Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis

Subbanna, Praveen Kumar Arinaganhalli; Singh, Negi Vir; Swaminathan, Rathinam Palamalai

doi:10.4103/2229-5178.93488

Cited by 14 publications

(16 citation statements)

References 9 publications

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“…This concept receives further support by noting that a disproportionately high rate of "isolated vasculitis" occurs in the male genital tract (testis, epididymis, and spermatic cord) compared with other organ systems in which isolated vasculitis is more rare than involvement by systemic vasculitis. [54][55][56][57][58][59][60][61][62][63][64][65][66][67] In 12 cases, varying combinations of testicular hemorrhage, necrosis, and reactive changes in residual germ cells caused concern on the part of referring pathologists for germ cell neoplasia, and in some cases a regressed germ cell tumor, a possibility enhanced by the well-known tendency of testicular tumors to undergo spontaneous regression. 33 These cases differed, however, from a necrotic neoplasm by showing numerous ghost outlines of necrotic, germ cell-containing seminiferous tubules rather than the unorganized coagulum containing remnant tumor cells that typify a necrotic neoplasm.…”

Section: Discussionmentioning

confidence: 99%

Testicular Hemorrhage, Necrosis, and Vasculopathy

Kao

Zhang

Ulbright

2014

American Journal of Surgical Pathology

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We report 30 cases of testicular hemorrhage and/or necrosis with associated vascular damage that caused clinical concern for a neoplasm and that raised the question of a vasculitis syndrome on pathologic examination. The patients were 12 to 66 years old (median, 33 y) and presented with pain (n=15), mass (n=12), or both (n=2); 1 case had no available clinical information. Ultrasonographic interpretations included a neoplasm in the differential diagnoses in 14 of 18 cases in which this information was available, and most (n=24) had orchiectomy because of this possibility. Only 4 were clinically suspected to represent testicular infarction. Circumscribed, hemorrhagic lesions occurred in 10 cases, less demarcated hemorrhagic foci in 5, and discrete nodules or ill-defined foci of varying color and consistency in the remainder. No clear testicular lesion was described in 2, with 1 of these having a "dusky" appearance. On microscopic examination all but 1 case showed damaged blood vessels (vasculopathy), with either associated hemorrhage/hematoma (n=24) and/or areas of parenchymal necrosis (n=21). One case showed only segmental tubular atrophy with interstitial inflammation and vasculopathy; no infarct or hemorrhage was identified. A variety of vascular changes was identified, including prominent intimal thickening in arteries (n=22) and fibrinoid change in both arteries (n=5) and vessels of indeterminate type (n=8). Medial fibrosis was present in veins (n=12) and vessels of indeterminate type (n=4), whereas thrombi (remote, recanalized, and/or recent) occurred in arteries (n =7), veins (n=9), and vessels of indeterminate type (n=11). Dilated, blood-filled vessels were present in the testis and/or paratestis in 15 cases. In addition, 7 cases showed arteriolar hyalinization, and 19 had inflammation of blood vessels. The latter was lymphohistiocytic and mostly light but occasionally prominent (n=5). Interstitial inflammation was seen adjacent to damaged testicular parenchyma in all 30 cases. Clinical follow-up in 20 patients (4 to 131 mo, mean 38 mo) showed no evidence of recurrence in the contralateral testis or later development of systemic vasculitis. The histologic findings were compared with those in 11 orchiectomies resected for clinical acute torsion. All clinical acute torsion cases showed both parenchymal and fibrinoid vascular necrosis, and 10 had hemorrhage/hematoma; they lacked vasculitis, interstitial inflammation, and chronic vascular changes. Testicular vasculopathy, characterized by acute and chronic vascular injury, commonly occurs in testes with parenchymal hemorrhage and necrosis that clinically mimic a tumor. It shares the acute features of recent torsion but also has findings indicative of chronic injury. Testicular vasculopathy is most likely a result of chronic intermittent torsion that leads to localized hemorrhage/necrosis and should be distinguished from cases of systemic vasculitis given the significantly different clinical implications.

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Section: Discussionmentioning

confidence: 99%

Testicular Hemorrhage, Necrosis, and Vasculopathy

Kao

Zhang

Ulbright

2014

American Journal of Surgical Pathology

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“…The diagnosis of CPAN is based on clinical features of isolated skin involvement confirmed by histopathological findings (5). Deep, surgical incisional biopsies are essential for the correct diagnosis of CPAN.…”

Section: Cpan Was Initially Described In 1931 Bymentioning

confidence: 99%

“…Nodular vasculitis and thrombophlebitis can be clinically or pathologically mistaken for CPAN (4). The diagnosis is based on skin biopsy, as there are no specific serological tests (5).…”

Section: Introductionmentioning

confidence: 99%

Cutaneous Polyarteritis Nodosa: Uncommon and Rare Form of Cutaneous Vasculitis

Lekić

Milčić

Popadić

et al. 2018

Serbian Journal of Dermatology and Venereology

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Cutaneous polyarteritis nodosa (CPAN) is a variant of polyarteritis nodosa that is limited primarily to the skin. It is a chronic recurrent disorder characterized by the presence of nodular lesions with or without ulceration on the distal third of the lower limbs. Nodular vasculitis and thrombophlebitis can be clinically or pathologically mistaken for CPAN. We present a case of a 51-year-old woman with painful nodules on the lower limbs. Some of the nodules were ulcerated. Histopathological examination of a nodule on deep incisional biopsy revealed fibrinoid necrosis of a medium-sized artery in the subcutis along with perivascular mixed infiltrate. The patient did not have any symptoms or signs of internal organ involvement. The possible etiological factor has not been detected. The patient was treated with oral prednisone 0.5 mg/kg/day and dapson 150 mg/day. Over the one-year follow-up the lesions showed regression, with one minimal relapse which resolved after the short course of oral prednisone.

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“…2 However, digital gangrene as the initial presentation of the disease is rare. Two cases have been reported by Subbanna et al 3 and Choi et al, 4 where patients of CPAN initially presented with digital gangrene of the upper limbs. Breast involvement in CPAN is not a common manifestation.…”

mentioning

confidence: 95%