This is not something that I have seen before. The biopsy was sent in by a rheumatologist from the left leg of 20 year old female. Interestingly, the rheumatologist said nothing else on the requisition sheet. So I got the chart. It turns out the patient has reflex sympathetic dystrophy (complex regional pain syndrome, type I) and has been seen and treated for years in the Pain Clinic here. And it turns out the patient has leg ulcers in the affected limb and the biopsy was taken to "rule out vasculitis". This is turning out to be difficult, as there is vasculitic change, but I cannot tell whether it is primary or secondary.

I guess the entry on this condition in Wikipedia is reasonable ..

There are some papers on this in the dermatology literature [1-5]. Ulcerations can occur in the skin in this condition [4,5]. I cannot find reports on vasculitis as a complication in the involved skin. Complex regional pain syndrome is mentioned in Andrews' Diseases of the Skin Clinical Dermatology, 9th and 10th editions. The condition is not mentioned in dermatopathology textbooks.

References:

1. Webster GF, Schwartzman RJ, Jacoby RA et al. Reflex sympathetic dystrophy. Occurrence of inflammatory skin lesions in patients with stages II and III disease. Arch Dermatol. 1991 Oct;127(10):1541-4. PMID: 1929462

2. Webster GF, Iozzo RV, Schwartzman RJ et al. Reflex sympathetic dystrophy: occurrence of chronic edema and nonimmune bullous skin lesions. J Am Acad Dermatol. 1993 Jan;28(1):29-32. PMID: 8425967

3. Shelton RM, Lewis CW. Reflex sympathetic dystrophy: a review. J Am Acad Dermatol. 1990 Mar;22(3):513-20. PMID: 2097997

4. Lipp KE, Smith JB, Brandt TP, Messina JL. Reflex sympathetic dystrophy with mutilating ulcerations suspicious of a factitial origin. J Am Acad Dermatol. 1996 Nov;35(5 Pt 2):843-5. PMID: 8912601

5. Sundaram S, Webster GF. Vascular diseases are the most common cutaneous manifestations of reflex sympathetic dystrophy. J Am Acad Dermatol. 2001 Jun;44(6):1050-1. PMID: 11369923

P.S. This was originally posted to the Dermpath-l mailing list July 18, 2008.