Haviv Ruby, Capua Maya, Amir Jacob, Harel Liora
Department of Pediatrics C, Schneider Children's Medical Center of Israel, Tel Aviv University, Sackler School of Medicine, Petach Tikvah, Israel.
Department of Pediatrics C, Schneider Children's Medical Center of Israel, Tel Aviv University, Sackler School of Medicine, Petach Tikvah, Israel ; Department of Pediatrics Children's Hospital at, Montefiore, New-York City, NY USA.
Pediatr Rheumatol Online J. 2014 Oct 10;12:46. doi: 10.1186/1546-0096-12-46. eCollection 2014.
Cutaneous Polyarteritis Nodosa (cPAN) was first described in 1931. cPAN is considered a rare disease, its true incidence is unknown. The age of onset is diverse. Most studies have shown no significant gender predominance. cPAN presents with distinct skin findings, such as a maculopapular rash, subcutaneous nodules, livedoid vasculitis, panniculitis, ischemic finger lesions, or erythematous patchy rash. Etiology is unclear. It is still believed to be an immune complex-mediated disease, although a possible mechanism recently proposed relates a familial form of the disease to impaired activity of Adenosine Deaminase 2. cPAN may reflect an underlying disease, infection or medical treatment. There is no consensus as to initial treatment, dosage and length of treatment. Patients with constitutional symptoms, visceral involvement, a more severe course of the disease, or high acute phase reactants, were treated mainly with systemic corticosteroids and/or cytotoxic agents for varying durations. However, persistence of cutaneous lesions has been documented. We describe a 14 year old male suffering from persistent cPAN, with no constitutional symptoms or involvement of internal organs. The patient was treated with a local corticosteroid-based ointment during exacerbations, until complete remission. Although reported in only one study, treatment with topical corticosteroid compound may result in significant improvement or complete regression of skin lesions in cPAN patients.
皮肤结节性多动脉炎(cPAN)于1931年首次被描述。cPAN被认为是一种罕见疾病,其确切发病率尚不清楚。发病年龄各异。大多数研究表明,该病无明显的性别优势。cPAN具有独特的皮肤表现,如斑丘疹、皮下结节、青斑样血管炎、脂膜炎、缺血性手指病变或红斑性斑疹。病因尚不清楚。尽管最近提出的一种可能机制将该疾病的家族形式与腺苷脱氨酶2活性受损联系起来,但人们仍然认为它是一种免疫复合物介导的疾病。cPAN可能反映潜在疾病、感染或医疗治疗。关于初始治疗、治疗剂量和疗程尚无共识。有全身症状、内脏受累、病情较重或急性期反应物水平高的患者,主要接受不同疗程的全身糖皮质激素和/或细胞毒性药物治疗。然而,已有皮肤病变持续存在的记录。我们描述了一名14岁男性,患有持续性cPAN,无全身症状或内脏受累。患者在病情加重期间接受局部糖皮质激素软膏治疗,直至完全缓解。尽管仅在一项研究中报道,但局部糖皮质激素复方制剂治疗可能会使cPAN患者的皮肤病变显著改善或完全消退。