Subacute Nodular Migratory Panniculitis (Vilanova Disease) 

Updated: Apr 17, 2017
Author: Dirk M Elston, MD; Chief Editor: William D James, MD 

Overview

Background

Vilanova disease, or subacute nodular migratory panniculitis, was first observed in 1954[1] and then named by Vilanova and Pinol Aguade in 1956.[2] 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.[3, 4, 5, 6]

Pathophysiology

This is a disease of the subcutaneous septa and the blood vessels in the legs.[7] 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.

Epidemiology

Frequency

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

Race

No racial predilection is apparent.

Sex

It is more common in women than in men.[8]

Age

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

Prognosis

The prognosis for this condition is good. It typically responds to treatment. However, the condition often recurs. This condition has resulted in no mortality.

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.

 

Presentation

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 Examination

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.[9]

 

DDx

Diagnostic Considerations

Also consider the following:

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

Differential Diagnoses

 

Workup

Laboratory 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.[10]

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 or adjacent deep dermis.[11] 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.[12]

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

Medical Care

Treatment with intralesional steroids may be effective. Otherwise, systemic medications, such as potassium iodide, dapsone, or indomethacin are used.[13, 14, 15, 16]

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

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.

Complications

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

Long-Term Monitoring

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.

 

Medication

Medication Summary

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.

Iodine products

Class Summary

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

Potassium iodide (SSKI, Pima)

Potassium iodide is most commonly used therapy for this condition. It works via potassium concentration in granulomas, which releases heparin and inhibits delayed-type hypersensitivity response. Response should be seen in all patients in 2-3 weeks.

Leprostatic agents

Class Summary

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.

Dapsone (Avlosulfon)

Dapsone is bactericidal and bacteriostatic against mycobacteria; its mechanism of action is similar to that of sulfonamides, where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth.

Triamcinolone (Aristocort, Aristospan)

Triamcinolone decreases inflammation by suppressing migration of PMN leukocytes and reversing capillary permeability. Intralesional injections may be used for localized skin disorders.