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Skin infiltrate (832940)
Skin infiltratenew
Subtitle: serial biopsies
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SergeyN
2017-02-15 16:33
INCTR - EBMWG Hematopathology Online
A consultation case. Not my field, but I suppose both patient and dermatologist are desperate.  
Female patient, born in 1970.  
2013, 2014 and 2015: small skin nodules on chin and nasolabial fold, removed by laser. Biopsy (as remembered by patient, material and report lost) had shown "lymphocytic infiltration" or "lymphocytic inflammation". Pancreas dysfunction from childhood, thyreoectomy in 2003, currently climacteric.  
01.04.16 - rapid onset of prominent painless edema of the chin region. Antihistamine, antibiotics and local treatment without effect. 05.16 - Dexasone i/v with edema regression, immediate relapse one day after treatment. Peroral Prednisone 30mg/d commenced with good effect for 2 months, relapse at decreasing dosage to 15mg/d.  
17.07.16 - visit to a new dermatologist. Several hard subcutaneous nodules of various sizes and deepness, papular and papular-pustular elements, teleangioectasies; recurrent hard edema (mostly in the morning). Dermatoscopy - dilated capillaries, hyperplastic sebaceous glands. Dg: granulomatous rosacea with gnatophyma? chronic angioedema? granulomatous diseases (lupus, sarcoidosis, TBC, lepra)? Prednosone further decreased to 7.5mg/d, Metronidazole and local therapy added. The condition worsened, deep subcutaneous infiltrates expanded. Dermatoscopy showed apple jelly-like changes in some papules.  
29.07 Punch biopsy performed (see photos marked 1-…). Visible massive lymphoid infiltration expanding into deep derma, predominantly rather monotonous T-cell. Lymphocytoma reported. Unfortunately, there is no block for additional stains.  
Prednisone 2.5mg/d. Treatment with Metronidazole and local treatment - negative dynamics.  
29.08 - IgM to B. burgdorferi found. Doxocyclin for 20 days administered without positive effect: clinical worsening, increased edema, new dermal and hypodermal nodules, general condition intact. IgМ to B. burgdorferi persist, normal IgG titre.  
07.11 Repeated biopsy from a deeper infiltrate (photos marked 2-…). Pseudolymphomatous folliculitis reported. Retrospective immunohistochemistry showed CD4+ infiltrate. TCR rearrangement is not available here.  
Working diagnosis - rosacea with persistent solid edema (the attached photos of the patient taken at that moment, scars are from previous biopsies). Isotretinoin 20mg/d commenced, moderate positive dynamics. In 1.5 months - gradual local worsening with erosions, submandibular lymphadenopathy and subfebrile fever appeared for the first time. No effect from antibiotics, bacteriology negative, fungi negative.  
31.01.2017 - third biopsy (3 - …). Isotrenoin discontinued, local changes persist. Lymph node biopsy planned.  
 
Well, I am not a particular specialist in skin pathology, lymphomas included, and rely on your assistance. Could it be an exotic lymphoproliferation, after all?  
 
Next biopsy, both skin and regional node, marked 4s... and 4n.... The skin infiltrate looks very much like biopsy Nr 2 (a lot of necrosis this time, though). Lymph node is mostly reactive, but there are a lot of intrasinusoidal CD4+ T-cells.  
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gaulier
2017-02-15 19:08
The T cell mainly CD 4 infiltration looks monotonous with exocytosis, without spongiosis .I do not see typical Pautrier microabcesses but numerous hallowed lymphocytes are present in CMM.Important would be to perform Bleu Alcian staining to identify a possible follicular mucinosis focus.  
CD5 and CD 7 immunostaining are suitable to identify a lack of expression for those antibodies on T cells.  
A thin 3 microns section with an excellent MGG will help to look for nuclear abnormalities on lymphocytes.  
Are they atypical lyhmphocytes in the peripheral blood?  
On those different pictures, I cannot rule out the diagnosis of T cell lymphoma
tzankov
2017-02-16 10:07
I discussed the case with our Deramtologist Prof. Häusermann; neither he, nor me think that this is a lymphoma. Prof. Häusermann asks, if there were any cosmetic proceedures on the patient's face (hyaluronate?) and asks for additional (more) clinical photographies. Can you stain the case for CD2, CD5, CD7, PD1 and KI67?
SergeyN
2017-02-16 13:33
Many thanks for the prompt replies!  
There have been no cosmetic procedures, particularly with injected material. Except the mentioned laser removal of superficial nodules (the last procedure had been approximately 6 month before the onset).  
 
I shall stain for CD5 and CD7 (CD2 and PD1 are not available) and ask for additional photos.  
 
There are more immunostains for the second biopsy added, marked 2-... A lot of histiocytes (PG-M1+) that could be partly responsible for CD4+ stain, a lot of Langerhans cells, CD99 positivity (whatever that means) and many GaranzymeB positive cells (more than CD8 and CD56). Ki67 added, too.
SergeyN
2017-02-23 13:00
Additional photos uploaded (taken at the time of biopsy 2).
tzankov
2017-02-23 17:04
I discussed the case once again with our dermatologist and dermatopathologist Prof. Haeusermann. We think that this is a reactive process (at least from the macroscopic appearance - suggestive of rosacea) with secondary changes (probably due to manipulation). There are no signs suggestive of a neoplastic process.
SergeyN
2017-02-27 16:37
Thank you, Alex! A load off shoulders...  
 
To clean up the case, I've uploaded CD7 and Giemsa
SergeyN
2017-03-30 15:12
The next biopsy! Both skin and regional node this time, marked 4s and 4n.  
 
It was reported as lymphoma, the patient is being prepared for chemo...  
tzankov
2017-03-30 15:41
I see a CD4 predominant paracortical process that respects the basic lymph node architecture. I would not be able to esablish the diagnosis of a lymphoma on the preented materiel (even clonaliyt can be obsevred in dermatopathic lymphandenopathy)...can you stain the lymph node for PD1, ICOS, p53, CD30?  
 
The skin looks very like a Riga-Fede with a kind of necrosis, which may also be explicable by local or previous irritation. Can you please stain it for CD30, MUM1, MYC, CD2, CD7 and CD5?
SergeyN
2017-04-07 09:42
Dear Alex, we don't have CD2, PD1, ICOS and MYC. CD5, CD30, MUM1 are, of course, routine. CD7 is tricky, but I managed with previous biopsies (have a look at photos 2CD7 immediately after the patients's macro).  
Overall, the last skin looks very much like the second biopsy, only deeper, with necrotic zones more visible.  
I think CD5 and CD30 have been stained in all biopsies, too, but not uploaded, I shall check.
SergeyN
2017-04-07 09:42
Dear Alex, we don't have CD2, PD1, ICOS and MYC. CD5, CD30, MUM1 are, of course, routine. CD7 is tricky, but I managed with previous biopsies (have a look at photos 2CD7 immediately after the patients's macro).  
Overall, the last skin looks very much like the second biopsy, only deeper, with necrotic zones more visible.  
I think CD5 and CD30 have been stained in all biopsies, too, but not uploaded, I shall check.
SergeyN
2017-04-07 09:45
There was an idea to treat the patient as lymphoma, with chemo. Personally, I am not sure there is sufficient pathologic evidence for such radical measures.
tzankov
2017-04-10 06:51
dear Sergej, I don't think I can help any more, the final decision if and how to treat the lesion should be taken by the patient and the treating physician, for sure.
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Last modified: 2017-04-07 09:46:37