Case Number : Case 1515 - 14 April Posted By: Guest

I see some naked granulomas within the dermis with perineural inflammation on last picture. The clinical findings of lung disease and hepatosplenomegaly may also be seen in cases of sistemic Sarcoidosis. So my first thought is Sarcoidosis, but I would do special stains before.

Leprosy-like Leishmaniasis

Non necrotizing dermal granulomatous inflammation with paucity of lymphocytes should make us put sarcoidosis in the differential. Special stains should be perform first of course but I think they will be negative. The clinical history fits with sarcoidosis although I think it would be important to find out if an immunodeficiency syndrome is suspected (like common variable immunodeficiency) in the patient as there can be some granulomatous dermal inflammation in these cases

Sorry, I meant to put in the history...the special stains were all negative.

Perineural inflammation is rare in sarcoidosis but it can be seen sometimes. I think that to be prudent, we should state that an infectious cause cannot be 100% ruled out even with negative special stains. CPC is of extreme importance in these kind of cases. My preferred diagnosis in this case are: Sarcoidosis/granulomatous inflammation due to immunodeficiency and far down I would put infectious etiology 

I have many doubts now. May be there are some Leishmania in my brain...

These are tuberculoid granulomas (lots of lymphocytes), following neurovascular bundles and adnexa.  Funny there is some leishmania-like granularity but suspect that is just a red herring. A high power would not go amiss though. DDx Sarcoid, lymphoma (can present with cutaneous granulomatous process completely mimicking sarcoid), good to consider immunodeficiency too. I've a sneaky suspicion will be a T-cell lymphoma or lymphomatoid granulomatosis.

Sarcoidosis.  In one series, leprosy-like perineural granulomas were present in a quarter of cases.  http://www.ncbi.nlm.nih.gov/pubmed/14690461.  Immunodeficiency diseases are possible, but given that these diseases are uncommon and that granulomas are uncommon in these diseases, I think they are a distant second. 

As far as I know, lymphomatoid granulomatosis shows an (1) angiocentric, (2) angiodestructive and (3) lymphocyte predominant infiltrate, none of which we have here.  Plus, lymphomatoid granulomatosis is not a nodal lymphoma and, therefore, lymphadenopathy is not a feature. 

Great discussion!

This is a case of granulomatous disease in association with Common Variable Immunodeficiency.

The patient had severe hypogammaglobulinemia at presentation 3 yrs back and has been on Ig replacement therapy. Biopsies from spleen and lymph node also showed non caseating granuloma.

Infectious aetiologies were ruled out clinically. 

The main differential was sarcoidosis (as there is a known association of sarcoidosis and CVID  and the patient had rasied ACE levels).  The distinction is difficult histologically (although I agree with Richard that the granulomata do show quite a bit of lymphocytic mantle). Based on clinical and radiological correlation, a diagnosis of granulomatous disease in association with CVID was favoured. (apparently raised serum ACE is not specific for sarcoidosis and can be seen with any granulomatous process). Patient was started on oral steroids (the recommended treatment for granulomas in CVID) and at the last visit, the skin lesions were healing well.

Forgot to mention....many thanks to my colleague, Dr Stefanos, for sharing this case and to Dr Darroch for his clinical input.

nice case