Case Number : Case 1729 - 12 January - Dr Arti Bakshi Posted By: Guest

Straight off her flight back from a well earned vacation Dr Arti Bakshi has a lovely case for you...

Geoff Cross - DermpathPRO Projects

Calciphylaxis. 

?In a patient with terminal lupus nephropathy

Looks to be dystrophic calcification in the septum with rather morphoeic change to the collagen.  How about the scleroderma spectrum of disorders? I was not sure I could locate the calcification in a vessel. Would need a bit more clinical/laboratory information to consider calciphylaxis.

This looks like dystrophic calcification with thickened collagen bundles and a few fibroblasts in the septum. May be Nephrogenic systemic fibrosis or Scleroderma. Clinical information is needed.

Thickened sclerotic subcutaneous septa with dystrophic calcification, and adnexal structures that seem to be entrapped favoring morphea/scleroderma spectrum.

Subcutaneous morphoea/morphoea profundus

My impression is deep morphea with dystrophic calcification. 

This was for me a difficult case and I took a while but I also thought of deep morphea as favoured diagnosis. The rash on the face and neck bothered me (not frequent in morphea to my knowledge, maybe they were early erythematous lesions of plaque morphea in this case?). In the cases of calciphylaxis that I have seen you find mainly small vessel calcification but a compromise of the whole panniculus and a richer inflammatory infiltrate.

My impression was that some small, intralobular, periseptal vessels were calcified. But maybe I was wrong...Waiting for the final diagnosis.

My initial impression (and report) was of deep morphoea too.

However, on further discussion with clinicians regarding the rash on face, I was told that the patient had been diagnosed as dermatomyositis and was on treatment for some years for this. (ofcourse all this was conveniently omitted on the histo request card!!). This raised the question whether the changes seen represent subcutaneous dystrophic calcification in association with dermatomyosistis. The patient does not have any other lesions or changes of scleroderma and this is an isolated solitary lump on the abdomen.  In order to have a single unifying diagnosis, the consensus was to regard this as subcutaneous dystrophic calcification secondary to DM.

I do agree that there is septal fibrosis in addition to calcification, which is difficult to explain (unless a secondary process). Although a coexistent solitary lesion of morphoea profunda remains a possibility, on checking the followup information today (the case is 2 yrs old), there has been no progression and no other lesions of morphoea have appeared.