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The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features.

Loricera J, Calvo-Río V, Ortiz-Sanjuán F, González-López MA, Fernández-Llaca H, Rueda-Gotor J, Gonzalez-Vela MC, Alvarez L, Mata C, González-Lamuño D, Martínez-Taboada VM, González-Gay MA, Blanco R - Medicine (Baltimore) (2013)

Bottom Line: Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies.The most common underlying malignancy is generally hematologic.In these cases the presence of cytopenias and immature cells may be red flags for the diagnosis of cancer.

View Article: PubMed Central - PubMed

Affiliation: From the Divisions of Rheumatology (JL, VC-R, FO-S, JR-G, CM, VMM-T, MAG-G, RB), Dermatology (MAG-L, HF-L), Pathology (MCG-V), and Pediatrics (LA, DG-L), Hospital Universitario Marqués de Valdecilla, IFIMAV, Santander, Spain.

ABSTRACT
Cutaneous vasculitis may be associated with malignancies, and may behave as a paraneoplastic syndrome. This association has been reported in a variable proportion of patients depending on population selection. We conducted the current study to assess the frequency, clinical features, treatment, and outcome of paraneoplastic vasculitis in a large unselected series of 766 patients with cutaneous vasculitis diagnosed at a single university hospital. Sixteen patients (10 men and 6 women; mean age ± standard deviation, 67.94 ± 14.20 yr; range, 40-85 yr) presenting with cutaneous vasculitis were ultimately diagnosed as having an underlying malignancy. They constituted 3.80% of the 421 adult patients. There were 9 hematologic and 7 solid underlying malignancies. Skin lesions were the initial clinical presentation in all of them, and the median interval from the onset of cutaneous vasculitis to the diagnosis of the malignancy was 17 days (range, 8-50 d). The most frequent skin lesions were palpable purpura (15 patients). Other clinical manifestations included constitutional syndrome (10 patients) and arthralgia and/or arthritis (4 cases). Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies. Specific treatment for vasculitis was prescribed in 10 patients; nonsteroidal antiinflammatory drugs (4 patients), corticosteroids (3 patients), chloroquine (1 patient), antihistamines (1 patient), and cyclophosphamide (1 patient). Ten patients died due to the malignancy and 6 patients recovered following malignancy therapy. Patients with paraneoplastic vasculitis were older, more frequently had constitutional syndrome, and less frequently had organ damage due to the vasculitis than the remaining patients with cutaneous vasculitis. In summary, cutaneous paraneoplastic vasculitis is an entity not uncommonly encountered by clinicians. The most common underlying malignancy is generally hematologic. In these cases the presence of cytopenias and immature cells may be red flags for the diagnosis of cancer. In patients with paraneoplastic cutaneous vasculitis, the prognosis depends on the underlying neoplasia.

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Skin biopsy of a patient with neoplasia presenting with palpable purpura. Typical histologic findings consistent with leukocytoclastic vasculitis. Neutrophilic infiltration, leukocytoclasia, fibrinoid necrosis, and erythrocyte extravasation into the vessel wall of arterioles, capillaries, and postcapillary venules from dermis are visible. [This figure can be viewed in color online at http://www.md-journal.com].
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Figure 2: Skin biopsy of a patient with neoplasia presenting with palpable purpura. Typical histologic findings consistent with leukocytoclastic vasculitis. Neutrophilic infiltration, leukocytoclasia, fibrinoid necrosis, and erythrocyte extravasation into the vessel wall of arterioles, capillaries, and postcapillary venules from dermis are visible. [This figure can be viewed in color online at http://www.md-journal.com].

Mentions: Skin punch biopsy was performed in all 16 cases. The characteristic histologic findings, such as neutrophilic infiltration; leukocytoclasia; and fibrinoid necrosis into the vessel wall of arterioles, capillaries, and postcapillary venules, were observed in all of them (Figure 2).


The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features.

Loricera J, Calvo-Río V, Ortiz-Sanjuán F, González-López MA, Fernández-Llaca H, Rueda-Gotor J, Gonzalez-Vela MC, Alvarez L, Mata C, González-Lamuño D, Martínez-Taboada VM, González-Gay MA, Blanco R - Medicine (Baltimore) (2013)

Skin biopsy of a patient with neoplasia presenting with palpable purpura. Typical histologic findings consistent with leukocytoclastic vasculitis. Neutrophilic infiltration, leukocytoclasia, fibrinoid necrosis, and erythrocyte extravasation into the vessel wall of arterioles, capillaries, and postcapillary venules from dermis are visible. [This figure can be viewed in color online at http://www.md-journal.com].
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4553993&req=5

Figure 2: Skin biopsy of a patient with neoplasia presenting with palpable purpura. Typical histologic findings consistent with leukocytoclastic vasculitis. Neutrophilic infiltration, leukocytoclasia, fibrinoid necrosis, and erythrocyte extravasation into the vessel wall of arterioles, capillaries, and postcapillary venules from dermis are visible. [This figure can be viewed in color online at http://www.md-journal.com].
Mentions: Skin punch biopsy was performed in all 16 cases. The characteristic histologic findings, such as neutrophilic infiltration; leukocytoclasia; and fibrinoid necrosis into the vessel wall of arterioles, capillaries, and postcapillary venules, were observed in all of them (Figure 2).

Bottom Line: Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies.The most common underlying malignancy is generally hematologic.In these cases the presence of cytopenias and immature cells may be red flags for the diagnosis of cancer.

View Article: PubMed Central - PubMed

Affiliation: From the Divisions of Rheumatology (JL, VC-R, FO-S, JR-G, CM, VMM-T, MAG-G, RB), Dermatology (MAG-L, HF-L), Pathology (MCG-V), and Pediatrics (LA, DG-L), Hospital Universitario Marqués de Valdecilla, IFIMAV, Santander, Spain.

ABSTRACT
Cutaneous vasculitis may be associated with malignancies, and may behave as a paraneoplastic syndrome. This association has been reported in a variable proportion of patients depending on population selection. We conducted the current study to assess the frequency, clinical features, treatment, and outcome of paraneoplastic vasculitis in a large unselected series of 766 patients with cutaneous vasculitis diagnosed at a single university hospital. Sixteen patients (10 men and 6 women; mean age ± standard deviation, 67.94 ± 14.20 yr; range, 40-85 yr) presenting with cutaneous vasculitis were ultimately diagnosed as having an underlying malignancy. They constituted 3.80% of the 421 adult patients. There were 9 hematologic and 7 solid underlying malignancies. Skin lesions were the initial clinical presentation in all of them, and the median interval from the onset of cutaneous vasculitis to the diagnosis of the malignancy was 17 days (range, 8-50 d). The most frequent skin lesions were palpable purpura (15 patients). Other clinical manifestations included constitutional syndrome (10 patients) and arthralgia and/or arthritis (4 cases). Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies. Specific treatment for vasculitis was prescribed in 10 patients; nonsteroidal antiinflammatory drugs (4 patients), corticosteroids (3 patients), chloroquine (1 patient), antihistamines (1 patient), and cyclophosphamide (1 patient). Ten patients died due to the malignancy and 6 patients recovered following malignancy therapy. Patients with paraneoplastic vasculitis were older, more frequently had constitutional syndrome, and less frequently had organ damage due to the vasculitis than the remaining patients with cutaneous vasculitis. In summary, cutaneous paraneoplastic vasculitis is an entity not uncommonly encountered by clinicians. The most common underlying malignancy is generally hematologic. In these cases the presence of cytopenias and immature cells may be red flags for the diagnosis of cancer. In patients with paraneoplastic cutaneous vasculitis, the prognosis depends on the underlying neoplasia.

Show MeSH
Related in: MedlinePlus