Case Number : Case 1789 - 06 April - Dr Richard A Carr Posted By: Guest

Dr Arti Bakshi is away on leave, so as a special treat we have a great case from Dr Richard A Carr, with Lots of IHC/Specials and Additional H&E from Biopsy B. at 6pm.

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Very difficult for me! I see some subtle dermal features of purpuric lesion, as extravasated red cells and hemosiderin deposits, associated with epidermalspongiosis and MF-like lymphocytic epidermal infiltrate...but much subtle...However I have a strong impression of Pigmented Purpuric Dermatosis ( lichen aureus might be present on trunk ).

The histopathological features r subtle for MF but the clinical description in such anatomical location is worrisome and worths further verification to exclude MF. More biopsies or IHC CD2,3,5 and 7. Clinical evaluation is also mandatory. As for the purpura in the dermis, purpuric MF can be the case. 

Agree with Mona's comment 

Agree- subtle/ difficult case. Not convinced for either PPD or MF. Some of the cells do look like mast cells to me. Cutaneous mastocytosis? Anyway this was my first thought. Might be barking down the wrong tree of course!! :)

Here the clinical information is very important (pictures if possible) Once I got a "rash/eruption" and when we called it was limited to the leg. If this was the case a lichenoid purpura is a good first differential. Mycosis fungoides (no epidermotropismus in these pictures, probably a little tagging at the EJ junction, no wiry collagen) simulating the picture of lichenoid purpura as mentioned previously is also posible. I would add IHC and clonality. Third drug reaction (Tirosine kinase can show this pattern with RBC and Gleevac MF like lesions or with RBC as mentioned at the last ISDP meeting)

PPD is my impression, but I think Mona´s comments fits very well to this case.

Agree my impression was also PPD vs PPD-like MF. Drugs can never be ruled out in cases like this even though we do not see eosinophils in this case

Agree with Mona's comment

ok   This might be a ppd-like MF, but the best spot diagnosis without other histology and IHC and molecular tests is for me. PPD

The lower legs are the preferred site of the persistent pigmented purpuric dermatoses, but lichen aureus may occur on the trunk, as Vincenzo pointed out. The very small focus of lymphocyte tagging along the dermoepidermal junction, with few necrotic keratinocytes, seems insufficient for mycosis fungoides. Bearing in mind that persistent pigmented purpuric dermatitis and mycosis fungoides may be biologically related, I prefer wait for new images thinking of the question raised by Toro et al: those two entities are simulant, precursor, or both?

Tricky case. Nothing diagnostic on this biopsy. Agree with the considerations and comments, PPD, Pupuric MF and drugs. Waiting for further images.

32 minutes ago, Nitin Khirwadkar said:

Tricky case. Nothing diagnostic on this biopsy. Agree with the considerations and comments, PPD, Pupuric MF and drugs. Waiting for further images.

New Images added, enjoy. Geoff

OK! PPD-like MF. IHC shows antigens loss, consistent with MF...but last HE img speaks more than IHC for me ( lamellar fibrosis and linear lymphocytic arrangement )!

Purpuric mycosis fungicides with the new images.

Agree that the last image is more important than the immunos. Infiltration of the epidermis by irregular lymphocytes, no edema, lymphocytes seem to be predominantly CD8+ with heterogeneous loss of CD7. CPC is still important and If molecular studies are available I would perform them.

Lovely ... 

Yes, immunohistochemical profile is consistent with lichen aureus-like mycosis fungoides.

I'm still thinking of persistent pigmented purpuric dermatitis and mycosis fungoides: simulant, precursor, or both?

Agree, last picture also helps a lot. PPD-like MF with CD8+ cells.

MF, PPD-like

This case proves to me the remarkable quality of responses we get on dermpathpro cases. You have all nailed the case from beginning to end. I reported A. as in keeping with PPD but given the findings in B. likely to represent, at the least, co-existent CD8+ MF (CD5 was also lost, not shown). Of course on the initial images CPC and r/o drugs are absolutely required as discussed fully above. I did not feel molecular studies would add anything (might even add confusion) and as it turned out there was a 20 year history and the patient was referred to a national expert clinician who confirmed the clinical features of early stage MF. Obviously demonstrating the same clone on the two separate biopsies would have been supportive but probably not necessary given the overall picture of Clinical-Histology-IHC.

I'm off to London this early morning to enjoy Eduardo Calonje's wonderful annual up-date in dermatopathology, opposite the Palace of Westminster in the depths of St Thomas' Hospital. Looks like the weather is set fair too. Hope to see some dermpathpro friends there today.

1 hour ago, Dr. Richard Carr said:

This case proves to me the remarkable quality of responses we get on dermpathpro cases. You have all nailed the case from beginning to end. I reported A. as in keeping with PPD but given the findings in B. likely to represent, at the least, co-existent CD8+ MF (CD5 was also lost, not shown). Of course on the initial images CPC and r/o drugs are absolutely required as discussed fully above. I did not feel molecular studies would add anything (might even add confusion) and as it turned out there was a 20 year history and the patient was referred to a national expert clinician who confirmed the clinical features of early stage MF. Obviously demonstrating the same clone on the two separate biopsies would have been supportive but probably not necessary given the overall picture of Clinical-Histology-IHC.

I'm off to London this early morning to enjoy Eduardo Calonje's wonderful annual up-date in dermatopathology, opposite the Palace of Westminster in the depths of St Thomas' Hospital. Looks like the weather is set fair too. Hope to see some dermpathpro friends there today.

St Thomas' Hospital, the last time I was there was for the birth of my eldest daughter Martha, 27 years ago  :-)