Case Number : Case 4019 - 16 June 2022 Posted By: Saleem Taibjee

Needs DIF. Given the history of celiac I'd guess IgA-related dermatitis herpetiformis.

Seems like partly excoriated or re-epithelized blister. There are significant neutrophils in dermal papillae. Needs DIF. 

acrodermatitis enteropathica vs other nutritional deficiency. 

Psoriasiform hyperplasia and upper epidermal pallor suggest a nutritional deficiency dermatosis, a result of malabsorption. Pellagra, necrolytic migratory erythema are the possibilities apart from acrodermatitis enteropathica or multiple deficiencies. Clinicopathological correlation will be needed. 

Interesting case. Initially from low power my thoughts were TEN. then i could see neuts in the dermal papillae 

the history tense blisters and distribution not so characteristic on the DH alone, usually small vesicles ...

acrodermatitis enteropathica usually around mouth and perianal skin , pellagra should be diarrhoea 

so may be combined pathology TEN and DH?

so CPC and IMF i would say ...

could it be necrolytic acral erythema

Great discussion / thoughts on this case, and this confirms it was worthwhile including this case.

This case illustrates the pitfall of biopsy/histology of a late/old blister. As Meenakshi has recognised, this is actually a re-epithelialised blister, and this accounts for the peculiar appearance of the epidermis and even the false impression of intra-epidermal/superficial clefting.

I have seen similar cases. For example, I recall one case which was reported and presented at a meeting as an unusual case of toxic epidermal necrolysis, but in reality I am sure was bullous pemphigoid in which the late stage of biopsy accounted for necrosis of the overlying epidermis.

Volha has recognised that tense blisters are very rarely seen in Dermatitis herpetiformis which is another important point in this case.

The clinician's impression was of bullous pemphigoid despite the younger age, and sometimes BP can be neutrophil-rich. Direct immunofluorescence was typical, with linear C3 and IgG within the basement membrane region.

BW

Saleem

she is young for BP ...

Yes, she is young for BP, but we do see cases.