Schaller Jörg, Carlson J Andrew
Department of Dermatohistology, Catholic Clinics, Duisburg, Germany.
J Am Acad Dermatol. 2009 Feb;60(2):277-88. doi: 10.1016/j.jaad.2008.09.024.
Treatment of systemic infections due to mycobacteria and HIV infection can lead to paradoxical worsening, the immune reconstitution inflammatory syndrome, in a minority of patients. Herein we describe a patient with Whipple's disease, a chronic systemic inflammatory disease caused by Tropheryma whipplei, who developed cutaneous and later ocular disease after initiation of antibiotic therapy. A 42-year-old man with a 12-year history of arthralgias presented with deteriorating health, including weight loss, diarrhea, fever, and acral hyperkeratosis. Whipple's disease was suspected and subsequently confirmed by finding periodic acid-Schiff (PAS)-positive foamy macrophages and T whipplei DNA by polymerase chain reaction (PCR) assays in duodenal biopsy specimens. After 5 weeks of antibiotic treatment with ceftriaxone, erythema nodosum (EN)-like lesions developed on the legs and trunk. Notably, lesional and nonlesional skin harbored intracellular and extracellular degenerated bacteria that were associated with a neutrophilic and granulomatous inflammatory response in lesional skin. Continued antibiotic therapy was associated with recurring EN-like skin nodules, orbital swelling, and facial herpes simplex virus 1 infection. Corticosteroid therapy controlled the duration and severity of the EN-like nodules and orbital swelling. Apart from cutaneous hyperpigmentation, skin disease in Whipple's disease is infrequent and can be categorized as disorders due to malnutrition from malabsorption or so-called reversal reactions consisting of reactive erythemas, and neutrophilic and granulomatous responses to T whipplei, the latter of which can represent an immune reconstitution inflammatory reaction after initiation of antibiotic therapy. Finally, based on the presence of T whipplei in normal skin, skin biopsy may serve as another site for diagnostic testing in patients suspected of having Whipple's disease.
分枝杆菌和HIV感染所致的全身感染治疗可能会在少数患者中导致矛盾性恶化，即免疫重建炎症综合征。在此，我们描述一名患有惠普尔病的患者，惠普尔病是一种由惠普尔嗜组织菌引起的慢性全身性炎症性疾病，该患者在开始抗生素治疗后出现皮肤病变，随后出现眼部疾病。一名有12年关节痛病史的42岁男性，健康状况恶化，包括体重减轻、腹泻、发热和肢端角化过度。怀疑为惠普尔病，随后通过在十二指肠活检标本中发现过碘酸希夫（PAS）阳性的泡沫状巨噬细胞以及通过聚合酶链反应（PCR）检测到惠普尔嗜组织菌DNA得以确诊。在用头孢曲松进行5周抗生素治疗后，患者双下肢和躯干出现结节性红斑（EN）样病变。值得注意的是，病变皮肤和非病变皮肤中都存在细胞内和细胞外变性细菌，这些细菌与病变皮肤中的中性粒细胞和肉芽肿性炎症反应有关。持续的抗生素治疗与复发性EN样皮肤结节、眼眶肿胀和面部单纯疱疹病毒1感染有关。皮质类固醇治疗控制了EN样结节和眼眶肿胀的持续时间和严重程度。除皮肤色素沉着外，惠普尔病中的皮肤疾病并不常见，可归类为因吸收不良导致营养不良引起的疾病，或所谓的逆转反应，包括反应性红斑以及对惠普尔嗜组织菌的中性粒细胞和肉芽肿性反应，后者可能代表抗生素治疗开始后的免疫重建炎症反应。最后，基于正常皮肤中存在惠普尔嗜组织菌，皮肤活检可作为疑似患有惠普尔病患者的另一个诊断检测部位。
