Case Number : Case 2334 - 28 May 2019 Posted By: Uma Sundram

Leukocytoclastic vasculitis + perivascular IgA = Henoch-Schonlein Purpura. Actually that's all I can think of right now, but I'm sure there are many other options.

epidermis and dermis

dermal vessel wall damage with neutrophils and neutrophil debri

DIF showing perivascular IgA I am surprised

correct me, I thought that IgA would deposit in the vessel walls

so also the age. the other thing is any history of bowel disturbances, and palmar rashes

I would like to keep atypical Dermatitis herpetiformis

22 hours ago, vincenzo said:

Leukocytoclastic vasculitis + perivascular IgA = Henoch-Schonlein Purpura. Actually that's all I can think of right now, but I'm sure there are many other options.

Agree with Vincenzo.

I can´t think of anything else but IgA vasculitis of adults. Maybe I am missing something... waiting for the conclusion.

Agree with the comments above. Waiting to learn more differentials.

Yes, I gave you all an easy one this time, but also wanted to point out that we are learning a lot now about IgA vasculitis as a community of practitioners. The patient came in with acute kidney injury and the rash developed on the legs first. The rash on the legs faded and a similar rash appeared on the arms with palmar involvement. What was called Henoch Schonlein purpura is now called IgA vasculitis. It is vastly more common in children than in adults. Interestingly, this patient had kidney and skin findings, but not GI or other organ involvement. She also had brittle diabetes which complicated the kidney findings. An excellent paper from cleveland clinic shows that the presence of perivascular and vessel wall deposition of IgA correlated very well with the clinical diagnosis of IgA vasculitis (HSP) in the context of LCV (Feasel P, 2018). I had not realized that this triad (clinical findings + IgA deposition + LCV) was so powerful, so wanted to share it with all of you. Also important to know that the bx has to be done fairly early (within 48 hrs of rash development) as IgA fades quickly.

On 30/05/2019 at 09:45, Uma Sundram said:

Yes, I gave you all an easy one this time, but also wanted to point out that we are learning a lot now about IgA vasculitis as a community of practitioners. The patient came in with acute kidney injury and the rash developed on the legs first. The rash on the legs faded and a similar rash appeared on the arms with palmar involvement. What was called Henoch Schonlein purpura is now called IgA vasculitis. It is vastly more common in children than in adults. Interestingly, this patient had kidney and skin findings, but not GI or other organ involvement. She also had brittle diabetes which complicated the kidney findings. An excellent paper from cleveland clinic shows that the presence of perivascular and vessel wall deposition of IgA correlated very well with the clinical diagnosis of IgA vasculitis (HSP) in the context of LCV (Feasel P, 2018). I had not realized that this triad (clinical findings + IgA deposition + LCV) was so powerful, so wanted to share it with all of you. Also important to know that the bx has to be done fairly early (within 48 hrs of rash development) as IgA fades quickly.

Beautiful case. Thanks!