Marcia Ramos-e-Silva, MD, PhD
Associate Professor, Sector of Dermatology and Post-Graduation Course in Dermatology
HUCFF-UFRJ, School of Medicine, Universidade Federal do Rio de Janeiro
Rio de Janeiro, Brazil.

Sueli CS Carneiro, MD, PhD
Dermatologist, Sector of Dermatology and Post-Graduation Course in Dermatology
HUCFF-UFRJ, School of Medicine, Universidade Federal do Rio de Janeiro
Associate Professor, Sector of Dermatology, Universidade do Estado do Rio de Janeiro
Rio de Janeiro, Brazil.

From the Sector of Dermatology and Post-Graduation Course in Dermatology,
HUCFF-UFRJ, School of Medicine, Universidade Federal do Rio de Janeiro
Rio de Janeiro, Brazil.

Cutaneous vasculitis constitute a heterogeneous group of infrequent diseases and the lack of sufficient knowledge about its pathogenesis leads to a great controversy related to its classification. Depending on the author, these disorders have been classified by various features, among them the clinical and histopathological characteristics, and the caliber of the affected vessels.1,2 This is the reason why the incidence of the primary and secondary clinical forms is not perfectly known. There is also enormous confusion about their etiology. Various reasonable classification schemes for vasculitis have been proposed but none was adopted worldwide. The lack of an universally accepted nomenclature results in an important communication problem on this subject.3

Introduction
Vasculitis may be considered an inflammatory syndrome with structural alterations of the vascular wall, complicated by lumen occlusion, leading to tissular iskemia.4 In the majority of classifications it is considered a spectrum of lesions that can be primary or secondary but diseases included as primary vasculitis in one author's classification can be even not mentioned in a scheme proposed by another. Vessels of any caliber may be altered in systemic vasculitis but in cutaneous forms alteration affects small vessels especially the post-capillary venules.2 The various forms differ one from another by age of onset, affected organ, and presence of periods of remission and exacerbation, among other features. Sometimes they may also be superimposed to other well defined diseases, as vascular disorders, neoplasia, allergic reaction, and infections.1,5,6,7

All infective agents can promote inflammation of arterial or venular wall of any diameter and of any organ.4 Palpable purpura is the most common manifestation of vasculitis, although erythematous macules, nodules, ecchimoses, erosions, ulcerations, hemorrhagic blisters, necrosis, and gangrene may also occur. Skin can be the target organ in this type of vascular alteration, but it may as well be the site of lesions resulting from systemic vasculitis. With relative frequency vasculitis of the skin may be the first manifestation of a very severe systemic disease.2,8

In Latin America, where there is a great deficiency of statistical data, it is very difficult to know the exact prevalence and incidence of these vasculitic diseases. Cutaneous vasculitis is usually diagnosed in tertiary hospitals where statistics about the subject are better known and which represent the reference source in the different regions. In one study from a Rheumatology Department in Sao Paulo, of 94 patients with vasculitis, 36% presented Takayasu arteritis, 16% thromboangiitis obliterans, 7% vasculitis associated to collagen disease, 6% polyarteritis nodosa, 18% showed undifferentiated vasculitis, while the others various less frequent types.9 Leprosy, in its form of erythema nodosum and of Lucio phenomenon, is probably the most common cause of vasculitis in the World, specially in underdeveloped and developing countries, due to its great incidence. There is a greater number of publications about rare forms and very few papers on the more common forms of vasculitis. Among the several types of vasculitis observed in Latin America, Wegener granulomatosis, Churg-Strauss disease, polyarteritis nodosa, Takayasu disease, erythema elevatum diutinum, systemic lupus erythematosus, rheumatoid arthritis and Sjögren syndrome vasculitis, those observed mostly in infancy, as Kawasaki disease, and those seen in association to some infections, as leprosy, tuberculosis and meningitis, will be discussed in this article, exploring some of the particularities of the cases observed in this region.

Wegener granulomatosis

Wegener granulomatosis is characterized by granulomatous vasculitis of the upper and lower respiratory tracts associated with glomerulonephritis. It is an uncommon disease although its incidence is difficult to determine. It can occur in any age although infrequent in preadolescents. When compared to whites, it is rare in blacks.10

Extensive necrotizing genital lesions of Wegener granulomatosis were observed in patients from Argentina who improved with corticoesteroids and immunosupressors,11 although prognosis is considered poor.12,13,14, In Cuba, unusual hyperkeratotic cutaneous lesions were observed in patients with Wegener granulomatosis. 15

A publication from Brazil gathering ten cases referred pulmonary, renal and nasal as the most frequent involvement. Cutaneous manifestations (oral, genital and cutaneous ulcerations, granulomatous vasculitis of small vessels and capilaritis) and of the central nervous system were observed in three cases, who were treated with pulsetherapy with corticosteroids and cyclophosphamide.16

Santiago et al., in Brazil, studying the frequency of ANCA with Elisa and indirect immunofluorescence (IFI) in 103 patients with primary vasculitis or secondary to diffuse collagen diseases, observed positivity by IFI in 7 of 17 GW patients, and 4 of 24 with PAN. With ELISA technique there was positivity in 4 of 24 cases of PAN, 4 of 17 GW cases, 1 of 16 cases of rheumatic polymialgia, 1 case of microscopic arteritis and one of non determined vasculitis. This data confirmed the value of ANCA detection in primary systemic vasculitis, in particular Wegener granulomatosis in this Brazilian sample.17

Also in Brazil, in the city of Rio de Janeiro, Fernandes et al., in two different case reports (Figure 1,2), referred localized cutaneous lesions consequent to necrotizing vasculitis and granulomatous capilaritis.18,19

Churg-Strauss disease

Churg-Strauss disease or allergic angiitis and granulomatosis is characterized by granulomatous vasculitis of multiple organ systems, particularly the lung. There is vasculitis of various-sized vessels, intra- and extravascular granuloma formation together with eosinophilic tissue infiltration, strong association with severe asthma and peripheral eosinophilia. Its exact incidence is difficult to determine although it is known that it can occur at any age with a possible exception of infants. Prednisone and cyclophosphamide are usually prescribed with a high rate of complete remission.20

The first Brazilian case of Churg-Strauss vasculitis (Figure 3, 4) was studied in Rio de Janeiro, in 1975, and this male patient exhibited a severe necrotizing lesion of the center of the face, high counts of eosinophils in the blood, in the facial lesion, as well as in the severe visceral lesions that led to death.21 At the Hospital of the Federal University of Rio de Janeiro, in 1993, three cases of Churg-Strauss angiitis in male patients were diagnosed. All had previous history of bronchic asthma, renal involvement and leucocitoclastic vasculitis with palpable purpura and pustules. Therapy with corticosteroid and clorambucil showed a satisfactory response in 2 cases. One evolved to death.22

Polyarteritis nodosa

Polyarteritis nodosa is a rare systemic illness with focal symptoms and signs due to local circulatory disturbances. It presents generalized necrotizing panarteritis due to alterations of small and medium sized muscular arteries. The lesions are usually cutaneous and/or subcutaneous palpable nodules, and there may be thrombosis, embolism, and rupture of the vessel wall. For the treatment a variety of drugs have been tried and corticosteroids associated or not to azathioprine or cyclophosphamide still seems to be the best choice.23

Research on the behavior of polyarteritis nodosa, performed in the Southern Central region of Brazil, at Campinas University, has shown that from 20 patients (12 men and 8 women), 16 had generalized while 4 benign cutaneous form. In the generalized form, diagnosis is made by arterial hypertension, muscle-skeletal and general symptoms. Cutaneous lesions occurred because of the vasculitis and presented as purpuric, nodular and ulcerated lesions, besides Raynaud phenomenon and digital necrosis. Age varied from 19 to 70-years and the mean time of sickness was 1,87 years. Five patients (four men) died because of renal and cardiac failure, and septicemia. HbsAg exam was positive in 2 of 8 cases.24

In South American literature, many publications about cutaneous polyarteritis nodosa show that lesions are subcutaneous nodules, livedo reticularis, ulceration and necrosis. 25,26,27,28,29,30

Takayasu arteritis

Takayasu arteritis or aortic arch syndrome is an uncommon inflammatory and stenotic disease of medium and large-sized arteries characterized by a strong predilection for the aortic arch and its branches. This disease is a panarteritis with inflammatory mononuclear cell infiltrates and occasionally giant cells. It occurs in all the World, although more prevalent in the Oriental races. Affects mainly adolescent girls and young women. There are general symptoms, pain over the affected vessels, ischemia in organs supplied by those vessels, and absence of pulse. Cardiomegaly and cardiac failure may occur. The course may stabilize, progress gradually or be fulminant; in fact it is extremely variable and spontaneous remission may occur. Some patients may benefit of the use of cyclosphosphamide, when resistant to steroids.20

Clinical and angiographic studies of Takayasu arteritis done in the Southern Central region of Brazil, in the period between 1993 and 1997, in 34 patients (28 women, 20 white with mean age of 27,7 years and time of disease between 2 and 360 months; median: 41 months) showed that the clinical complaints at the time of diagnosis were: claudication on superior limbs in 32%, claudication on inferior limbs in 23%, weight loss in 15%, fever in 15%, congestive cardiac insufficiency in 9%, headache in 6%, arterial hypotension in 6% and pre-cordial pain in 6%. During the follow-up, 60,7% presented absence or decrease in the superior limbs' pulse, 56% showed a difference of arterial pressure higher than 10 mmHg in arms, 53% had arterial murmur and 29% cardiac murmur. Of 28 patients that were submitted to pan-angiography, 21,4% were classified as type I, 3.6% as type IIa, 3.6% as type III, 14,3% as type IV and 57,1% as type V. None of the patients were classified as type IIb. There was more than one case of Takayasu arteritis in three families. Treatment was performed with prednisone in 33 patients; 11 needed association with methotrexate in variable dosage of 7,5 to 15 mg/week. Nine patients were submitted to some kind of surgical procedure. Evolution showed that the majority of patients (59%) had their disease controlled.31

Titton et al.32 presented a case of Takayasu arteritis in a 26-year-old woman with hyperchromic and desquamative cutaneous lesions showing a pelagroid aspect. Histopathology of this case showed parakeratosis, epidermal keratosis and lymphocytic perivascular infiltrate.

Diffuse morphea with cutaneous involvement of face, neck, superior limbs, trunk and abdomen, and histopathologic alterations of scleroderma in a patient with Takayasu arteritis was reported by Mahayri et al..33 From ten children (4 boys and 6 girls with age variation from 2 months to 12 years) with renovascular hypertension, evaluated in Chile, between 1976 and 1986, 4 had a diagnosis of Takayasu arteritis.34

Erythema elevatum diutinum

Erythema elevatum diutinum (Figure 5) is a rare and chronic disorder of unknown etiology characterized by persistent red, purple, and yellowish papules, plaques, and nodules. Distribution is usually symmetric over the extensor surface of the limbs. It affects mostly patients in the sixth decade and in general responds dramatically to dapsone. This disease is a leucocitoclastic vasculitis associated to a marked dermal inflammatory infiltrate and can be associated to many diseases as diabetes, leukemia, myeloproliferative syndromes and others.20

The 10-year data of the Hospital of the Federal University of Rio de Janeiro, showed only one patient with diagnosis of erythema elevatum diutinum.35

Systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a disease of unknown etiology in which tissues and cells are damaged by deposition of pathogenic autoantibodies and immune complexes. Ninety percent of cases occur in women. Any age can be affected, although most cases are in child-bearing age. Vasculitic skin lesions (Figure 6, 7) appear in 20 % of the patients with SLE and include subcutaneous nodules, ulcers (usually on the legs), purpura, and infarcts of skin or digits. Besides vasculitis there is increased evidence that antibodies against phospholipids (anticardiolipin) play an important role in the clotting process; and thrombosis in capillaries, small vessels, and medium-sized veins and arteries can be a major problem. There is no cure for SLE, but its control may be achieved with corticosteroids, salycilates, antimalarials, and/or cytotoxic agents, as cyclophosphamide, azathioprine, and clorambucil, still controversial in this disease, depending on the clinical and laboratorial features of each case.36

Costallat & Coimbra,37 in the Southern central region of Brazil, found cutaneous vasculitis in 7,13%, livedo reticularis in 5 patients. Another study38 performed at the southeast of Brazil showed 20 systemic lupus male patients and, among these, three with cutaneous vasculitis represented by ulceration in inferior limbs and/or palpable purpura.

Rheumatoid arthritis and Sjögren disease

Rheumatoid arthritis is a chronic, multisystem disease of unknown etiology that affects the peripheral joints in a symmetric distribution,39 while Sjögren syndrome is an immunologic disorder characterized by progressive destruction of the exocrine gland leading to mucosal and conjuntival dryness (sicca syndrome) accompanied by a variety of autoimmune phenomena.40 Leucocytoclasic vasculitis has also been described in Sjögren syndrome41 and in rheumatoid arthritis in Brazil.42

Kawasaki disease and other vasculitis in infancy

In infancy the most important vasculitis is Kawasaki disease or mucocutaneous lymph node syndrome. It is an acute, febrile, multisystem, usually benign, and self-limited disorder of children characterized by nonsuppurative cervical adenitis; unresponsiveness to antibiotics; and changes in the skin and mucous membranes such as edema, congested conjunctivae, erythema of the oral cavity, lips, and palms, and desquamation of the skin of the fingertips. Total recovery usually occurs although up to 2.8% of patients develop fatal complications, as vasculitis of the coronary arteries. Early administration of high-dose intravenous gamma globulin associated to aspirin may be effective in reducing the prevalence of coronary artery alterations.20

In Argentina, Kawasaki disease represents one of the most frequent forms of vasculitis in children, right after Henoch-Schoënlein purpura and erythema nodosum. In a series studied in four pediatric hospitals in the cities of Buenos Aires and La Plata between the years of 1976 e 1994, 238 cases of Kawasaki disease were diagnosed. Sixty-three percent of the cases occurred in boys with mean age of two and a half years (minimum of one month and maximum of l5 years). Three patients belonged to families of Japanese origin (1%), which is a percentage much greater than the total of the Japanese population in Argentina, thus being statistically significant. Cardiac involvement was observed in 39% of the patients. Aneurysms and/or coronary arteries dilation were observed in 24% of the cases, and 8 patients had peripheral arteries aneurysms. Other vasculitis observed were Takayasu disease and among the ones secondary to other entities Churg-Strauss disease, vasculitis due to cryoglobulinemia-associated infection, seropositive juvenile rheumatoid arthritis, systemic lupus erythematosum, dermatomyositis and other collagen diseases.43

Among pediatric patients seen in Latin America there are some other series published about vasculitis as manifestation of other diseases. An evaluation of a group of 17 patients with SLE in infancy (American College of Rheumatology - ACR criteria), observed at the south of Brazil, cutaneous vasculitis was found in 53%.44 In another evaluation of 2140 consultations in an pediatric out-patient clinic at the Southern Central region of Brazil, of which 349 were new cases, vasculitis together with other collagen diseases was responsible for 12% (24 cases).45 Still from the Southern Central region of Brazil, 11 cases of cutaneous polyarteritis nodosum in infancy presented subcutaneous nodules in 6 (54%), ischemia and necrosis in six (54%), livedo reticularis in five (45%) and a possible etiologic association of this entity with group A b-hemolytic streptococcus. Seven of ten children (70%) presented at time of diagnosis, high titers of antistreptolysine O (ASLO), suggesting a recent streptococcal infection.46

Infectious diseases

Various viral, bacterial, fungal, protozoan, and helminthic agents may affect blood vessels during acute infection, its regression period, or even in its chronic phase. The complete pathogenesis is unknown but it may be due either to their direct, toxic, or immunologic action, or the conjunction of two or all of these mechanisms.

Meningococcaemia which usually affects infants may promote vascular lesions, as palpable purpura, petechiae, at times with vesicular or pustular centre, appearing asymmetrically on trunk or extremities, forming ecchymotic and necrotic areas.47 At the Hospital of the Federal University of Rio de Janeiro, Brazil, during the last few years severe cases of vasculitis associated to meningococcaemia were observed. Young patients presented vasculitic skin lesions including gangrene of one or more areas of the superior and/or inferior extremities, during the remission of the infection. (Figure 8, 9)

In Latin America many other infectious agents may lead to vasculitis, even promoting a necrotizing form. Of all possibilities in this region leprosy is the far most common. Mycobacterium leprae may lead to two main forms of specific vasculitis: erythema nodosum leproticum, and Lucio's phenomenon.

Erythema nodosum leproticum (ENL) or type 2 leprosy reaction (Figure 10), occurs in lepromatous and borderline lepromatous patients, most frequent in the latter half of the initial year of treatment. Tender, inflamed subcutaneous nodules develop generally in crops. Each nodule lasts a week or two, but new lesions can appear. Histology shows a pattern similar to Arthus phenomenon.48 Corticosteroid may be introduced but the best treatment is thalidomide, which cannot be prescribed to women in fertile age.49

Lucio's phenomenon (Figure 11, 12) is a necrotizing cutaneous reaction described mainly in association to diffuse, infiltrative, non-nodular form of leprosy mentioned by Lucio and Alvarado in 1842. This vasculitis presents necrosis of arterioles, after a severe invasion of the endothelium by Mycobacterium leprae.50 There may be hemorrhagic infarct of extensive cutaneous areas caused by the occlusion of even deep dermis and subcutaneous venous plexus.51 Lucio's phenomenon may be treated with the usual polychemotherapy but other drugs as pentoxyphiline may be added.50

Tuberculosis has been considered responsible for some nodular forms of cutaneous vasculitis, especially the form known as erythema induratum of Bazin (Figure 13), which is considered the most frequent manifestation: 66% in the series studied and 20% of all cases of skin tuberculosis.52 It seems related to a hipereactivity to antigens of Mycobacterium tuberculosis. Patients show strongly positive Mantoux test and the vascular changes may be associated to a chronic granulomatous infiltrate. With the recent increase in incidence of tuberculosis, greater attention is being given to this entity especially with the use of new techniques, as PCR, in the attempt to elucidate and define the relation of tuberculosis with this type of nodular vasculitis.

Acknowledgements: Dr. Mauro Goldfarb (Rio de Janeiro, Brazil) helped in obtaining the literature used in this article and Prof. Nurimar Fernandes (Rio de Janeiro, Brazil) and Prof. Tania Cestari (Porto Alegre, Brazil) reviewed the final version of the paper. Figures 5 to 13 belong to the slide collection of the Sector of Dermatology of the Hospital of the Universidade Federal do Rio de Janeiro (HUCFF-UFRJ).

References
1. Hérnandez-Rodriguez PJ. Las vasculitis necrosantes: inmunopatogenia, clasification y cuadro clinico. Acta Med Hosp Clin Quir Hermanos Ameijeiras 1988;2(1):194-207.
2. Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol 1998;39:667-687.
3. Jennette CJ, Milling DB, Falk RJ. Editorial: Vasculitis affecting the skin. A review. Arch Dermatol 1994;130:899.
4. Lie JT. Systemic pulmonary and cerebral vasculitis. In: Stehbens WE, Lie TJ. eds. Vascular Pathology. London:Champman & Hall, 1995:623-656.
5. Chakravarty K, Scott AGI, Blyth J, Courteney-Harris RG. Wegener's granulomatosis in the elderly: unusual presentations and misdiagnosis. J Rheumatol 1994;21:1157-1159.
6. Mandell BF, Hoffman GS, Differentiating the vasculitides. Rheum Dis Clin North Am 1994;20:409-442.
7. Somer T, Finegold SM. Vasculitides associated with infection, immunization and antimicrobial drugs. Clin Infect Dis 1995; 20:1010-1036.
8. Lotti T. Comacchi C, Ghersetich I. Cutaneous necrotizing vasculitis. Int J Dermatol 1996;35:457-474.
9. Sato EI, Hatta FS, Castro CHM, Nunes DS, Santo BE. Avaliação multidisciplinar das vasculites sistêmicas num centro de atendimento terciário: experiência da Unifesp. Rev Bras Reumatol 1998;38(3):133-138.
10. Fauci AS. The vasculitis syndromes. In: Wilson JD, Braunwald E, Isselbacher KJ et al. eds. Harrison's Principles of Internal Medicine. 12 ed. New York: McGraw-Hill; 1991:1456-1463.
11. Kaplan H, Sevinsky B, Lacentre E. Granulomatosis de Wegener. Rev Argent Dermatol, 1986;67:118-25.
12. Correa JC, Azevedo AGP, Rubens J, Rocha G. Granulomatose de Wegener: análise de dois casos. J Bras Med 1985;48:34-38.
13. Rojas SL, Schweitzer BM. Granulomatosis de Wegener tratada com cotrimoxazol. Enfermedades respir cir torac 1988;4:111-115.
14. Pedrini H, Fernandes SRM, Billis A. Glomerulonefrite granulomatosa com necrose papilar renal em granulomatose de Wegener. J Bras Nefrol 1988;10:75-78.
15. Nápoles-Diaz E, Roiz-Balaguer M, Rodriguez-Rivera L. Manifestaciones cutáneas no frecuentes en la granulomatosis de Wegener. Rev Cuba Med 1988;27:92-97.
16. Fernandes SRM, Samara AM. Granulomatose de Wegener: análise clínica e histopatológica. Rev Bras Reumatol 1991;31:218-222).
17. Santiago MB, Bueno C, Gaburo Júnior N, Mattos N, Oliveira RM. Anticorpos contra citoplasma de neutrófilos (ANCA) em portadores de vasculites. Rev Bras Reumatol, 1990;30:117-120.
18. Fernandes NC, Cardoso ICL, Serpa SS, Rettore FC, Maceira J. Granulomatose de Wegener localizada: relato de caso. An bras Dermatol, 1996;71:41-44.
19. Fernandes NC, Paes C, Castilho M Maceira J. Granulomatose de Wegener localizada com manifestação ocular: relato de caso. An bras Dermatol, 1998;73:107-110.
20. Fauci AS. The vasculitis syndromes. In: Wilson JD, Braunwald E, Isselbacher KJ et al. eds. Harrison's Principles of Internal Medicine. 12 ed. New York: McGraw-Hill, 1991:1456-1463.
21. Ramos-e-Silva J. Vasculite alérgica de Churg-Strauss. Bol Acad Nac Med 1975;146(6):13-33.
22. Sauaya D, Tuma F, Azevedo MNL et al. Angiite de Churg-Strauss. Relato de três casos. Rev Bras Reumatol 1993;33:248-250.
23. Ryan TJ. Cutaneous vasculitis. In: Champion RH, Burton JL, Burns DA, Breathnach SM. eds. Rook/Wilkinson/Ebling textbook of dermatology. 6 ed. Oxford:Blackwell Science, 1998:2155-2227.
24. Fernandes SRM, Costalat LTL, Amstalden EMI, et al. Poliarterite nodosa: contribuição ao estudo clínico, histopatológico e laboratorial. Rev Bras Reumatol 1997;37:194-200.
25. Rubinstein J, Swami JG, Leite N et al. Poliarterite nodosa cutânea: relato de três casos; sendo dois com poliartrite. Rev Bras Reumatol 1985;25:37-40.
26. Pernambuco JC, Fleury RN, Arone SE, Mello CR. Poliarterite nodosa cutânea. Rev Bras Reumatol 1984;24:219-222.
27. Gianelli MA, Proença NG, Muller H, Lauand N. Poliarterite nodosa cutânea. An bras Dermatol 1985;60:145-147.
28. Hassan ML, Baldrich A, Mugico B, Del Castilho MRP, Stengel F. Periartritis nodosa predominantemente cutânea: consideraciones a proposito del estudio de dos casos: revision del tema. Rev Argent Dermatol 1990;71:48-57.
29. Iglesias A, Ariza A, Donado M, Chinchilla A, Egea E, Blanco A. Panarteritis nodosa variante ulcero-necrotical. Acta Méd Colomb 1986;11:291-296.
30. Gajardo J, Escalona A. Poliarteritis nodosa cutánea. Dermatologia (Santiago de Chile) 1993;9:197-199.
31. Sato EI, Sassaki Jr RH, Leão CS, Hatta FS, Nunes DS, Santo BE. Arterite de Takayasu: estudo clínico e angiográfico. Rev Bras Reumatol 1998;38:9-14.
32. Titton JA, Azeka TN, Koyama RE, Fillus Neto J. Arterite de Takayasu: relato de um caso com manifestação cutânea incomum. Rev Bras Reumatol 1993;33:199-203.
33. Mahayri N, Fernandes SRM, Marques Neto JF, Amara AM. Rev Bras Reumatol 1987;27:31-34.
34. Saiech C, Diaz V, Valdés F, Ramirez K, Once JD. Hipertensão renovascular em pediatria. Rev Chil Pediatr 1987;58:382-6.
35. Binenboim C, Pereira FBC, Azevedo PAVS et al. Eritema elevatum diutinum: estudo retrospectivo dos últimos 10 anos do HUCFF-UFRJ. F Méd (BR) 1997;114:109-112.
36. Hahn BH Systemic lupus erythematosus. In: Wilson JD, Braunwald E, Isselbacher KJ et al. eds. Harrison's Principles of Internal Medicine. 12 ed. New York:McGraw-Hill, 1991:1432-1437.
37. Costallat LTL, Coimbra AMV. Lesões cutâneas no lupus eritematoso sistêmico. Estudo clínico de 272 casos. Rev Bras Reumatol 1994;34:30-37.
38. Guimarães SJ, Klumb EM, Pinheiro SV, Cavalcante EF, Lederman R, Leite N. Lupus eritematoso sistêmico em pacientes do sexo masculino. Estudo de 20 casos. Rev Bras Reumatol 1993;34:133-137.
39. Lipsky PE. Rheumatoid arthritis. In: Wilson JD, Braunwald E, Isselbacher KJ et al. eds. Harrison's Principles of Internal Medicine. 12 ed. New York: McGraw-Hill; 1991:1437-1443.
40. Lane HC, Fauci AS. Sjögren's syndrome. In: Wilson JD, Braunwald E, Isselbacher KJ et al. eds. Harrison's Principles of Internal Medicine. 12 ed. New York: McGraw-Hill; 1991:1449-1450.
41. Fernandes SEM, Yamada RM, Bértolo MB, Amstalden EMI, Bonfiglioli R, Samara AM. Envolvimento pulmonar e vasculite na síndrome de Sjögren. Rev Bras Reumatol, 1997;37:17-22.
42. Carneiro SCSC, Trope BM, Roimicher L, Filgueira AL, Marques AS. Vasculite leucocitoclásica em paciente com artrite reumatóide clássica. An bras Dermatol 1991;66:145-8.
43. Cuttica R J. Vasculitis en la infancia. Rev Bras Reumatol, 1995;35(Supl):S7- 8.
44. Toffoli SM, Von Mulhen CA, Brenol JCT, Tourinho T. Lupus eritematoso sistêmico (LES) na infância. Rev Bras Reumatol, 1995;35(Supl): S18.
45. Machado CM, Carvalho Jr J, Martin JC, Borri FC. Análise do atendimento ambulatorial em reumatologia pediátrica. Rev Bras Reumatol, 1995;35(Supl):S19-20.
46. Kiss MHB, Silva CHM, Silva CAA, Janz LL. Poliarterite nodosa cutânea na infância: relato de 11 casos e possível associação com o estreptococo b-hemolítico do grupo A. Rev Bras Reumatol 1998;38:373-377.
47. Canizares O. Head, neck trunk, axillae and groins. In: Canizares O, Harman RRM. eds. Clinical Tropical Dermatology. 2 ed. Oxford:Blackwell Scientific Publications, 1992:763-790.
48. Miller RZ. Leprosy (Hansen's disease). In: Wilson JD, Braunwald E, Isselbacher KJ et al. eds. Harrison's Principles of Internal Medicine. 12 ed. New York: McGraw-Hill; 1991:645-678.
49. Souza MCF, Oliveira SHW, Durães SM, Garcia CC, Nery JAC. Morbidade do eritema nodoso em mulheres em idade fértil - observação clínica. Hansen Int 1997;22(1):96.
50. Souza CS, Figueiredo F, Foss NT. Fenômeno de Lúcio e pentoxifilina. Hansen Int 1997;22(1):125.
51. Fleury RN, Ura S, Opromolla DVA. Fenômeno de Lucio (eritema necrosante). Hansen Int 1995;20(2): 60-65.
52. Adriaans B, Dominguez-Soto L, Canizares O, Harman R, Lavalle P, Novales J. Tropical Bacterial Dermatoses II. In: Canizares O, Harman RRM. eds. Clinical Tropical Dermatology. 2ed. Oxford: Blackwell Scientific Publications, 1992:201-222.

Figure 1 - Wegener granulomatosis (Courtesy of Nurimar Fernandes) 18
Figure 2 - Wegener granulomatosis (Courtesy of Nurimar Fernandes) 19
Figure 3 - Churg-Strauss vasculitis (From the collection of João Ramos-e-Silva) 21
Figure 4 - Churg-Strauss vasculitis (From the collection of João Ramos-e-Silva) 21
Figure 5 - Erythema elevatum diutinum 35
Figure 6 - Vasculitis in systemic lupus erythematosus
Figure 7 - Vasculitis in systemic lupus erythematosus
Figure 8 - Vasculitis after meningitis
Figure 9 - Vasculitis after meningitis
Figure 10 - Erythema nodosum leproticum
Figure 11 - Lucio's phenomenon
Figure 12 - Lucio's phenomenon
Figure 13 - Erythema induratum of Bazin