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In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels. Its main causes are autoimmune disorders and (occasionally) infections. Treatment depends on the cause. While most vasculitides are rare diseases, they generally affect several organ systems and can cause severe disability. more...

VACTERL association
Van der Woude syndrome
Van Goethem syndrome
Varicella Zoster
Variegate porphyria
Vasovagal syncope
VATER association
Velocardiofacial syndrome
Ventricular septal defect
Viral hemorrhagic fever
Vitamin B12 Deficiency
VLCAD deficiency
Von Gierke disease
Von Hippel-Lindau disease
Von Recklinghausen disease
Von Willebrand disease


The types of vasculitis are distinguished by the type of blood vessel affected (aorta, large arteries, arterioles, capillaries and veins), the appearance of biopsy tissue of affected organs on light microscopy, and if necessary, with the help of immunohistochemistry (use of monoclonal antibodies against specific inflammatory protein markers).

Other diagnostic tools are the detection of circulating antibodies that are associated with forms of vasculitis. While these measurements have a low positive and negative predictive value (due to the high rates of both false positives and false negatives), they can direct the clinician to specific causes for vasculitis.


Infectious vasculitis is generally treated with directed antibiotics, while autoimmune forms often require treatment with immune suppression: steroids, DMARDs ("steroid-sparing agents") or cyclophosphamide (a mild form of chemotherapy). For very severe forms, bone marrow transplantation is presently being investigated as the ultimate silencing of the immune system.

Causes and types

  • Large vessel vasculitis
    • Giant cell arteritis (also temporal arteritis)
    • Takayasu's arteritis
  • Medium-sized vessel vasculitis
    • Polyarteritis nodosa
    • Kawasaki's disease
    • Cerebral vasculitis (primary granulomatous)
  • Small-vessel vasculitis
    • Associated with ANCAs (anti-neutrophil cytoplasmatic antibody):
      • Microscopic polyangiitis
      • Wegener's granulomatosis
      • Churg-Strauss syndrome
      • Drug-induced
    • Associated with deposition of immune complexes:
      • Henoch-Schönlein purpura (HSP)
      • Cryoglobinemic vasculitis
      • Lupus erythematosus vasculitis
      • Rheumatoid vasculitis
      • Sjögren's syndrome vasculitis
      • Urticarial vasculitis associated with decreased complement
      • Behçet's disease
      • Goodpasture's syndrome
      • Serum sickness-vasculitis
      • Drug-induced
      • Infection-induced (not infectious)
    • Paraneoplastic
      • Lympho- and myeloproliferative neoplasm associated
      • Carcinoma-associated
    • Inflammatory bowel disease vasculitis


  • Jenette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337(21):1512-23. PMID 9366584.


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Acute pustular psoriases complicated by leukocytoclastic vasculitis - Case Reports
From Journal of Drugs in Dermatology, 4/1/03 by Michael Jude Welsch

Acute pustular psoriasis is characterized by fiery-red erythema followed by formation of pustules. Precipitating factors include drugs, infections, pregnancy, solar irradiation, and psychological stress. We present a case of a woman who developed acute onset of pustular psoriasis precipitated by hydroxychloroquine therapy and systemic steroids. The patient's course was complicated by leukocytoclastic vasculitis presumptively caused by levofloxacin.


Pustular psoriasis is an acute form of psoriasis characterized by numerous pinpoint pustules appearing in clusters that may coalesce to form lakes of pus. Fever, generalized weakness, malaise, and leukocytosis are prominent features. Numerous triggering factors have been implicated. Drugs (Table I) are most commonly reported as triggering agents although infections (17,18), solar irradiation (15,18,19) emotional stress (19,20), pregnancy (21), and hypocalcemia (22) have also been cited.

Case Report:

A 65 year old female with a past medical history significant for hypertension, chronic renal insufficiency, hyperlipidemia, gout, arthritis, diverticulitis, and recently diagnosed Sjogren's syndrome was referred to the Dermatology Clinic with the complaint of an generalized pustular eruption that began on the upper torso four days prior. The patient was taking losartan, nifedipine, lasix, clonidine, simvastatin, and sulfinpyrazone. She had started hydroxychloroquinine two weeks prior for symptoms suggestive of Sjogren's syndrome. The eruption began on the chest as an erythematous plaque and had been treated with oral corticosteroids.

Physical exam revealed erythematous, arcuate plaques studded with pustules (Figures 1 and 2). Histologically, subcorneal and intercorneal pustules were noted. Bacterial and fungal cultures were negative. The prednisone was tapered, hydroxychloroquine discontinued, and acetretin begun. The patient had an initial white blood cell count (WBC) of 14,000 without signs or symptoms of infection.


In five days, the patient's complete blood count was significant for an increased WBC to 24,200. She was started empirically on levofloxacin 500 mg a day. Three days later, she complained of a new rash on her lower body. The lower extremities had confluent, purpuric plaques with few grayish centers. No pustules remained on the patient's trunk.

Levofloxacin was discontinued. Biopsy of the lesion revealed leukocytoclastic vasculitis. Blood cultures, urine culture and chest roentgenogram were negative. The patient remained afebrile, was treated with topical corticosteroids and anti-pruritics, and required oral narcotics for pain relief. One week after admission, some fading of the initial purpuric lesions was evident. Secondary infection was suspected based on the slow resolving diffuse erythema with yellow crusts. Bacterial cultures of the lesions grew methicillin-resistant staphylococcus aureus. Biopsies demonstrated diffuse impetiginous changes of the epidermis. Aggressive debridement with chlorhexidine brush and soap was performed on a daily basis. Acticoat dressings were applied to pseudoeschar areas. On hospital day 14, she received vancomycin to hasten clearing of the lower extremity lesions. She remained afebrile without leukocytosis or signs of systemic infection. She was discharged after 3 weeks of hospitalization. At follow-up she was markedly improved. Some residual edema and erythema of the lower extremities remained but no recurrence of psoriatic lesions occurred.


The average age of onset for pustular psoriasis is 50 years. Males and females are affected about equally (18). Pustular psoriasis may appear as localized pustular psoriasis, which runs a chronic course, or generalized pustular psoriasis (23). The localized form is a chronic pustular dermatosis of the palms and soles and has two main divisions. Acrodermatitis continua usually begins unilaterally as a pustule on the distal extremity often involving the nailbed (Figure 3). Nails may float off in lakes of pus. Massive digital hyperkeratosis may be noted. Eventually, extension to the remaining digits and more generalized flares may occur (Figures 4 and 5). Pustules occur on fiery red skin that is often painful and tender (Figure 6). Chronic palmoplantar pustulosis is a more indolent form of disease. It is often a bilaterally symmetric pustular dermatosis with deep-seated pustules generally restricted to the palms and soles (Figure 7). The generalized pattern (Figures 8-10) is also referred to as the von Zumbusch pattern, named after Ludwig von Zumbusch, who described a pustular eruption in a patient with psoriasis vulgaris in 1910 (24).


Hypocalcemia secondary to concomitant hypoalbuminemia occurs often in patients with yon Zumbusch psoriasis (18,22). Hypocalcemia has also been reported to cause flares of pustular psoriasis that repeatedly cleared in response to restoration of norMal serum calcium levels (22). The pathogenesis of pustular psoriasis is not completely understood.

The differential diagnosis of a patient with a pustular eruption includes pustular psoriasis, acute generalized exanthematous pustulosis (AGEP), and infection. AGEP is usually triggered by a drug in a patient without a prior history of psoriasis. Its course is characterized by rapid spontaneous healing (25). Infectious causes are ruled out by negative gram stain and cultures. In our case, the arcuate nature of the plaques, history of arthritis consistent with psoriatic arthritis, exacerbation by oral corticosteroids, associated toxicity, and slow response to treatment argues in favor of pustular psoriasis.

Therapy is aimed initially at identification and removal of any offending agents. Retinoids such as acitretin at doses of 0.5-1.0 mg/kg/day leads to rapid clearing of pustulation (18,26). Methotrexate (15,18), hydroxyurea (15,18,27), dapsone (18,28), photochemotherapy (10,18), and cyclosporin (29) have also been used with varying degrees of efficacy.

Leukocytoclastic vasculitis (LCV) is a histologic diagnosis characterized by segmental inflammation and fibrin deposition within blood vessels with fragmentation of polymorphonuclear leukocytes and formation of nuclear dust (leukocytoclasis). Infections, chemicals, foods, and drugs can precipitate LCV. LCV may also occur in association with chronic diseases and malignant neoplasms. No cause is identified in 60% of cases (30). The fluoroquinolones ciprofloxacin and ofloxacin have been reported previously as provocative agents of LCV (31,32), but this is the first reported case suggesting levofloxacin-induced LCV.


(1) Reshad H, Hargreaves GK, Vickers CF. Generalized pustular psoriasis precipitated by phenylbutazone and oxyphenbutazone. Br J Dermatol 1983; 109:I l 1-113.

(2) Shelley WB. Birch pollen and aspirin psoriasis. A study of salicylate hypersensitivity. JAMA 1964; 189:985-988.

(3) Baker H. Corticosteroids and pustular psoriasis. Br J Dermatol 1976; 94:83-88.

(4) Vine JE, Hymes SR, Warner NB, et al. Pustular psoriasis induced by hydroxychloroquine. A case report and review of the literature. J Dermatol 1996; 23:357-361.

(5) Katz M, Seidenbaum M, Weinrauch L. Penicillin-induced generalized pustular psoriasis. J Am Acad Dermatol 1987; 17:918-920.

(6) Ryan TJ and Baker H. The prognosis of generalized pustular psoriasis. Br J Dermatol 1971; 85:407-411.

(7) Gupta AK, Sibbald RG, Knowles SR, et al. Terbinafine therapy may be associated with the development of psoriasis de novo or its exacerbation: four case reports and a review of drug-induced psoriasis. J Am Acad Dermatol 1997; 36:858-862.

(8) Wakefield PE, Berger TG, James WD. Atenolol-induced pustular psoriasis. Arch Dermatol 1990; 126:968-969.

(9) Hu CH, Miller AC, Peppercorn R, et al. Generalized pustular psoriasis provoked by propranolol. Arch Dermatol 1985; 121:1326-1327.

(10) Lowe NJ and Ridgway HG. Generalized pustular psoriasis precipitated by lithium carbonate. Arch Dermatol 1978; 114:1788-1789.

(11) Barth JH and Baker H. Generalized pustular psoriasis precipitated by trazodone in the treatment of depression. Br J Dermatol 1986; 115:629-630.

(12) Georgala S, Rigopoulos D, Aroni K, et al. Generalized pustular psoriasis precipitated by topical calcipotriol cream. Int J Dermatol 1994; 33:515-516.

(13) Ogawa M, Baughman RD, Clendenning WE. Generalized pustular psoriasis: induction by topical use of coal tar. Arch Dermatol 1969; 99:671-673.

(14) De Silva BD, Benton EC, Tidman MJ. Generalized pustular psoriasis following withdrawal of oral cyclosporin treatment for palmo-plantar pustulosis. Clin Exper Dermatol 1999; 24:10-13.

(15) Lindgren S and Groth O. Generalized pustular psoriasis. Acta Derm Venereol 1976; 56:139-142.

(16) Shelley WB. Generalized pustular psoriasis induced by potassium iodide. JAMA 1967; 201:1009-1014.

(17) McFadyen T and Lyell A. Successful treatment of generalized pustular psoriasis (von Zumbusch) by systemic antibiotics controlled by blood culture. Br J Dermatol 1971; 85:274-276.

(18) Zelickson BD and Muller SA. Generalized pustular psoriasis. A review of 63 cases. Arch Dermatol 1991; 127:1339-1345.

(19) Rosen RM. Annular pustular psoriasis induced by UV radiation from tanning salon use. J Am Acad Dermatol 1991; 25:336-337.

(20) Park BS and Youn JI. Factors influencing psoriasis: an analysis based upon extent of involvement and clinical type. J Dermatol 1998; 25:97-102.

(21) Finch TM and Tan CY. Pustular psoriasis exacerbated by pregnancy and controlled by cyclosporin A. Br J Dermatol 2000; 142:582-584.

(22) Stewart AF, Battaglini-Sabetta J, Millstone L. Hypocalcemia-induced pustular psoriasis of von Zumbusch. Ann Intern Med 1984; 100:677-680.

(23) Farber EM and Nall L. Pustular psoriasis. Cutis 1993; 51:29-32.

(24) Zumbusch LR yon. Psoriasis und pustuloses Exanthem. Arch Dermatol Syphilol 1910; 99:335-336.

(25) Roujeau JC, Bioulac-Sage P, Bourseau C, et al. Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol 1991; 127:1333-1338.

(26) Rubin MG and Hanno R. Short term etretinate for pustular psoriasis. J Am Acad Dermatol 1985; 12:896-897.

(27) Stein KM, Shelley WB, Weinberg RA. Hydroxyurea in treatment of pustular psoriasis. Br J Dermatol 1971; 85:81-85.

(28) Macmillan AL and Champion RH. Generalized pustular psoriasis treated with dapsone. Br J Dermatol 1973; 88:183-185.

(29) Meinardi MM, Westerhof W, Bos JD. Generalized pustular psoriasis (von Zumbusch) responding to cyclosporin A. Br J Dermatol 1987; 116:269-270.

(30) Lotti T, Ghersetich I, Comacchi C, et al. Cutaneous small-vessel vasculitis. J Am Acad Dermatol 1998; 39:667-689.

(31) Choe U, Rothschild BM, Laitman L. Ciprofloxacin-induced vasculitis. N Engl J Med 1989; 320:257-258.

(32) Huminer D, Cohen JD, Majadla R, et al. Hypersensitivity vasculitis due to ofloxacin. Br Med J 1989; 299:303.



Michael Jude Welsch, MD

University of Chicago

Dept of Medicine--Section of Dermatology

5841 S. Maryland, MC 5067

Chicago, IL 60637-1470


Phone: (773) 702-1611

COPYRIGHT 2003 Journal of Drugs in Dermatology
COPYRIGHT 2003 Gale Group

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