Case Number : Case 2841- 27 May 2021 Posted By: Saleem Taibjee

?leishmaniasis 

? Perniosis/Chilblains - especially if it fits with a special hobby from the history.  

I am struck by the angiocentricity of the infiltrate, and also worried by the cytology of many of the cells.  Despite the age of the patient I would want to be sure that we are not dealing with angioimmunoblastic T-cell lymphoma.  I see the granularity but doubt whether Leishmaniasis would be so angiocentric.  Chilblains is also a good thought from the clinical history, but the pleomorphism of the cells would put me off that diagnosis.

superficial and deep dermal perivascular lymphocytes and plasma cells-

IHC to know the clonality of the cells is needed

Interim comment: Yes, I did think there are atypical / larger cells within the infiltrate when reporting this case. What immunopanel / differential diagnosis should be considered?

I dont see subcutaneous fat involvment

to start with CD3,  Cd20, CD 30 [ Lymphomatois papulosis type E], CD56, EBVNA

The atypical lymphocytes appear large like chunks of coal. CD30 positive atypical lymphocytes have been described in some cases of perniosis.

MPO CD34  CD56  CD68, CD33 CD99 to rule out myelo/monocytic leukemia. 

It's a very well-sampled biopsy - with a strikingly dense superficial and deep perivascular and interstitial abnormal infiltrate. It consists of medium to large-sized haematolymphoid-looking cells with irregular outline. Agree with Dr Logan, looks quite worrisome to me. I would wish to exclude extranodal NK/T-cell lymphoma, nasal type which classically shows angiocentric atypical lymphocytes (should be EBER+ CD56+ cytotoxic markers+ e.g. TIA1). May have nasal mass clinically. More common in East-Asian descent. DDx is Hydroa vacciniforme-like lymphoproliferative disease (similar; requires clinical correlation). Other lymphomas with angiocentric growth include primary cutaneous aggressive epidermotropic CD8-positive T-cell lymphoma (no epidermotropic infiltrate in the provided images?) and primary cutaneous γδ T-cell lymphoma (no subcutaneous infiltrate in the provided images).

Or some pseudolymphoma requiring clinical correlation? Not very cold here, so we have less chilblain cases. Can they show such atypical cells?

Apologies for the delay on this case. Here are some selected immunos below:

Cd123, MPO positive suggests a hemolymphoid neoplasm, but morphology does not suggest a BPCN

i dont know let me wait

This recent case was quite perplexing. As you have all noted, there were undoubtedly large atypical cells within the infiltrate. However, although difficult to demonstrate on the immuno images, there was not one consistent immunostain labelling all of these cells. A few of the larger cells seemed to stain for CD30 (and I wondered about a folliculotropic lymphoproliferative disorder such as LyP), perhaps a few for CD20, perhaps a few for CD123, perhaps even a few for MPO. But nothing consistent. EBV-ISH was negative ruling out some of the other considerations such as angioimmunoblastic lymphoma.

In asking Alistair Robson for his opinion, he had noted the infiltrate appeared to becoming more dense on the subsequent immunos, and hence we cut another H&E (see below). Lo and behold, this now looked like a more straightforward neutrophilic folliculitis, albeit with a few scattered atypical cells. Furthermore the clinician indicates that the lesions have fully settled with antibiotics as well as a short course of prednisolone, suggesting that either this was a bacterial folliculitis or acneiform process. It just goes to show how atypical the cells can be in reactive/inflammatory processes. In effect this case was resolved by cutting deeper into the biopsy, and is another example of the potential pitfall of examining punch biopsies by embedding whole rather than bisecting.

"Deeper cuts are the best stain" (Rapini)

See: Case 849  18.9.13  - hidradenoma papilliferum could be missed by superficial biopsy

        Case 917  24.12.13 - molluscum contagiosum masquerading as lymphoma

        Case 949 11.2.14 - scabies not obvious on first sections

        Case  2182  19.10.18 - digital papillary adenocarcinoma

        Case 2611  9.7.20 - scabies again

        Case 2841 27.5.21 - this case

A very educative case. Thanks. 

Thanks sir , for making us to be grounded