracellular matrix)
Oral MM pemphigoid
MC site is desquamative gingitivits
- vermillion border spared unlike pemphigus
- relatively painless erosions
Ocular MM pemphigoid
MC presentation is unilateral conjunctivitis which progresses to bilateral
- gradual scaring of conjunctiva leads to obliteration of conjunctival sac
- leads to blindness in ~20% of patients
Treatment of MM Pemphigoid
treat as like BP (be aggressive in ocular/laryngeal/genital involvement)
Topical treatment of MMP
- oral (hydrogen peroxide + dexamethasone + diphenydramine all swish and spit OR fluocinonide gel)
- eye (artificial tears + systemic steroids as topicals less effective)
Systemic treatment of MMP
Dapsone
Steroids
steroid sparing agents such as Imuran, Cyclophosphamide, methotrexate, cyclosporine, or CellCept
Benign chronic bullous dermatosis of childhood clinical presentation
Large tense blisters on face (around mouth), lower trunk, inner thighs, and genitalia
- resolves by puberty at the latest
- normal jejunal biopsy
Benign chronic bullous dermatosis of childhood DIF
linear IgA deposits at BMZ
Benign chronic bullous dermatosis of childhood treatment
Dapsone as in DH followed by steroids if needed
Herpes gestationis etiology
Genetic predisposition
- mediated by IgG1 specific for the 180kDa component of hemidesmosomes
