AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Vilanova disease, or subacute nodular migratory panniculitis, was first observed in 1954 and then named by Vilanova and Aguade in 1956. Vilanova's original 14 patients were all women, most of whom were in their fifth decade of life. Since then, this condition has been diagnosed in men and women.

The nomenclature of this and related diseases is confusing, and some authors believe that Vilanova disease is merely a version of erythema nodosum because the histology is the same. The term chronic erythema nodosum is often used interchangeably with subacute nodular migratory panniculitis.

Pathophysiology

This is a disease of the subcutaneous septa and the blood vessels in the legs. This type of panniculitis exhibits greater septal thickening, granulomatous infiltration of the septa, and an absence of phlebitis. Although alpha1-antitrypsin deficiency, infectious etiology (streptococcal infection), and thyroid disease have been suggested, no specific etiologic agent has been found.

Frequency

United States

Because of the ambiguity of this diagnosis versus other closely related conditions, no frequency has been determined.

Mortality/Morbidity

This condition has resulted in no mortality. It responds well to treatment, but recurrences are possible.

Race

No racial predilection is apparent.

Sex

It is more common in women than in men.

Age

Vilanova disease has most frequently been reported in the third to sixth decades of life.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

• Most commonly, patients describe a painless red nodule on the anterolateral aspect of the lower extremities that enlarges via centrifugal expansion or by the confluence of other nodules in proximity. Some nodules may exhibit central clearing with a morpheaform appearance. Usually, no trauma to the affected area is reported.
• Some patients describe concurrent sore throat, fever, or arthralgias.

Physical

• The examination typically reveals a unilateral, single, discrete, erythematous nodule or plaque on the anterolateral part of a lower extremity.
• The lesion displays peripheral extension, and the central portion may have a yellowish hue later in the course.
• A characteristic lack of ulceration of these lesions is noted.
• Over weeks or months, the nodules migrate (hence the name) and can become crescentic in nature.

Causes

Although this disease has been likened to erythema nodosum, the typical causative agents for classic erythema nodosum are not applicable to Vilanova disease. No obvious cause is apparent, but some reports document streptococcal infection in some patients.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Weber-Christian disease
Pancreatic panniculitis
Panniculitis artefacta
Alpha1-antitrypsin deficiency–associated panniculitis
Lupus panniculitis

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• The erythrocyte sedimentation rate is commonly elevated in patients with Vilanova disease.
• The antistreptolysin O titer should be checked because it is elevated in some patients.
• The physician should perform a tuberculin skin test because other diseases in the differential diagnosis can have tuberculous etiologies.
• Serum rheumatoid factor levels have been elevated in some patients, although the reason for this is not known.
• A CBC count can be performed to rule out infection, especially if the patient has described a sore throat or a fever.
• An antinuclear antibody or anti-dsDNA antibody test can be ordered to help rule out lupus panniculitis.
• An alpha1-antitrypsin level can be ordered to help rule out deficiency-induced panniculitis.
• Anti-DNAse B titers also may be elevated, as they are often elevated in various forms of panniculitis.
• Several cases have been reported of Vilanova disease associated with thyroid disease. Thyrotropin and free T4 levels should also be checked.

Imaging Studies

• A chest radiograph should be obtained to rule out sarcoidosis and tuberculosis.

Procedures

• A skin biopsy is necessary to make the diagnosis.

Histologic Findings

Subacute nodular migratory panniculitis is histologically characterized by granulation tissue–like capillary proliferation and septal widening secondary to granulomas and fibrosis. A mild lymphocytic and giant cell inflammation is observed, often lining the walls of the septa. Eosinophils and neutrophils may also be observed. The endothelial cells of the small vessels proliferate and can fill the entire lumen. The capillaries can take on a coiled, swollen appearance. Little to no vasculitis or phlebitis is present. The septa of the subcutaneous tissue exhibit granulomatous changes and notable changes in collagen fibers.

In contrast, chronic erythema nodosum exhibits small vessel vasculitis and only mild septal change. Disease may extend to the adjacent fat lobules, and the inflammation often extends up to the deep dermis. Septal fibrosis is not present in chronic erythema nodosum.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

Treatment with intralesional steroids may be effective. Otherwise, systemic medications, such as potassium iodide or dapsone, are used.

Surgical Care

No surgical treatment is indicated.

Consultations

Consult a dermatologist to perform a skin biopsy.

Diet

No specific dietary requirements are indicated.

Activity

Activity is ad lib, taking care to avoid trauma to affected areas.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Therapy for this condition is not mandatory, and several factors should be weighed prior to treatment. The factors that should be considered include the extent to which the disease disturbs the patient and the potential adverse effects of the medication.

Drug Category: Iodine products

Treatment with intralesional steroids may be effective. Otherwise, systemic medications, such as potassium iodide or dapsone, are used.

Drug Category: Leprostatic agents

These agents may have immunomodulatory effects. Dapsone has been reported as being a successful treatment of subacute migratory panniculitis, but treatment is not well established.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• Inpatient hospitalization is not required.

Further Outpatient Care

• Patients should be monitored for adverse effects of medications and progress of treatment for 1-2 months or for the duration of therapy. Otherwise, routine follow-up care should be performed on an outpatient basis. As mentioned previously, relapses are common.

In/Out Patient Meds

• Patients should be treated as mentioned above (see Medical Care and Medication). When the condition subsides, no prophylaxis is necessary.

Complications

• No severe complications have been reported for this condition. Complications from medical therapy can occur.

Prognosis

• The prognosis for this condition is good. It typically responds to treatment. However, the condition often recurs.

Patient Education

• Patients should be informed that the risks of treatment of this condition might outweigh the benefits. The decision to treat should be on an individual basis.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Pitfalls would be most likely situated around unusual complications of drug therapy.

Special Concerns

• In pregnant patients, the oral medications should not be used. Intralesional steroids or simply no treatment are the best alternatives.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Bafverstedt B. Erythema nodosum migrans. Acta Derm Venereol. 1954;34(3):181-93. [Medline].
• De Almeida Prestes C, Winkelmann RK, Su WP. Septal granulomatous panniculitis: comparison of the pathology of erythema nodosum migrans (migratory panniculitis) and chronic erythema nodosum. J Am Acad Dermatol. 22(3):477-83. [Medline].
• Fine RM, Meltzer HD. Chronic erythema nodosum. Arch Dermatol. Jul 1969;100(1):33-8. [Medline].
• Lee UH, Yang JH, Chun DK, Choi JC. Erythema nodosum migrans. J Eur Acad Dermatol Venereol. Jul 2005;19(4):519-20. [Medline].
• Montgomery H, O'Leary P, Barker N. Nodular vascular diseases of the legs. JAMA. 1945;128:335-41.
• Niemi KM, Forstrom L, Hannuksela M, et al. Nodules on the legs. A clinical, histological and immunohistological study of 82 patients representing different types of nodular panniculitis. Acta Derm Venereol. 1977;57(2):145-54. [Medline].
• Perry HO, Winkelmann RK. Subacute nodular migratory panniculitis. Arch Dermatol. Feb 1964;89:170-9. [Medline].
• Ross M, White GM, Barr RJ. Erythematous plaque on the leg. Vilanova's disease (subacute nodular migratory panniculitis). Arch Dermatol. Dec 1992;128(12):1644-5, 1647. [Medline].
• Schulz EJ, Whiting DA. Treatment of erythema nodosum and nodular vasculitis with potassium iodide. Br J Dermatol. Jan 1976;94(1):75-8. [Medline].
• Vilanova X, Pinol Aguade J. Subacute Nodular Migratory Panniculitis. Br J Dermatol. Feb 1959;71(2):45-50. [Medline].

Article Last Updated: Feb 26, 2007