Bullous pemphigoid

Clinical and laboratory features[2]Yeh SW, Ahmed B, Sami N, et al. Blistering disorders: diagnosis and treatment. Dermatol Ther. 2003;16:214-223. http://www.ncbi.nlm.nih.gov/pubmed/14510878?tool=bestpractice.com

• In the prodromal, non-bullous phase, pruritus with eczematous or urticarial lesions for weeks to months.

• In the bullous stage, tense vesicles or bullae on apparently normal or erythematous skin of the pre-existing eczematous or urticarial eruption.

• Blisters may be several centimetres in diameter with a clear serous fluid that may become bloody after several days.

• If the blisters burst, the eroded, crusty areas slowly heal to leave post-inflammatory hyperpigmentation with occasional milia.

• Lesions are symmetrical and favour the flexural aspects of the extremities, lower trunk, and abdomen.

• Oral lesions present in up to 30% of patients.

• A biopsy for light microscopy typically shows sub-epidermal blister with a variably dense dermal inflammatory cell infiltrate rich in eosinophils.

• The blister cavity often contains a fibrin mesh and eosinophils.

• Direct immunofluorescence of peri-lesional uninvolved skin shows a linear band of IgG and/or C3 (and rarely other Ig classes) along the basement membrane zone.

• Indirect immunofluorescence testing of serum shows a positive titre for antibodies to bullous pemphigoid antigens.

Karnofsky Performance Status Scale definitions rating (%) criteria

This scale classifies patients according to their functional impairment. It is scored as follows.

Able to carry on normal activity and to work; no special care needed

• 100%: normal no complaints; no evidence of disease

• 90%: able to carry on normal activity; minor signs or symptoms of disease

• 80%: normal activity with effort; some signs or symptoms of disease.

Unable to work; able to live at home and care for most personal needs; varying amount of assistance needed

• 70%: cares for self; unable to carry on normal activity or to do active work

• 60%: requires occasional assistance, but is able to care for most personal needs

• 50%: requires considerable assistance and frequent medical care.

Unable to care for self; requires equivalent of institutional or hospital care; disease may be progressing rapidly

• 40%: disabled; requires special care and assistance

• 30%: severely disabled; hospital admission is indicated although death not imminent

• 20%: very sick; hospital admission necessary; active supportive treatment necessary

• 10%: moribund; fatal processes progressing rapidly

• 0%: dead.