Subacute Nodular Migratory Panniculitis (Vilanova Disease)
Updated: May 13, 2022
Author: Dirk M Elston, MD; Chief Editor: William D James, MD
Overview
Practice Essentials
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 terms chronic erythema nodosum and erythema nodosum migrans are often used interchangeably with subacute nodular migratory panniculitis.[3, 4, 5, 6, 7]
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.
Complications
Koebnerization to plaque psoriasis has been reported.[8]
Diagnostics
A chest radiograph should be obtained to rule out sarcoidosis and tuberculosis.
A skin biopsy is necessary to make the diagnosis.[9]
Also see Workup.
Treatment
See Medical Care.
Consultations
Consult a dermatologist to perform a skin biopsy.
Background
Pathophysiology
This is a disease of the subcutaneous septa and the blood vessels in the legs.[10] 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
Because of the ambiguity of this diagnosis versus other closely related conditions, no frequency has been determined.
No racial predilection is apparent, but it is more common in women than in men.[11]
Vilanova disease has most frequently been reported in the third to sixth decades of life.
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 in most cases, but reports document streptococcal infection, viral infection, and medications as causes in some patients.[12, 13, 14]
DDx
Diagnostic Considerations
Also consider the following:
-
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.
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.[15] 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.[16]
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.[17, 18, 19, 20]
In pregnant patients, the oral medications should not be used. Intralesional steroids or simply no treatment are the best alternatives.
In a single reported case of idiopathic Vilanova disease in a 30-year-old man, treatment with saturated solution of potassium iodide and topical heparin was successful.[7]
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.