Sellier S, Levesque H, Courville P, Joly P
Clinique Dermatologique, CHU de Rouen, Rouen.
Ann Dermatol Venereol. 2006 May;133(5 Pt 1):459-62. doi: 10.1016/s0151-9638(06)70940-1.
Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy is often poorly specific for maculopapular eruptions. We report an atypical case of neutrophilic disorder associating subcorneal pustules and hypodermal nodules in a male patient with angioimmunoblastic lymphadenopathy.
A 62 year-old man with angioimmunoblastic lymphadenopathy was treated with oral prednisone (15 mg/day) and cyclophosphamide. Pustules and deep nodules occurred simultaneously on the arms and trunk, the abdomen and the back respectively. Biopsy of these lesions showed an infiltration of neutrophil polymorphonuclears in the median and superficial dermis with a subcorneal pustule and nodular infiltration of neutrophil polymorphonuclears in the deep dermis and hypodermis respectively. Increasing the dose of prednisone to 30 mg/day led to rapid and complete healing of the cutaneous lesions.
This atypical case of neutrophilic disorder associating acute subcorneal pustules and deep dermal-hypodermal nodules seems to correspond to a unique type of neutrophilic disorder that differs from those previously reported in the literature. Neutrophilic disorders have been reported only rarely in patients with angioimmunoblastic lymphadenopathy. This case illustrates the wide range of clinical and histological lesions found in neutrophilic disorders, a number of which may coexist in a single patient.
血管免疫母细胞性淋巴结病患者的皮肤受累通常对斑丘疹表现缺乏特异性。我们报告一例血管免疫母细胞性淋巴结病男性患者，其出现了一种非典型的中性粒细胞疾病，伴有角层下脓疱和皮下结节。
一名患有血管免疫母细胞性淋巴结病的62岁男性患者接受了口服泼尼松（15毫克/天）和环磷酰胺治疗。脓疱和深部结节分别同时出现在双臂和躯干、腹部和背部。这些病变的活检显示，中性多形核粒细胞在真皮中层和浅层浸润，伴有角层下脓疱，而在真皮深层和皮下组织分别有中性多形核粒细胞的结节状浸润。将泼尼松剂量增加至30毫克/天导致皮肤病变迅速完全愈合。
这种伴有急性角层下脓疱和真皮深层 - 皮下结节的非典型中性粒细胞疾病似乎对应于一种独特类型的中性粒细胞疾病，不同于先前文献中报道的那些。血管免疫母细胞性淋巴结病患者中中性粒细胞疾病的报道很少。该病例说明了中性粒细胞疾病中发现的广泛的临床和组织学病变，其中一些可能在单个患者中共存。
