Loche F, Raynal H, Bazex J
Department of Dermatology, Purpan Hospital, Place du Dr Baylac, 31059 Toulouse, France.
Eur J Dermatol. 1998 Jan-Feb;8(1):63-5.
We report a case of pseudoxanthoma elasticum associated with an acneiform eruption involving the cervical area. When she was 16 years-old our patient was diagnosed with pseudoxanthoma elasticum, affecting the skin (flexural, cervical and neck areas) and the eyes (bilateral ocular angioid streaks). Ten years later, acneiform lesions (inflammatory and comedones) developed on these lesions: - the inflammatory lesions were characterized by phagocytosis of pathological elastic fibers inducing granuloma - the histological aspects of pseudoxanthoma elasticum were observed around large comedones. This association is rarely reported and we think that atypical, cervical acneiform lesions may be an indication of pseudoxanthoma elasticum. The mechanism for this association is not clearly understood. In our case, Von-Kossa staining was negative for the granulomatous lesions and positive for the comedones: calcification could protect elastopathic fibers from phagocytosis. Treatment is difficult: anti-acneic treatments are not effective except for tetracylines, the anti-inflammatory effects of which can improve granulomatous lesions. As its efficacy has been reported in elastosis perforans serpiginosa, we used liquid nitrogen cryotherapy on the inflammatory lesions with good results (separation of epidermis from dermis perhaps promoting transepithelial elimination of the abnormal dermal elastic tissue).
我们报告一例弹性假黄瘤合并颈部痤疮样皮疹的病例。患者16岁时被诊断为弹性假黄瘤，累及皮肤（屈侧、颈部和项部区域）及眼睛（双侧眼血管样条纹）。十年后，这些部位出现了痤疮样损害（炎性丘疹和粉刺）：炎性损害的特征为病理弹性纤维被吞噬，形成肉芽肿；在大粉刺周围可见弹性假黄瘤的组织学表现。这种关联鲜有报道，我们认为非典型的颈部痤疮样损害可能是弹性假黄瘤的一个指征。这种关联的机制尚不清楚。在我们的病例中，冯科萨染色显示肉芽肿性损害为阴性，粉刺为阳性：钙化可能保护弹性病变纤维不被吞噬。治疗困难：除四环素外，抗痤疮治疗无效，四环素的抗炎作用可改善肉芽肿性损害。鉴于液氮冷冻疗法在匐行性穿通性弹力纤维病中的疗效已有报道，我们对炎性损害采用液氮冷冻治疗，效果良好（表皮与真皮分离可能促进异常真皮弹性组织的经上皮清除）。
