Case Number : Case 1893 - 30 Aug - Dr Richard Carr Posted By: Guest

The differential dx is large, but I thought strongly about Lyme disease in this case. Did the "fibromyalgia" start after the skin lesions? Have to perform special stains in cases like this one, eventually PCR.

The last image reminds me of Castleman's disease?? Hyaline vasclar type?? Might be wrong though!

Rosai-Dorfman disease.

Difficult case.  Histologically is consistent with Rosai-Dorfman Disease. Alternating  plasma cells rich dark areas and atypical histiocites rich light areas. The histiocytes are RDD typical cells. But there’s something disturbing: many eosinophils and Castleman like lymphoid follicles. And an odd clinical history.  

However agree with dermpath1   RDD is my first spot...surely the most difficult case of RDD I have ever seen (if am not wrong). 

Nodular lymphoid infiltrates with plasma cells and eosinophils with angiocentric arrangement.Fibromyalgia, arthralgia and a fixed rash suggest lymphomatoid granulomatosis. Although lymphomatoid granulomatosis presents with nodules or plaques, macular lesions are also reported

What do you think about IgG4-related disease? I don't have experience with cutaneous lesions.

I thought about the possibility of cutaneous Castelman's disease vs cutaneous plasmacytosis, if one consider these separate entities.

It is rare in Caucasians, would be interesting to know results of serum protein electrophoresis and futher IHC plus complete staging for evidence of systemic disease.

Clinical picture of red/brown lesions is suggestive as well, especially macules and thin dermal plaques mainly. Site usually trunk but extremities can be involved.

Very interesting case, not sure if one "Spot diagnose"such cases!

3 hours ago, vincenzo polizzi said:

Robledo F. Rocha  336

Posted 3 hours ago · Report

What do you think about IgG4-related disease? I don't have experience with cutaneous lesions.

• Ok Robledo.   Your thought is very interesting.   I thought of an association between RDD and IgG4-RD, after your comment.  Obliterative lymphocytic  phlebitis and abortive lymphoid follicles could be better justified!
• But the eosinophils? So I started thinking of an association between RDD and LCH...So this case might be so confusing or is my mind a bit confusing?...The only clear thing for me is the component RDD-like

 

Dear Vincenzo, eosinophils can be found in the inflammatory infiltrate of IgG4-related disease. Indeed, eosinophils count per high power field can reach an amount as large as 289!

Dear Robledo, thanks for your useful comment. 

About eosinophils in RDD foundthis

http://www.archivesofpathology.org/doi/full/10.5858/arpa.2010-0554-CR

It is funny that eosinophils are quite prominent in the first biopsy and then we barely see them in the following samples

No idea!! Some odd fixed lymphomatous drug reaction with eos?

Some great suggestions. All accept RDD and Castleman's we already did consider!  Prof. Werner Kempf kindly performed PCR studies for Lyme and all the viruses we could think off (HSV/VZV, EBV etc), IgG4 was not particularly in excess. Our final conclusion was some sort of pseudolymphoma/reactive plasmacytosis akin to APACHE (the latter suggested by Werner). Sometimes skin cases are exceedingly frustrating and clearly given that the patient (who must think we are stupid) submitted to 3 biospies it's curious we cannot give a better answer!

11 hours ago, Dr. Richard Carr said:

Some great suggestions. All accept RDD and Castleman's we already did consider!  Prof. Werner Kempf kindly performed PCR studies for Lyme and all the viruses we could think off (HSV/VZV, EBV etc), IgG4 was not particularly in excess. Our final conclusion was some sort of pseudolymphoma/reactive plasmacytosis akin to APACHE (the latter suggested by Werner). Sometimes skin cases are exceedingly frustrating and clearly given that the patient (who must think we are stupid) submitted to 3 biospies it's curious we cannot give a better answer!

Wow! Thanks for the interesting case. Glad to know that others see such cases too. 

Thank you!

Just now, Dr. Richard Carr said:

Thanks Sasi - Reactive cutenous lymphoid hyperplasia with plasmacytosis, APACHE-like (aetiology remains obscure).

On 8/31/2017 at 20:30, Dr. Richard Carr said:

Some great suggestions. All accept RDD and Castleman's we already did consider!  Prof. Werner Kempf kindly performed PCR studies for Lyme and all the viruses we could think off (HSV/VZV, EBV etc), IgG4 was not particularly in excess. Our final conclusion was some sort of pseudolymphoma/reactive plasmacytosis akin to APACHE (the latter suggested by Werner). Sometimes skin cases are exceedingly frustrating and clearly given that the patient (who must think we are stupid) submitted to 3 biospies it's curious we cannot give a better answer!

Thanks!