Polyarteritis nodosa (PAN) presents pathologically as an ongoing segmental inflammatory response within the media of small and medium sized muscular arteries. This page is regarding Cutaneous polyarteritis nodosa, one that effects the skin.
PAN, c-PAN, classic polyarteritis nodosa, systemic vasculitis, necrotizing inflammatory lesions, hepatitis B–related PAN, HBV-related PAN, non–HBV-related PAN
Suggestive evidence for immunological involvement. This would include a hypersensitivity to certain infections, especially Group A strep, Hepatitis C, Human Immunodeficiency virus, Parvovirus B19 (Fifth disease).
• Tissue deposition of immune complexes • Hepatitis B antigenemia in 30% of cases • Hepatitis B antigen in circulating immune complexes • Hepatitis B antigen, complement and IgM demonstrated in vascular walls
Purpura, urticaria, subcutaneous hemorrhages, polymorphic rashes, subcutaneous nodules (uncommon but characteristic – tender lumps under the skin), persistent livedo reticularis and Raynaud's phenomenon (rare), blistering and ulcerations,
For cutanous polyarteritis nodosa a biopsy of the lesion is often performed
The diagnosis of PAN is made by first sampling accessible tissue by biopsy, preferably clinically abnormal tissue. The most accessible tissue sites for biopsy are skin, sural nerve, testes, and skeletal muscle. Histology reveals a focal necrotizing arteritis of generally mixed cellular infiltrate within the vessel wall.
The sural nerve may be affected in PAN, even in the absence of symptomatic neuropathy but with demonstrable abnormalities on nerve conduction. If a biopsy is obtained from the sural nerve, an entire cross-sectional segment of the nerve should be obtained to allow adequate sampling of epineurial arteries.(1)
Blood tests are generally not useful for PAN.
Cutaneous PAN usually runs a chronic course lasting from months to years with exacerbations and remissions. Neurological symptoms and muscular aches and pains usually resolve over a matter of months whilst skin lesions take longer to heal.
Remissions may occur spontaneously or after treatment with oral corticosteroids , cyclophosphamide or other immunosuppressive medications used to control the acute exacerbation and relieve pain. They can be stopped as symptoms become less severe or subside.
Non-steroidal anti-inflammatory drugs are also used and are suitable alternative first-line agents in patients when corticosteroids are contraindicated. Ulcerating skin lesions may be treated with bland topical preparations such as active manuka honey and covered with special dressings to improve healing. Occasionally skin grafts are advised but they may fail because of the damage to the blood vessels supplying nutrition to the skin. If the PAN is caused by streptococcal infection, it is wise to remain on long term penicillin to prevent further attacks such as streptococcal tonsillitis or cellulitis.
Viral infections may sometimes respond to a course of an antiviral agent.
In a few cases, over time cutaneous PAN may progress to systemic PAN. Patients with cutaneous PAN should be followed-up regularly so that any signs of progress to the systemic form may be picked up early. (2)
Rev Med Interne. 2005 Jan
Vignes S, Stoyanova M, Vasseur E, Haicault de la Regontais G, Hanslik T. Unité de Lymphologie, hôpital Cognacq-Jay, site Broussais, 102, rue Didot, 75014 Paris, France. email@example.com firstname.lastname@example.org
INTRODUCTION: 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.
CONCLUSION: 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.
Fleuret C, Kupfer-Bessaguet I, Prigent S, Hutin P, Staroz F, Plantin P.
Service de dermatologie, CHIC de Cornouaille, 29000 Quimper, France. email@example.com
INTRODUCTION: Periarteritis nodosa (PAN) is a form of vasculitis affecting the small and medium-sized arteries. Below, we report a case of cutaneous PAN relapsing in streptococcal infections over a period of 30 years and progressing towards systemic vasculitis.
CASE REPORT: A 35-year-old man was hospitalised for a retro-pharyngeal access associated with fever, arthralgia, myalgia and inflammatory subcutaneous nodules. Peripheral neurological signs were also seen with deficiency of the elevator muscles in the right foot. Examination of a biopsy from a nodule showed a characteristic image of PAN. Following drainage of the abscess, a favourable outcome was obtained with antibiotics and systemic corticosteroids. History taking showed that the patient had presented similar episodes since the age of 5 years involving arthralgia, myalgia and inflammatory subcutaneous nodules. These episodes appeared to follow a streptococcal infection, of which there was either clinical suspicion or objective elevation of antistreptolysin O (ASLO) titre. Skin biopsy resulted in diagnosis of cutaneous PAN 25 years earlier. In all cases, improvement was achieved by oral corticosteroids combined with treatment of the actual infection.
DISCUSSION: In addition to the classic association with hepatitis B, and occasionally hepatitis C, PAN may be associated with streptococcal infections. The cases of post-streptococcal PAN described in the literature are predominantly cutaneous, although it is not rare to find associated arthromyalgia and sensory neurological impairment. We examined three cases of cutaneous PAN with long-term follow-up described in the literature. They began in childhood and the outcome was benign, with no systemic manifestations. Our case differed in terms of the appearance of motor neurological involvement.
CONCLUSION: Post-streptococcal PAN of childhood onset generally carries a better prognosis than adult systemic forms. However, our case shows that on rare occasions, there may be very long progression complicated by systemic involvement.
Full Text Article
2008 Apr PubMed
2008 Apr Lippincott, Williams & Wilkins
2007 June Lippincott, Williams & Wilkins
2007 June Elsevier ScienceDirect
2006 May [SpringerLink
2006 Apr Journal of Korean Medical Science
2006 Mar Blackwell Synergy
2006 Jan-Mar PubMed
2005 Oct Elsevier ScienceDirect
M30.020: Polyarteritis nodosa, cutaneous
M30 Polyarteritis nodosa and related conditions M30.0 Polyarteritis nodosa M30.1 Polyarteritis with lung involvement [Churg-Strauss]
Allergic granulomatous angiitis
M30.2 Juvenile polyarteritis M30.3 Mucocutaneous lymph node syndrome [Kawasaki] M30.8 Other conditions related to polyarteritis nodosa
Polyangiitis overlap syndrome
2008 ICD-9-CM Diagnosis 446.0 Polyarteritis nodosa
446.0 also known as:
DiseaseDB Cutaneous periareritis nodosa