Background

Skin involvement in granulomatosis with polyangiitis (GPA) is common and can appear as an initial presentation of the disease or more commonly through its course.

Case presentation

We report a case of a 24-year-old male patient, previously diagnosed as having GPA, admitted with fever, hemoptysis, generalized hemorrhagic blisters associated with arthralgia, fatigue, myalgia, nasal crusting, and vertigo. Three weeks prior to admission, he developed erythematous papules on both elbows, and purpuric papules on both lower limbs. Histopathological examination revealed: interstitial granulomatous dermatitis (elbows) and foci of dermal hemorrhage, foci of interstitial histiocytes and zones of altered necrobiotic collagen (lower limbs) consistent with cutaneous lesions of GPA. Two weeks later, his rash progressed to widespread purpura associated with hemorrhagic blisters. Another biopsy revealed leukocytoclastic vasculitis with fibrinoid necrosis of the vessel walls associated with perivascular infiltrate of neutrophils, nuclear dust and extravasated erythrocytes without an associated granulomatous inflammation or necrobiosis. The constellation of the results of the three biopsies together with clinical correlation pointed to a flare of GPA.

Conclusion

Skin involvement in GPA is quite common, and it can manifest in different forms in the same patient. Our patient developed three different skin pathologies within a short period of time.

中文翻译：

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肉芽肿性多血管炎皮肤受累的三种模式。

背景

肉芽肿性多血管炎 (GPA) 中的皮肤受累很常见，并且可以作为疾病的初始表现出现，或者更常见于病程。

案例展示

我们报告一例 24 岁男性患者，之前被诊断为 GPA，因发热、咯血、全身性出血性水疱伴关节痛、疲劳、肌痛、鼻结痂和眩晕入院。入院前三周，患者双肘出现红斑丘疹，双下肢出现紫癜性丘疹。组织病理学检查显示：间质性肉芽肿性皮炎（肘部）和真皮出血病灶、间质组织细胞病灶和坏死胶原蛋白改变区（下肢）与 GPA 皮损一致。两周后，他的皮疹发展为与出血性水疱相关的广泛紫癜。另一项活检显示白细胞破碎性血管炎伴血管壁纤维素样坏死，伴血管周围中性粒细胞浸润，核尘和外渗的红细胞没有相关的肉芽肿性炎症或坏死。三个活检结果的组合以及临床相关性表明 GPA 突然发作。

结论

GPA 中的皮肤受累很常见，并且可以在同一患者身上以不同的形式表现出来。我们的患者在短时间内出现了三种不同的皮肤病变。