random Flashcards
paraneoplastic pemphigus
very rare, a/w malignancy, severe PV like mucus membrane lesions with dusky center like EM, Rapidly progressing. tx: find malignancy and treat
bp presentation
early prodrome, itchy non bullous rash, tense large bullae form bilaterally, symmetrical on flexural surfaces, heals with milia
mucous membrane pemphigoid
rare, 40-60 years old, chronic progressive and usually scars, painful mucosa erosions, tense blisters, sore throat, conjunctivitis. if severe or ocular need systemic rituximab and prednisone, ENT, gastro, optho consult.
MMF cellcept
immunosuppression, slow onset 2-3 mo, off label for inflam skin conditions, 500mg nightly to reduce GI distress and then slowly weekly increase in 500mg increments, mycophenolate rems teratogenic in 1st trimester for childbearing females
Azathioprine (Imuran)
immunosuppression, antiinflammatory, off label for AIBD. TPMT thiopurinemethyltranserase to measure activity in the RBCs, if low level do not use, if intermediate can start low at 1mg a day, if high start normal dose of 2-2.5mg/kg/day
pemphigoid genstationis
urticarial plaques and papules, vesicles, ocurrs in 2nd and 3rd trimsters, in belly button, associated with graves and thyroid, risk to fetus low birth weight, preterm, can have blisters, can occur in future pregnancy and menses and OCPs
polymorphous eruption of pregnancy
urticarial papules, late in 3rd, spares belly button, starts in striae, due to weight gain, no risk to fetus, resolves and 2 weeks after delivery and wont recur
DH presentation
intensely itchy, extensor surfaces, excoriations and papulovesicular, clustered and herpetiform, symmetrical, 8-12 hour prodrome burning before vesicles form. dx- need a dIF to show granular deposition of IgA autoantibodies to epidermal transflutaminase. screen- thyroid disease, family members, gi consult
DH mgmt
life long gluten free to control skin and gut and decrease lymphoma, no grains like wheat, barley, rye, spelt. Iodine and NSAIDs can induce eruptions. ROCs are OK. Dapsone.
Dapsone
myeloperoxidase enzyme inhibition for DH, off label for other AIBD, vasculitis. G6PD. increases with sulfa, mtx, chloroquine
hemolytic anemia in dapsone
can occur around 12 weeks, rbc and retic count drop,
methemolobinemia in dapsone
ha, fatigue, weakness, cyanosis, pale, s/s hypoxia
linear IgA
linear deposition of IgA in BMZ, antibodies to type 7 collagen, symptoms similar to BP, annular arcuate, may or may not look like MMF. Tx - dapsone
epidermolysis bullosa acquisita
20-30s, 80-90s, associated with chrons and SLE - screen. trauma induced bullae on hands, feet, fingers, toes, heals with mila and scarring, no mucus membranes need punch with DIF. tx dapsone, topical steroids. avoid trauma
ACLE
butterfly rash, patches and plaques, may be bullous or discound or erosions, waxes and wanes, photodistributed, duration: hours to days. risk of SLE > 90%. Multisystem, patients are sick. >90 percent progress to SLE
Subacute cutaneous LE
annular, polycyclic with central clearing, psoriasiform, persistent with flares, photodistrib NOT usually below waist, <50 Percent progress to SLE, limited and less severe than ACLE