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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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Related in: MedlinePlus

Workup in a patient with cutaneous vasculitis to determine the presence of an underlying neoplasm. (Modified from reference 36, Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, Hunder GG. Cutaneous vasculitis and cancer: a clinical approach. Clin Exp Rheumatol. 2000;18:305–307.)
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Figure 3: Workup in a patient with cutaneous vasculitis to determine the presence of an underlying neoplasm. (Modified from reference 36, Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, Hunder GG. Cutaneous vasculitis and cancer: a clinical approach. Clin Exp Rheumatol. 2000;18:305–307.)

Mentions: Clinicians should be aware of the potential association between cutaneous vasculitis and neoplasm. Gonzalez-Gay et al36 proposed a workup to exclude a neoplasm in a patient with cutaneous vasculitis (Figure 3). Such a procedure should include the following:


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)

Workup in a patient with cutaneous vasculitis to determine the presence of an underlying neoplasm. (Modified from reference 36, Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, Hunder GG. Cutaneous vasculitis and cancer: a clinical approach. Clin Exp Rheumatol. 2000;18:305–307.)
© Copyright Policy
Related In: Results  -  Collection

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

Figure 3: Workup in a patient with cutaneous vasculitis to determine the presence of an underlying neoplasm. (Modified from reference 36, Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, Hunder GG. Cutaneous vasculitis and cancer: a clinical approach. Clin Exp Rheumatol. 2000;18:305–307.)
Mentions: Clinicians should be aware of the potential association between cutaneous vasculitis and neoplasm. Gonzalez-Gay et al36 proposed a workup to exclude a neoplasm in a patient with cutaneous vasculitis (Figure 3). Such a procedure should include the following:

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