A 50-year-old man presented with cutaneous small vessel vasculitis CSVV 皮肤小血管炎

A 50-year-old otherwise healthy man is present with 10 months of tender skin rashes with mild itch, reddish-brown spots over the arms, legs, buttocks and torso. Patients recalled the crops of purple spots appearing after his Europe business trip one year ago. The lesion spreaded to his arms and legs over the next 2 weeks, and then the buttocks later on. He was diagnosed with cutaneous small-vessel vasculitis (CSVV) and was given steroids cream which was unavailing. Then he was administrated with 2 weeks of oral steroids prednisolone. The itch tuned down significantly, but the reddish-brown lesion did not subside. 

Laboratory studies were performed two months ago and showed a serum SGPT/ALT level of 71 units per liter (reference range, less than 51 units per liter) and serum Gamma GT level of 85 units per liter (reference range, less than 70 units per liter), and serum lipid level, Total Cholesterol, HDL, LDL, CHolesterol/HDL Ratio, and Triglycerides boardline high. Urinalysis showed urine Glucose +++, Urobilinogen +, Bacteria +, White Blood Cells levels of 8 per unit volume (reference range, less than 7). Oral steroids was discontinued.

He came to seek a third opinion. Upon examination, palpable purpura and nonblanching petechiae were presented on areas of the hand, arms, buttocks and legs.  The scaling on shiny erythematous plaques of the lower limbs is minimal. He was prescribed with herbal medicinal powder Originax and Derma-zema and was told to take it twice daily. He was then reviewed every 4 weeks with his progress tracked by photographs and blood/urine test scheduled at week 12. The lesions improved significant with herbal medicine within 8 weeks of treatment. However, patient did not show up for the fouth consulation at week 12.

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Discussion

Cutaneous small-vessel vasculitis (CSVV), formerly called hypersensitivity angiitis, drug-induced vasculitis, leukocytoclastic vasculitis, cutaneous leukocytoclastic angiitis, serum sickness, serum sickness-like reactions, and allergic vasculitis, is classified as a skin-related autoimmune disorder—a disease which occurs when the body’s immune system mistakenly attacks blood vessels, which can restrict blood flow and damage vital organs and tissues [1]. CSVV is very rare, with an estimated 38 to 55 per million adults per year diagnosed with CSVV annually.

The cause of CSVV is unkown, but CSVV can be triggered by an allergic reaction to drugs, such as antibiotics, diuretics and blood pressure medicines, as well as foods or food additives. Other triggers also includes upper respiratory tract infections, and viruses such as hepatitis B or C, and HIV. There is no type of test that diagnoses CSVV, but a skin biopsy is typically helpful, which shows the inflammation of the small blood vessels that confirms the diagnosis of CSVV [2-5].

一名 50 岁的健康男性出现 10 个月的皮疹，伴有轻度瘙痒，手臂、腿部、臀部和躯干出现红褐色斑点。患者回忆起一年前他在欧洲出差后出现的大量丘疹，接下来的 2 周皮损蔓延到他的手臂和腿部，后来又蔓延到臀部。他被诊断出患有皮肤小血管炎 CSVV，并给予类固醇乳膏，但无效。随后给予口服激素泼尼松龙2周，瘙痒明显减轻，但红褐色皮损未消退。

两个月前进行的血液检测报告显示血清 SGPT/ALT 水平为 71 单位/升（参考范围，低于 51 单位/升）和血清 Gamma GT 水平为 85 单位/升（参考范围，低于 70 单位/升) 和血脂水平、总胆固醇、HDL、LDL、胆固醇/HDL 比率和甘油三酯水平偏高。尿液分析显示尿液葡萄糖+++，尿胆原+，细菌+，白细胞水平为8（参考范围，小于7）。患者停用口服类固醇。

他来寻求第三方意见。经检查，手、手臂、臀部和腿部区域有可触及的紫癜和按之不发白的瘀血斑点。下肢有光泽的红斑斑块上的鳞屑很少。处方中药Originax 和 Derma-zema，每天服用两次。然后每 4 周对他进行一次复查，并通过照片和安排在第 12 周进行的血液/尿液检查来跟踪他的进展。在开始治疗后的 8 周内，皮肤小血管炎皮损显着改善，遗憾的是患者没有来第12周的复诊。

讨论

皮肤小血管炎 (CSVV)，以前称为过敏性血管炎、药物性血管炎、白细胞破碎性血管炎、皮肤白细胞破碎性血管炎、血清病、血清病样反应和过敏性血管炎，被归类为皮肤相关的自身免疫性疾病，当自体的免疫系统错误地攻击自己皮肤小血管时会发生这种情况，这会阻碍血流并损害重要的器官和组织[1]。 CSVV 非常罕见，估计每年每百万成年人中有 38 至 55 人被诊断出患有 CSVV。

CSVV 的病因尚不清楚，但 CSVV 可能由对药物（如抗生素、利尿剂和降压药）以及食品或食品添加剂的过敏反应引发。其他诱因包括上呼吸道感染、乙型或丙型肝炎等病毒以及 HIV。没有单一的测试来诊断 CSVV，但皮肤活检通常是有帮助的，它显示皮肤小血管的炎症证实了 CSVV 的诊断 [2-5]。

Reference

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  2. Mullick FG, McAllister HA Jr, Wagner BM, Fenoglio JJ Jr. Drug related vasculitis. Clinicopathologic correlations in 30 patients. Hum Pathol 1979; 10:313.
  3. Martinez-Taboada VM, Blanco R, Garcia-Fuentes M, Rodriguez-Valverde V. Clinical features and outcome of 95 patients with hypersensitivity vasculitis. Am J Med 1997; 102:186.
  4. Sais G, Vidaller A, Jucglà A, et al. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol 1995; 131:1399.
  5. Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore) 1998; 77:403.