Panniculitides of particular interest to the rheumatologist

Abstract The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.

Clinical and Histopathological Study of 24 Cases with Cutaneous Polyarteritis Nodosa / 대한피부과학회지

BACKGROUND: Cutaneous polyarteritis nodosa (PAN) is a rare form of vasculitis relating to small-to-medium-sized arteries, it frequently causes impairment and presents a therapeutic challenge. Nevertheless, it remains an understudied disease and only a few clinical studies on cutaneous PAN with small number of patients have been conducted in Korea so far. OBJECTIVE: The aim of this study is to obtain a better understanding of the clinical and histopathological features for cutaneous PAN. METHODS: This was a retrospective study of cutaneous PAN observed at our center over an 12-year period. Clinical manifestations and histopathological features of 24 patients with cutaneous PAN were being evaluated. RESULTS: The ratio of males to females was 1 : 2.42 and the most frequently occurred age group was the third decade. The most commonly involved sites were the lower legs (100%), thighs (37.5%), arms (12.5%) and trunks (8.3%). The most common clinical findings were painful nodules, as seen in 62.5% (reticular erythema in 25%, scattered erythematous patches in 25%, and ecchymotic patches in 4.2%). Erythrocyte sedimentation rates are elevated in 61.9% of 21 patients. Histopathologically, the fibrinoid necrotizing vasculitis of small arteries in the deep dermis and panniculus were observed and the arteries were being classified into 4 stages: the acute stage (3.6%), subacute stage (57.2%), reparative stage (32.1%), and healed stage (7.1%). The most commonly treatment modality was systemic corticosteroids (62.5%). The clinical course was the chronically relapses and our study showed that the cutaneous PAN does not progress to the systemic PAN. CONCLUSION: This study suggested that the cutaneous PAN has a variable course, with repeated exacerbations, but does not progress to the systemic PAN. And no definitively effective therapy for cutaneous PAN has been established, additional prospective studies are necessary in order to determine the effectiveness of the various treatment modalities.

Poliarteritis nodosa cutánea atípica Una forma atípica de la presentación de la poliarteritis nodosa cutánea / No presenta

En este artículo presentamos un caso inusual de poliarteritis nodosa cutánea (PANC), en donde se presentan lesiones nodulares eritematodolorosas que comprometen muchas zonas del cuerpo, incluyendo miembros inferiores, superiores, glúteos, tronco, glándula mamaria derecha y cuero cabelludo. Las lesiones histológicas son características de vasculitis. Este tipo de manifestación clínica no había sido informada previamente en la literatura, por lo cual consideramos que es una forma atípica de presentación de la PANC.

In this article we present an unusual case of cutaneous polyarteritis nodosa (CPAN), which are painful erythematous nodular lesions that engages many areas of the body, including superior and inferior legs, buttocks, trunk, mammary gland and scalp. The histological lesions are characteristic of vasculitis. This type of clinical manifestation has not been reported previously in the literature, so we believe that this is an atypical form of the CPAN.

Poliarterite nodosa cutânea na infância com gangrena digital e possível associação com o estreptococo beta-hemolítico do grupo a: relato de caso e revisão de literatura / Cutaneous polyarteritis nodosa in children presenting with digital gangrene and possible association with group a beta hemolytic streptococcus infection

A poliarterite nodosa cutânea (PAN cutânea) é uma forma particular de vasculite envolvendo artérias de pequeno e médio calibres. A doença pode ser diferenciada da poliarterite nodosa sistêmica pela sua limitação à pele e ausência de envolvimento visceral. Manifesta-se habitualmente por nódulos subcutâneos, livedo reticular e ulceração cutânea. Artralgia, mialgia, neuropatia e sintomas constitucionais, como febre e mal-estar, podem estar presentes. O diagnóstico é, por vezes, de difícil realização, baseando-se na forte suspeita clínica e no exame histológico da pele. Neste trabalho é descrito um caso de PAN cutânea em uma criança de 9 anos com alteração isquêmica grave da ponta dos dedos e nódulos subcutâneos como manifestações iniciais e a provável associação com o estreptococo beta-hemolítico grupo A (EBHGA). A revisão de literatura revela que PAN cutânea na infância está freqüentemente associada à infecção estreptocócica e que atenção especial deveria ser dada a crianças com menos de 10 anos de idade pelo alto risco relatado de auto-amputações.

Cutaneous polyarteritis nodosa (cutaneous PAN) is a particular form of vasculitis involving small and medium sized arteries. The disease can be differentiated from classic PAN by its limitation to the skin and lack of progression to visceral involvement. Usually it manifests with subcutaneous nodule, livedo reticularis and cutaneous ulceration. Arthralgia, myalgia, neuropathy and constitutional symptoms such as fever and malaise may also be present. Sometimes the diagnosis is difficult, being just based on clinical suspicion and skin histologic exam. In this paper the authors describe the case of cutaneous PAN in a 9 years old child with severe ischemic changes in the fingertips, and subcutaneous nodules as initial signs of cutaneous PAN and probable association with the group A beta hemolytic streptococcus (GABHS). The review of the literature shows that the cutaneous PAN in the childhood may be associated to streptococcal infection and, for this reason, a special care should be given for those under 10 years old due to the high risk of self amputation.

Five Cases of Cutaneous Polyarteritis Nodosa Treated with Dapsone / 대한피부과학회지

Cutaneous polyarteritis nodosa (PAN) is a benign form of vasculitis of small and medium-sized arteries with chronic benign course without systemic involvement. Corticosteroids are the mainstay of treatment for cutaneous PAN but aspirin and non steroidal anti-inflammatory drugs (NSAIDs) are also used for conservative treatment. We treated five patients with cutaneous PAN using oral dapsone. All patients initially showed good response to dapsone, but exacerbation occurred once the dose of dapsone was tapered or stopped. Patients had mild side effects of dapsone which were hemolytic anemia (4 cases) and abnormal liver function (2 cases). No patient has developed major organ system involvement during 1 to 13 years of follow-up. Instead of using oral Corticosteroid, oral dapsone may be an effective and relatively safe alternative therapy for chronic benign cutaneous PAN.

Three Cases with Cutaneous Polyarteritis Nodosa / 대한류마티스학회지

Systemic polyarteritis nodosa is a necrotizing vasculitis that involves small and medium-sized muscular arteries in the multiple organ systems, whereas cutaneous polyarteritis nodosa (CPAN) is a localized disease characterized by necrotizing vasculitis of small and medium-sized arteries in the skin without life-threatening organ involvement. CPAN is usually limited to skin, muscle, and joints. It is chronic but takes a benign course. The most common cutaneous manifestations include nodules, ulcers and a livedo reticularis pattern on the extremities. We describe three cases with CPAN showing necrotizing vasculitis on the skin without systemic symptoms or visceral involvement. In Korea, most previously published cases with CPAN have shown a good response to a short term of corticosteroids or colchicine administration. However, two of our three patients required another immunosuppressive agent in addition to corticosteroids to manage their intractable skin lesions, and the remaining one showed a good response to a short course of prednisolone. In addition, there was one patient with CPAN having hepatitis B surface antigen, which might be associated with his refractory skin disease.

A Case of Cutaneous Polyarteritis Nodosa Associated With Chronic Hepatitis B Infection / 대한피부과학회지

Polyarteritis nodosa (PAN) is regarded as an immune-complex mediated vasculitis and can present as extrahepatic manifestation of hepatitis B virus (HBV) infection. When treating HBV-associated PAN with immunosuppressive agent such as systemic steroid, the possibility of viral reactivation should be kept in mind and eagerly checked. Herein we report a case of HBV-associated cutaneous polyarteritis nodosa and its treatment outcome with combination therapy of steroid and antiviral agent, lamivudine for 6 weeks. Our experience suggests that more clinical data should be collected to determine the proper indication and end-point of antiviral therapy in chronic HBV carriers who receive immunosuppressant therapy.

A Case of Cutaneous Polyarteritis Nodosa

Cutaneous polyarteritis nodosa(CPAN) is a benign form of rare vasculitis of small and medium-size arteries with a recurrent but benign course without systemic involvement. We experienced a 61-year-old male who had two months history of multiple deep-purpurish livedo reticularis on both lower legs. Noncutaneous manifestations including malaise, fever, myalgia, and arthritis were absent. A skin biopsy specimen from the livedo reticularis on the leg showed perivascular and trans-mural neutrophilic and lymphocytic infiltration of medium-sized arteries in the dermal-subcutaneous junction and fibrinoid necrosis of the vessel walls. The patient was treated with colchicine for 2months and showed markded improvement.