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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Relapsing polychondritis is an infrequently diagnosed, though not necessarily uncommon, systemic disorder characterized by recurrent and potentially destructive inflammation of cartilaginous structures, the eye, and the audiovestibular and cardiovascular systems. Although dermal involvement occurs in approximately 25% of patients with relapsing polychondritis, in only few cases has a skin biopsy been obtained revealing lesions such as leukocytoclastic vasculitis, livedo reticularis, erythema nodosum or keratodermia blenorrhagicum. We describe a patient with relapsing polychondritis in whom a cutaneous polyarteritis nodosa preceded cartilage inflammation by 6 months. Cutaneous polyarteritis nodosa is a rare form of vasculitis that appears to be limited primarily to the skin, muscles, and joints. In contrast to the systemic form of the disease it is characterized by the absence of visceral lesions and a relapsing but benign course. The present case and the fact that vasculitis is a concomitant feature in approximately 30% of patients with relapsing polychondritis [21] demonstrates that this condition may not represent a distinct clinical entity.
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PMID:Relapsing polychondritis presenting as cutaneous polyarteritis nodosa. 809 25

Cutaneous polyarteritis nodosa (CPAN) is a benign form of vasculitis of small and medium-size arteries with a recurrent but benign course without systemic involvement. This entity is very rare in children, with about 45 cases described in the literature we reviewed. Herein we report a 10-year-old girl with typical CPAN. Trigger factors such as streptococcal infection were not detected. Of four episodes over the last 2 years, only one required treatment with a moderate-sized oral dose of prednisone. In diagnosing CPAN, it is necessary to rule out systemic polyarteritis nodosa, which is also rare in children, as well as other panniculitides and vasculitides.
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PMID:Cutaneous polyarteritis nodosa in a child. 957 91

Cutaneous polyarteritis nodosa (CPN) is an uncommon form of vasculitis. It exists as a separate entity, though bearing similar name with polyarteritis nodosa (PAN) which is an aggressive systemic vasculitis with multi-organ involvement. CPN runs a chronic but benign course. Its aetiology is unknown and it usually presents with painful nodules on the legs with mild constitutional symptoms, and extracutaneous features of arthralgias, arthritis, neuropathy and myopathy. No mortality has been reported thus far. It is therefore important to distinguish CPN apart from PAN. Symptomatic treatment with judicial use of systemic steroids and anti-inflammatory agents will suffice in most cases.
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PMID:Cutaneous polyarteritis nodosa: a case report and literature review. 1010 67

Cutaneous vasculitides continue to be difficult to diagnose and treat because of the complexity of these disorders. Current classification schemes are based on clinical findings and histopathologic criteria, so biopsy of an early evolving lesion is the most effective means of establishing the diagnosis. We describe the case of a 74 years old man suffering from livedo reticularis and painful nodular lesions, with ulcers, on the legs. Establishing a precise diagnosis has required biopsy of the lesions which showed histopathologic feature of cutaneous polyarteritis nodosa and the designation of cutaneous vasculitis as "isolated" or "primary" was based on lack of systemic disease documented by clinical findings, laboratory tests and instrumental investigations. Remission occurred after moderate doses of steroid therapy and after a year of management with low dose of steroid therapy the patient experienced significant improvement. Cutaneous polyarteritis nodosa is usually a benign disease, however it may relapse, and a long range follow up of the patient is necessary because of the possibility of systemic involvement.
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PMID:[Polyarteritis nodosa cutanea: a case report]. 1287 45

The purpose of this study was to present the clinical courses and histologic findings of 4 children with cutaneous vasculitis characterized by tender cutaneous nodules and fever in the absence of major organ involvement. We conducted a retrospective chart review of 4 patients with cutaneous vasculitis followed up for a mean of 68 months (range, 12-114 months). The patients included 3 boys and 1 girl (ages at onset, 2-10 years). Clinical and laboratory manifestations included tender erythematous cutaneous nodules (n = 4/4), fever 39 degrees C or higher (4/4), nondeforming arthritis (3/4), leukocytosis and elevated erythrocyte sedimentation rate (4/4), positive antinuclear antibodies (1/4), and elevated streptococcal enzymes (3/4). Skin biopsy results showed inflammation of medium-sized cutaneous arteries with a mixed inflammatory cell infiltrate consistent with cutaneous polyarteritis nodosa (4/4). Patients were treated with prednisone with good initial response, but exacerbation occurred once prednisone was tapered. Additional medications given were methotrexate (2/4), dapsone (2/4), colchicine (1/4), and cyclophosphamide (1/4). One patient is in clinical remission after 48 months of disease; the others have continuing disease that requires treatment. Patients with evidence of streptococcal infection received oral penicillin prophylaxis; two of the three patients had recurrent attacks of vasculitis despite penicillin. No patients have developed major organ system involvement after 12 to 114 months of follow-up. Cutaneous polyarteritis nodosa in children is a recognizable entity characterized by painful nodules, fever, absence of major organ involvement, and chronic or recurrent course. Patients should be screened for streptococcal infection and treated with antibiotics when needed.
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PMID:Cutaneous polyarteritis nodosa in children. 1619 7

Cutaneous polyarteritis nodosa (CPAN) is an uncommon form of vasculitis involving small and medium sized arteries of unknown etiology. The disease can be differentiated from polyarteritis nodosa by its limitation to the skin and lack of progression to visceral involvement. The characteristic manifestations are subcutaneous nodule, livedo reticularis, and ulceration, mostly localized on the lower extremity. Arthralgia, myalgia, peripheral neuropathy, and constitutional symptoms such as fever and malaise may also be present. We describe a 34-yr-old woman presented with severe ischemic change of the fingertip and subcutaneous nodules without systemic manifestations as an unusual initial manifestation of CPAN. Therapy with corticosteroid and alprostadil induce a moderate improvement of skin lesions. However, necrosis of the finger got worse and the finger was amputated.
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PMID:Cutaneous polyarteritis nodosa presented with digital gangrene: a case report. 1661 34

Recently described macular arteritis presents as asymptomatic hyperpigmented macules, runs a chronic, indolent course, and shows lymphocytic arteritis at various stages of evolution ranging from fibrinoid necrosis to endarteritis obliterans. Herein, we present another case that was clinically suspected to be unilateral plantar pompholyx. A 47-year-old male presented with a 2-month history of persistent, reticulated, asymptomatic, nonblanching erythematous and brawny macules, and scattered, slightly scaly papules over the plantar instep of his left foot. Two punch biopsies extending to the subcutis revealed healed arteritis (endarteritis obliterans with fragmented elastic lamina) in the subcutis in one biopsy and purpura and hemosiderin deposition surrounding small subcutaneous arterioles and venules in the second. Additional histologic features included lymphocytic eccrine hidradenitis and chronic spongiotic dermatitis. Extensive laboratory work did not reveal evidence of systemic disease. Despite topical corticosteroid therapy, he has had persistent, asymptomatic disease after 6 months follow-up. The differential diagnoses considered included pigmented purpuric dermatitis, perniosis (chilblains), plantar eccrine hidradenitis, Buerger disease, livedo vasculopathy, and cutaneous polyarteritis nodosa. Cutaneous polyarteritis nodosa also runs a benign course and is denoted by muscular vessel vasculitis, which can resolve with retiform hyperpigmentation. Based on these overlapping clinical-pathologic features, macular arteritis might represent a latent, non-nodule forming chronic variant of cutaneous polyarteritis nodosa.
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PMID:"Macular arteritis": a latent form of cutaneous polyarteritis nodosa? 1836 Jan 18

Adult onset Still's disease (AOSD) is a well recognized clinical disorder characterized by a typical rash. However, there have been several atypical cutaneous findings reported in patients with AOSD. Cutaneous polyarteritis nodosa (PAN) is a necrotizing vasculitis of the small and medium sized arteries, distinguishing itself from systemic PAN by its restriction to the skin and to the neurological and osteo-muscular systems. We report the case of a patient with AOSD who is unique in having developed cutaneous polyarteritis nodosa (PAN) during the active phase of her disease. To the best of our knowledge, no previous cases of AOSD associated with a cutaneous variant of PAN have been reported.
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PMID:Cutaneous polyarteritis nodosa in adult onset Still's disease. 1959 25

Sixteen cases of cutaneous polyarteritis nodosa referred to our Department from 1985 to 2003 were studied clinically and histopathologically. Laboratory data, treatments and clinical courses were also evaluated retrospectively. All cases had nodules and/or indurated erythemas on their lower extremities. All cases showed necrotizing vasculitis of small muscular arteries in the subcutaneous tissues and/or occlusion of those arteries histopathologically. Fifteen cases also had accumulation of plasma protein in vessels of the dermis and subcutaneous tissues. Laboratory data showed high activity of platelets and coagulation in some cases. Eleven cases had been effectively treated with non-steroidal anti-inflammatory drugs. Eight cases were observed for at least 5 years (the longest for approximately 19 years) and had good prognoses and no systemic involvement. Cutaneous polyarteritis nodosa seems to be a benign disease, and differs from systemic polyarteritis nodosa although their histopathological features are common. Cutaneous polyarteritis nodosa might involve local dysfunction of the circulation from the dermis to the subcutaneous area. A review of the published work shows that the cause(s) of most cases of cutaneous polyarteritis nodosa is unknown, that no controlled trials for treatment of cutaneous polyarteritis nodosa compared to polyarteritis nodosa have been reported, and that no definitively effective therapy for cutaneous polyarteritis nodosa has been established.
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PMID:Cutaneous polyarteritis nodosa: a report of 16 cases with clinical and histopathological analysis and a review of the published work. 2017 28

Cutaneous polyarteritis nodosa is an infrequent necrotizing vasculitis of small and medium-sized arteries, with a recurrent and chronic course, that can be associated with fever, arthralgia, myalgia and neuropathy without visceral involvement. We report a cutaneous polyarteritis nodosa case.
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PMID:[Childhood cutaneous polyarteritis nodosa]. 2054 27


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