Good Allison J, Snodgrass Alexandra L, De Benedetto Anna, Motaparthi Kiran
Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida.
J Cutan Pathol. 2019 Jan;46(1):74-79. doi: 10.1111/cup.13368. Epub 2018 Nov 5.
Collagenous and elastotic marginal plaques of the hand (CEMPH) is a rare, chronic keratoderma characterized by hyperkeratotic linear plaques located along the radial and ulnar aspects of the hands bilaterally. As an isolated finding, CEMPH occurs secondarily to chronic trauma and photodamage. Herein, CEMPH is described as a manifestation of alkaptonuria (AKU). In addition to keloidal collagen, ochronotic fibers and fragmented, thickened elastic fibers were observed. Additionally, mucin deposition-not previously described in this clinical context-was also identified. Given their overlapping clinicopathologic features, CEMPH due to AKU should be distinguished from the acquired variant as well as acrokeratoelastoidosis.
手部胶原性和弹性组织变性边缘性斑块（CEMPH）是一种罕见的慢性角化病，其特征为双侧手部桡侧和尺侧出现角化过度的线性斑块。作为一种孤立的表现，CEMPH继发于慢性创伤和光损伤。在此，CEMPH被描述为黑尿症（AKU）的一种表现。除了瘢痕疙瘩样胶原外，还观察到褐黄病性纤维和破碎、增粗的弹性纤维。此外，还发现了黏液沉积——在这种临床情况下此前未被描述过。鉴于其重叠的临床病理特征，由AKU引起的CEMPH应与后天性变种以及肢端角化性弹性组织变性相鉴别。
