Case Number : Case 2086 - 5 June 2018 Posted By: Uma Sundram

Galli-Galli disease

Interesting. Never heard of Galli Galli disease. Thank you.

What's about the clinical information:  recent 20 pound weight loss?

If the weight loss is an indication of malignancy, this acantholytic disorder could very well be paraneoplastic pemphigus.

Completely agree with Anil. Paraneoplastic Pemphigus was my first spot. Oral mucosa?

Also thought of paraneoplastic pemphigus

If Vincenzo and Raul both agree, I heave a sigh of relief! :-). I know I am on the right path!

My first thought was paraneoplastic pemphigus. The clue hinted a lot.

Thanks Anil, but do not trust me so much. I’m an old general pathologist trying to learn a little dermatopathology. Anyway thanks. At my age like compliments even if you don’t deserve :-)

Vincenzo, I am an old clinician with great interest in and little knowledge of dermatopathology. You have spent many more hours looking through the microscope than me and definitely know more than me!

I hope one day I will have as much knowledge and passion as you guys show in this and other forums. Thanks Vincenzo and Anil for sharing your precious points of view.

You all are wonderful to work with and I feel like I know you well :)

Thank you again for your passionate support of this forum. Yes, answer is paraneoplastic pemphigus. I like galli galli disease too (rare inherited disease of keratotic dysmaturation that has histologic overlap with the more hyperplastic lesions of pemphigus) but DIF in this case is positive (chicken wire intercellular pattern with IgG).  A separate serum sample (IIF) was submitted for both monkey esophagus and rat bladder assessment and both were positive. A systemic work up revealed DLBCL of the lymph node with pelvic lymph adenopathy; the patient had oral lesions as well.