Nofal A, Assaf M, Nofal E, Alradi M
Department of Dermatology, Faculty of Medicine, Zagazig University, Zagazig, Egypt.
J Eur Acad Dermatol Venereol. 2014 Apr;28(4):397-404. doi: 10.1111/jdv.12226. Epub 2013 Aug 6.
Generalized eruptive keratoacanthoma (GEKA) of Grzybowski is a sporadically occurring, extremely rare variant of keratoacanthoma characterized clinically by severely pruritic, generalized eruption of numerous small follicular papules, often with a central keratotic plug, and histologically by typical features of solitary keratoacanthoma. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is not yet determined. Herein, we review the different aspects of this rare entity, including pathogenesis, clinical and histopathological features, differential diagnosis, course and prognosis. Different therapeutic approaches and their impact on the course and prognosis of the disease are also evaluated and presented. We propose two sets of diagnostic criteria to define the disease more precisely and to avoid overlapping and confusion with other types of multiple keratoacanthoma. The first set comprises constant clinical and histopathological features that almost always present in every case and the second set includes variable features that were reported in some patients, and to which any emerging finding could be added to avoid missing cases. Although different therapeutic options have been used, either as single agents or in combinations, there is no standard therapy for GEKA and the disease still represents a therapeutic challenge.
格日博夫斯基泛发性疹性角化棘皮瘤（GEKA）是一种散发性出现的、极为罕见的角化棘皮瘤变体，临床特征为大量小毛囊丘疹严重瘙痒、泛发，常伴有中央角质栓，组织学表现为孤立性角化棘皮瘤的典型特征。尽管新病例报告不断增加，但尚未确立明确的诊断标准，最佳治疗方法也尚未确定。在此，我们回顾了这一罕见疾病的不同方面，包括发病机制、临床和组织病理学特征、鉴别诊断、病程及预后。还评估并介绍了不同的治疗方法及其对疾病病程和预后的影响。我们提出了两组诊断标准，以更精确地定义该疾病，并避免与其他类型的多发性角化棘皮瘤重叠和混淆。第一组包括几乎在每个病例中都始终存在的恒定临床和组织病理学特征，第二组包括一些患者中报告的可变特征，任何新出现的发现都可添加到其中以避免漏诊。尽管已使用了不同的治疗选择，无论是单一药物还是联合使用，但GEKA尚无标准治疗方法，该疾病仍然是一个治疗挑战。
