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Previous Volume 354:2393-2394 June 1, 2006 Number 22 Next

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To the Editor: The Medical Mystery in the April 6 issue¹ involved a 62-year-old man who presented with a one-month history of digital gangrene in the right hand (Figure 1A). The skin of his arms and legs had mottled discolorations with palpable cutaneous nodules (Figure 1B). A deep incisional-biopsy specimen from a cutaneous nodule revealed occluded middle-sized arteries with lymphoplasmacellular and neutrophilic infiltration (Figure 1C). On the basis of the clinical and histologic findings, the diagnosis of cutaneous polyarteritis nodosa was made. The patient underwent amputation of the necrotic regions. During glucocorticoid treatment, new cutaneous nodules occurred. Cyclophosphamide treatment was effective in the control of the disease. At one year, the patient was in complete remission, and the immunosuppressive therapy was tapered down.

View larger version (80K): [in this window] [in a new window]   Figure 1. Polyarteritis Nodosa in a 62-Year-Old Man. Panel A shows digital gangrene affecting the right hand, Panel B discoloration of the legs in a net-like pattern with palpable cutaneous nodules,and Panel C a biopsy specimen of a nodule (hematoxylin and eosin).

 

Stefan Schanz, M.D.
Anja Ulmer, M.D.
University of Tuebingen
D-72076 Tuebingen, Germany

Editor's note: We received 918 responses to this Medical Mystery from 74 countries. Of those responses, 59 percent were from physicians in practice, 24 percent from physicians in training, 11 percent from medical students, and 6 percent from other readers. As in past Medical Mysteries, many of the responses reflected a team effort, such as those submitted by physicians in a few residency programs after they had discussed the case in a teaching conference. One father–daughter duo worked through this case as a family activity.

Forty-four percent of the responses correctly identified polyarteritis nodosa, a necrotizing vasculitis that often involves medium-sized arteries. Many of the responses also emphasized the cutaneous variant that this patient had. Seventeen percent of respondents implicated other types of vasculitis, including microscopic polyangiitis, Wegener's granulomatosis, the Churg–Strauss syndrome, Takayasu's arteritis, giant-cell arteritis, and vasculitis secondary to connective-tissue disease. Other common responses included thromboangiitis obliterans (Buerger's disease), the antiphospholipid-antibody syndrome, cholesterol emboli, leprosy, sarcoidosis, and scleroderma.

Important clues to the correct diagnosis of polyarteritis nodosa include the findings of cutaneous nodules, digital infarction (Figure 1A), livedo reticularis (Figure 1B), and vasculitis on histologic evaluation (Figure 1C).

References

1. Schanz S, Ulmer A. A medical mystery -- gangrene and cutaneous nodules. N Engl J Med 2006;354:1515-1515. [Free Full Text]

PDF PDA Full Text Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited E-mail When Letters Appear Related Article by Schanz, S. PubMed Citation