Atmatzidis Douglas H, Hoegler Karl, Weiss Amy, Lambert W Clark, Schwartz Robert A
Department of Dermatology, Rutgers University - New Jersey Medical School, Newark, NJ.
Department of Pathology, Rutgers University - New Jersey Medical School, Newark, NJ.
Skinmed. 2019 Sep 9;17(3):161-170. eCollection 2019.
Cutaneous deposition disorders represent an array of conditions resulting from the accumulation of endogenous and exogenous substances within the skin. Many of the deposition diseases resemble each other and can also be confused with disorders not related to deposition. Porphyria cutanea tarda (PCT) results from dysfunction particularly in the fifth enzyme of the heme synthesis pathway, leading to increased skin fragility and bullae among other abnormalities. Ochronosis develops from alkaptonuria or exogenous sources, creating deposition of ocher-colored pigment in the skin. Hemochromatosis is a systemic disorder that can be inherited or acquired, altering skin pigmentation in more than 90% of patients. PCT can be an initial manifestation of hemochromatosis. Argyria is an acquired disorder of silver deposition that can also cause pigmentation similar to ochronosis. These uncommon but not rare disorders may resemble and be confused with each other in multiple ways.
皮肤沉积性疾病是指因内源性和外源性物质在皮肤内蓄积而导致的一系列病症。许多沉积性疾病彼此相似，也可能与非沉积性疾病相混淆。迟发性皮肤卟啉症（PCT）是由于血红素合成途径中第五种酶功能异常所致，会导致皮肤脆性增加和大疱等异常情况。褐黄病由尿黑酸尿症或外源性因素引起，会在皮肤中形成赭色色素沉积。血色素沉着症是一种可遗传或后天获得的全身性疾病，超过90%的患者会出现皮肤色素沉着改变。PCT可能是血色素沉着症的初始表现。银质沉着症是一种后天性银沉积疾病，也可能导致类似于褐黄病的色素沉着。这些不常见但并非罕见的疾病可能在多种方面彼此相似且容易混淆。
