Reversible lower limb lymphedema as the first manifestation of cutaneous periarteritis nodosa
Vignes S, Stoyanova M, Vasseur E, Haicault de la Regontais G, Hanslik T.
Unite de Lymphologie, hopital Cognacq-Jay, site Broussais, 102, rue Didot, 75014 Paris, France. firstname.lastname@example.org
Cutaneous periarteritis nodosa is a localized form middle-sized arteriolitis. Its main clinical features are nodules, livedo reticularis and ulcers of the lower limbs. EXEGESIS: We reported a 45 year-old woman with distal lower limb lymphedema. Lymphedema initially spontaneously remitted but recurred nine months after, accompanied with nodules. Cutaneous periarteritis nodosa was diagnosed on histological findings and absence of extracutaneous manifestations.
Lymphoscintigraphy showed functional lymphatic insufficiency. Treatment consisted of prednisone followed by colchicine and dapsone. After six months of follow-up, patient remained symptom-free.
We report the first case of lower limb lymphedema associated with cutaneous periarteritis nodosa. Parallel course and unusual corticosensivity of lymphedema and nodules raised the problem of their non coincidental association.
PMID: 15639327 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... med_docsum
What is Cutaneous Polyarteritis Nodosa?
Cutaneous Polyarteritis Nodosa (also known as Cutaneous Periarteritis Nodosa or CPN) is a very rare disorder of the immune system. It is known as a vasculitis, which is inflammation of the blood vessels, and involves the medium and small-sized arteries found in the deeper layers of the skin. Therefore, these patients will present with skin problems. CPN is different from systemic polyarteritis nodosa (PAN), which can affect other organs including the liver, kidney, heart, lung, and gastrointestinal tract.
Already a very uncommon disease, CPN is even more rarely diagnosed in childhood. There is no association of degree of severity with age of onset. Both genders are equally affected.
SIGNS & SYMPTOMS:
Most skin manifestations start in the legs but the arms, trunk, head, and buttocks may also be affected. These include: multiple tiny lumps appearing under the skin, painful skin nodules, blistering and ulcerations, and livedo reticularis (lesions radiating out in a starburst formation).
• Nonspecific symptoms like feeling fatigued and unwell, fever, muscle aches, tenderness and stiffness, joint pain, and sore throat.
• Neurological symptoms include numbness, tingling, sensory changes, weakness, and absent reflexes.
• Occasionally patients with CPN will have high blood pressure.
The exact etiology of CPN is unknown, but scientists believe this is a disorder of the immune system. The body normally produces antibodies that target foreign particles called antigens to form immune complexes. These immune complexes cause the cells of the immune system to fight infection. In CPN, immune complexes are triggered to deposit and bind to the walls of arteries, causing the body’s immune system to attack these blood vessels. Possible triggers include infections like streptococcus, Hepatitis B and C, HIV, parvovirus B19 infection, and tuberculosis. Patients with inflammatory bowel disease may be at increased risk for getting CPN.
Your dermatologist will take surgical sample of your child’s skin lesion to look at under the microscope for diagnosis. A positive specimen will show inflammation of the all of the blood vessels in the lesion. It is important for your doctor to distinguish CPN from PAN because the clinical course and management is different. Therefore, other tests including chest x-ray, electrocardiogram, renal angiogram, and abdominal ultrasound may be done to rule out involvement of the other organs, which would lead to a diagnosis of PAN. Laboratory tests looking for infectious processes may help identify the trigger for CPN, but treating the infection may not change the course of disease.
Treatment involves controlling the inflammation in the blood vessels and pain relief. Oral steroids are the first line of treatment and are continued until symptoms become less severe. Disease flare-ups can be controlled with intermittent intravenous steroids. Your doctor may prescribe a non-steroidal anti-inflammatory drug like indomethacin for added inflammatory control as well as for pain relief. Other possible treatment regimens include low-dose methotrexate and cyclophosphamide. If streptococcal infection is diagnosed, your child may receive penicillin for treatment.
Ulcerating skin lesions may be treated with special dressings to improve healing. Sometimes skin grafts are done if the ulceration is severe.
Doctors have to distinguish cutaneous PAN from systemic PAN because the clinical course and management is very different. Systemic PAN usually has a more serious clinical course and prognosis is guarded. Patients with CPN have a less severe, but chronic course. The neurologic manifestations can go away in 2 to 8 weeks. Skin nodules may take years to clear. Remission can occur spontaneously or with steroid therapy, but patients may relapse and get the symptoms again. These exacerbations never seem to seriously affect the health of the individual. Because of the chronic, relapsing course of the disease, patients with CPN will have to be followed closely by their doctor. Overall, these patients are able to continue on with their regular daily activities as long as their disease process is under control.
Informative New Zealand website for patients and parents about cutaneous PAN. Graphic photographs are also included.
http://www.madisonsfoundation.org/conte ... sp?did=368