Search

Churg-Strauss syndrome (CSS) is a systemic vasculitis affecting both small- and medium-sized blood vessels, almost invariably affecting the lung, and frequently associated with cutaneous involvement. Microvascular vaso-occlusion leading to digital gangrene is not a feature of CSS. We report an unusual case of a patient with CSS with antiphospholipid antibodies who developed severe digital gangrene in addition to cutaneous vasculitis. The presence of antiphospholipid antibodies is not a feature usually seen in association with CSS. While the full clinical spectrum of CSS is still being defined, the identification of additional features associated with this syndrome might help to better understand the pathogenesis of the disease and to have an impact on both management and prognosis.

Juvenile gangrenous vasculitis of the scrotum was described by Piñol et al in 1974 as a unique variant of scrotal gangrene of unknown origin, occurring exclusively in young individuals. It was characterized by an acute onset of skin ulcers undergoing complete resolution after appropriate therapy, with no relapses. We present a typical case of this extremely rare disease affecting a 16-year-old boy in whom the scrotal ulcerations were preceded by an episode of pharyngitis with fever. The condition promptly regressed after administration of intramuscular betamethasone in combination with oral ciprofloxacin. According to Piñol et al, juvenile gangrenous vasculitis of the scrotum, although poorly known to dermatologists, should be regarded as a distinctive entity within the wide group of scrotal gangrenes. On the other hand, the hypothesis that this condition may well represent a variant of pyoderma gangrenosum is discussed.

Background: Acral vascular syndromes associated with malignancy have rarely been reported., Objective: Our purpose was to assess the clinical and evolving features of paraneoplastic acral vascular syndromes., Patients and Methods: Two cases of paraneoplastic gangrene are described and analyzed together with previously reported cases identified by a MEDLINE search., Results: Among the 68 patients identified, 40 had gangrene, 16 had acrocyanosis, and 12 had Raynaud's phenomenon. The male to female ratio was 0.89; median age was 59 years. Fingers were affected in 94%. Adenocarcinomas were the predominant associated malignancies (41%), and metastases were observed in 41%. The acral vascular syndromes in 48% of the patients definitively regressed after tumor treatment. Forty-four percent of the patients died within 2 years. A favorable cutaneous outcome was obtained with prostacyclin infusions in 6 patients., Conclusion: A neoplastic origin of acral vascular syndrome should be considered in elderly patients, especially men, in the absence of usual causative conditions.

We studied 218 patients (372 limbs) and 25 normal subjects (50 limbs) with resting ankle index (RAI), treadmill exercise (TE), and postocclusive reactive hyperemia (PORH) to determine whether diagnostic accuracy is improved through the use of stress testing. In addition, we studied 10 patients with stable claudication (20 limbs) to determine the reproducibility of the three measures. RAI was the most reproducible measure, with the smallest variance between testing days (P less than 0.001). RAI differentiated between arteriographically diseased and normal limbs with a sensitivity of 97% and a specificity of 100%, whereas the corresponding values for TE were 97% and 96% and for PORH 89% and 96%. Recovery to baseline index was prolonged in the diseased group compared with normal (p less than 0.001 for both TE and PORH), but this was of limited discriminative value. Receiver-operating characteristic curve analysis documented that RAI was as diagnostically useful as TE and that both were more valuable than PORH (P less than 0.02). However, the routine addition of stress testing increased diagnostic yield by only 1.6% and cost $1100 for each limb correctly diagnosed through the addition of stress testing. RAI is a simple, accurate, and reproducible test. Routine stress testing is not cost effective, adding little diagnostic information to RAI, and it should be reserved for the small subset of symptomatic patients with normal RAI.