Case Number : Case 4629 - 05 August 2025 Posted By: Admin_Dermpath

prebullous pemphigoid 

I presume the description "since 8-9 years" really means "for" 8-9 years, rather then "since the age of 8-9 years", which of course raises other possible diagnoses, however unlikely.

There are intra-epidermal vesicles containing eosinophils, but no suggestion of a sub-epidermal split.  I don't think the intra-epidermal position of the vesicles is due to re-epithelialisation after healed blistering.  Such eosinophil-containing vesicles can be seen in the pre-bullous phase of pemphigoid but it would be unusual for this phase to persist for 8-9 years without "maturing" into overt bullous pemphigoid.  Obviously immunofluorescence would clinch the diagnosis.  If negative, then alternative diagnoses would have to be considered such as an "eczematous" drug reaction, insect bites etc.

I would wouldnt rule out BP and Duhring but how about prurigo pigmentosa (nagashima)? For BP and Duhring the split is not in the right place (subepidermal). Would like to see DIF as the first two have pretty distinct patterns and the third is negative (i think)

DIF showed intercellular positivity with IgG and C3. Thus, the diagnosis is pemphigus herpetiformis which usually shows eosinophilic and/or neutrophilic spongiosis with intraepidermal vesicles / pustules, while acantholysis may be inconspicuous in many cases.

On 18/08/2025 at 14:50, Richard Logan said:

I presume the description "since 8-9 years" really means "for" 8-9 years, rather then "since the age of 8-9 years", which of course raises other possible diagnoses, however unlikely.

There are intra-epidermal vesicles containing eosinophils, but no suggestion of a sub-epidermal split.  I don't think the intra-epidermal position of the vesicles is due to re-epithelialisation after healed blistering.  Such eosinophil-containing vesicles can be seen in the pre-bullous phase of pemphigoid but it would be unusual for this phase to persist for 8-9 years without "maturing" into overt bullous pemphigoid.  Obviously immunofluorescence would clinch the diagnosis.  If negative, then alternative diagnoses would have to be considered such as an "eczematous" drug reaction, insect bites etc.

Yes. It is for 8-9 years, some grammatical errors while writing the history.