bullous pemphigoid

Question 1

immunopath

tx

Answer 1

■ Abullous autoimmune disease usually in elderly patients.
■ Pruritic papular and/or urticarial lesions with large tense bullae.
■ Subepidermal blisters with eosinophils.
■ C3 and IgG at epidermal basement membrane, antibasement membrane IgG autoantibodies in
serum.
■ Autoantigens are keratinocyte hemidesmosome proteins.
■ Therapy includes topical and systemic glucocorticoids and other immunosuppressives.

Question 2

age gender

Answer 2

AGEOFONSET Sixtyto80years.

SEX Equal incidence in males and in emales. No known racial predilection

Question 3

interaction of autoantibody w what antigen

Answer 3

Interaction o autoantibody with BP antigen [BPAG1 (BP230) and BPAG2 (type XVII collagen)]inhemidesmosomeso basalkerati- nocytes (Fig. 6-1) is ollowed by complement and mast cell activation, attraction o neutro- phils and eosinophils, and release o multiple bioactive molecules rom in ammatory cells

Question 4

skin lesion

Answer 4

SKINLESIONS Erythematous,papular,or urticarial-type lesions (Fig. 6-13) may precedebullae ormationbymonths.Bullae: small (Fig. 6-13) or large (Fig. 6-14) tense,
rm-topped, oval or round; arise in normal, erythematous,orurticarialskinandcontain serous (Fig. 6-14) or hemorrhagic uid. Local- ized or generalized, usually scattered but also grouped in arci orm and serpiginous patterns. Bullae rupture less easily than in pemphigus,

Question 5

pedilsction

Answer 5

Axillae;medialaspects o thighs, groins, and abdomen; exor aspects o orearms; lower legs (o en rst mani esta- tion); generalized.
MUCOUS MEMBRANES Practically only in the mouth (10 to 35%)

Question 6

dermatpTh

other lab test result

Answer 6

DERMATOPATHOLOGY Light Microscopy. Neutrophilsin“Indian- le”alignmentat dermal–epidermal junction; neutrophils, eosinophils, and lymphocytes in papillary dermis;subepidermalbulla.
Electron Microscopy. Junctional cleavage, i.e.,splitoccursinlaminalucidao basement membrane (see Fig. 6-1).
IMMUNOPATHOLOGY Linear IgG deposits along thebasementmembranezone.AlsoC3,which mayoccurintheabsenceo IgG.
SERUM Circulating antibasement
membrane IgG antibodies detected by IIF

Question 7

tx

Answer 7

Systemicprednisonewithstartingdoseso 50 to 100 mg/d continued until clear, either alone orcombinedwithazathioprine,150mgdaily,
or remission induction and 50 to 100 mg
or maintenance; in re ractory cases, IVIG; plasmapheresis.Rituximabi.v.e ectiveinsome cases,whereasinothersithelpssparingcorti- costeroids. In milder cases, sul ones (dapsone), 100 to 150 mg/d. Low-dose methotrexate 2.5 to10mgweeklyPOise ectiveandsaein theelderly.Inverymildcasesand orlocal recurrences, topical glucocorticoid or topical tacrolimus therapy may be bene cial. etracy- cline ± nicotinamide has been reported to be e ectiveinsomecases.