On this Mnemonic Monday, we challenge you to remember the list of subepidermal and pauci-inflammatory disorders in dermatopathology:
Porphyria cutanea tarda (PCT)
Epidermolysis bullousa acquisita (EBA)
Toxic epidermal necrolysis/Stevens-Johnson syndrome (TEN/SJS)
Bullous pemphigoid
Cicatricial pemphigoid
Dermatitis herpetiformis
Bullous lupus erythematosus (think “SLE”)
Linear IgA bullous dermatosis
Mnemonic:
Poor Earl’s toxic breath caused the dentist to use his SLEeves to light incense.
Porphyria cutanea tarda (PCT)
Epidermolysis bullousa acquisita (EBA)
Toxic epidermal necrolysis/Stevens-Johnson syndrome (TEN/SJS)
Bullous pemphigoid (BP)
Cicatricial pemphigoid
Dermatitis herpetiformis
SLE = bullous lupus erythematosus
L(ight) i(ncense) = Linear IgA bullous dermatosis
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Study More!
Need a refresher on subepidermal and pauci-inflammatory disorders in dermatopathology? Check out pages 335-336 of your 2021 Derm In-Review Study Guide.
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Further Reading
Check out the following case report published in the Journal of Drugs in Dermatology (JDD):
Recalcitrant Cicatricial Pemphigoid Treated With the Anti-TNF-Alpha Agent Etanercept
Cicatricial pemphigoid (mucous membrane pemphigoid) is a group of chronic disorders characterized by recurrent subepithelial blisters and scarring, predominantly of the mucous membranes.1,2 The authors report the case of a 63-year-old female with a 20-year history of cicatricial pemphigoid who had failed treatment with cytoxan, dapsone, systemic steroids and minocycline. She achieved complete clinical remission with the addition of etanercept 50 mg twice a week with azathioprine 150 mg daily. This patient has remained clear for two years with this combination and has tolerated a tapering of the azathioprine from the initial dose of 250 mg. Etanercept is a recombinant human dimeric fusion protein which acts as a competitive inhibitor of TNF-alpha by binding to both soluble and receptor-bound molecules of TNF-alpha.14 There have been a few reports of successful use of TNF-alpha inhibitors in cicatricial pemphigoid. This report will discuss the possible mechanism of action of the addition of TNF-alpha inhibitors as a steroidsparing agent in these patients.
Read the full article here.
Test your knowledge!
Which of the following shows granular deposition of IgA in the dermal papillae and along the basement membrane zone on direct immunoflourescence:
A: IgA pemphigus
B: Bullous pemphigoid
C: Linear IgA dermatosis
D: Dermatitis herpetiformis
E: Herpes gestationalis
To find out the correct answer and read the explanation, click here.
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