Wegener’s granulomatosis is an organ- and/or life-threatening autoimmune disease of as yet unknown etiology. The classic clinical triad consists of necrotizing granulomatous inflammation of the upper and/or lower respiratory tract,  necrotizing glomerulonephritis,  and an autoimmune necrotizing systemic vasculitis affecting predominantly small vessels. 

The detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 (PR3-ANCA)  is highly specific for Wegener’s granulomatosis.  ANCA positivity is found only in about 50% of the patients with localized Wegener’s granulomatosis (which is restricted to the respiratory tract and affects

< or = 5% of the patients), whereas PR3-ANCA positivity is seen in 95% of the patients with generalized Wegener's granulomatosis


Vasculitis is an independent risk factor for diffuse endothelial dysfunction and may be a consequence of TNF-alpha action on endothelial cells (9,10)

Polyarteritis nodosa has been progressive illness resulting in a systemic necrotizing vasculitis which may affect the kidneys,  gastrointestinal tract,  skin,  nerves and muscles. Churg-Strauss is a hypereosinophilic syndrome inducing systemic vasculitis (10). The subjects affected may be tested for the presence of the FIP1L1-PDGRFA mutation (13)

In conclusion, hormonal contraceptives may induce allergic vascularities. It is hypotesized as possible etiology a reaction of cell-mediated immunity. Affected subjects may present cutaneous involvement alone or life-threatening systemic involvement, which may result in severe and sometimes fatal illness. 

Although,  oral contraceptives when implicated induce generally mild vasculitis,  a rare case of vasculitis with cutaneous necrosis HC-  containing levonorgestrel 0.15 μg and ethinylestradiol 0.03 mg related was reported (14)

Rosa Sabatini and Giuseppe Loverro
Dept. Obstetrics and Gynecology,
General Hospital Policlinico-University of Bari, Italy


[1]  Stone,  J.H.  (2007). Vasculitis:a   collection   of   pearls   and myths.Rheum.Dis.Clin.North Am,33(4), 691-739.
[2]  Youinou, P. (2008). Vasculitis: Current Status and Future Directions. Clin. Rev. Allergy Immunol. 
[3]  Mullick,  F.G.,  McAllister,  H.A.,  Wagner,  B.M.,  Fenoglio,  J.J.Jr.  (1979). Drug   related   vasculitis. Clinicopathologic   correlations   in   30   patients. Hum.Pathol, 10(3), 313-25.
[4]  Mat,C.,Yurdakul, S., Tuzuner, N., Tuzun, Y. (1997). Small vessel vasculitis and vasculitis confined to skin.Baillieres Clin. Rheumatol, 11(2), 237-57.
[5]  Thomas,  P.,  Dalle,  E.,  Revillon,  B.,  Delecour,  M.,  Devarenne,  M.F., Pagniez, I. (1985). Cutaneous effects in hormonal contraception. NPN Med, 5(81),19-24
[6]  Siroux,  V.,  Oryszczyn,  M.P.,Varraso,  R.,  Le Moual,  N.,  Bousquett,  J., Charpin,  D., Gormand,  F.,  Kennedy,  S.,  Maccario,  J.,  Pison,  C.,  Rage,  E., Scheinmann,  P.,Vervioet,  D.,  Pin,  I.,  Kauffman,  F.  (2007). Environmental factors for asthma severity and allergy results from the EGEA study. Rev.Mal.Respir, 24(5), 599-608
[7]  Presl,  J.  (1982). Steroidal contraceptives and the immune system.  Cesk Gynekol, 47(2), 599-608.
[8]  Lamprecht,  P.,  Gross,  W.L.  (2004).  Wegener’s granulomatosis.  Herz,  Feb, 29(1), 47-56.Review. 
[9]  Bonsib, S.M. (2001). Polyarteritis nodosa. Semin. Diagn. Pathol, 18(1), 14-23
[10]  Melimich,  B.,  Holl-Ulrich,  K.,  Merz,  M.,Gross,  W.L. (2007). Hypereosinophilic   syndrome   andChurg-Strauss   syndrome:  is   it clinically relevant to differentiate these syndromes? Internist.(Berl.).
[11]  Ben   Ghotbel,  I.,  Dhrif,  A.S.,  Miled,  M.,  Houman,  M.H.  Cutaneous manifestations   as   the   initial   presentation   of   Wegener’s granulomatosis. Presse Med,36(4 Pt 1), 619-22. 
[12]  Bounny,  C.,  Nievergelt,  H.,  Braathen,  L.R.,  Simon,  D.  Wegener’s granulomatosis   presenting   as   pyoderma   gangrenosum.  Clinic   and Polyclinic for Dermatology, Inselspital, University of Bern, Switzerland. 
[13]  Levy,  S.A.,  Franca,  A.T.,  De La Reza,  D.,Valle,  S.O.,Correla,  A.H. (2006). Asthma and Churg-Strauss syndrome.J.Bras.Pneumol,  32(4),  367-70.
[14]  Mosovich,  B.,  Biton,  A.,  Avinoach,  I.  (1991). Vaculitis with cutaneous necrosis induced by oral contraceptive .Harefuah, 120(8), 451-3.

Page 2 of 21 2

Provided by ArmMed Media