Dermatology -- Bullae ===================== þ Pemphigus Vulgaris: - often confused with TEN - superficial blisters: intradermal lysis on H+E stain, direct immunoflorescence is specific - younger people (30's, 40's) - may have sudden onset that mimics TEN - extremly responsive to prednisone 80 mg daily or equivalent. - 100% mortality before steroids - autoimmune antibodies against desmosome in skin - Quest can do blood immunoassay to tell which it is - often see multiple doctors, given multiple Medrol dosepacks - give 30 mg a day of prednisone (don't give dosepack, don't give short course) will keep in check until able to see derm. - likes the scalp - painful mouth ulcers (30% present this way) - test for Nikolsky sign: press skin next to blister or urticarial lesion sideways, þ Pemphigoid: - Deeper blisters - older people, generally over 60-70 - itching is prominent - insidious onset - autoimmune antibodies against desmosome in skin - nonpainful mouth ulcers þ TEN vs. SSSS - Since SSSS cleaves above the dermoepidermal junction, substantial pigment usually remains on the affected skin. TEN cleaves at the dermoepidermal junction, so that the pigment is entirely removed when the skin desquamates. - Mucosal involvement is unusual with SSSS, and minimal when present. Mucosal involvement is common with TEN, and usually significant. - The age can be very helpful. SSSS is almost always seen in children under 6 years of age, although adults can (rarely) be affected. TEN is almost always seen in adults. Refs: Rosen 3rd ed. p 2405, Tintinalli 3rd ed. p 873 þ Toxic Epidermal Necrolysis (TEN) overlap with SJS þ Staph Scalded Skin (SSS) Syndromes (SSSS) þ Toxic Shock Syndrome (TSS)