Pseudoxanthoma elasticum-like papillary dermal elastolysis

Abstract

Letters 897An Bras Dermatol. 2017;92(6):891-900.According to the literature, hospitalization is required in some cases, which did not happen in our case because of the early intervention (Table 2). 5 Considering a reduced risk of agranulocytosis development and in accordance with Carneiro et al. (2011), 5 our aim was not to question DDS therapy for leprosy, but to stimulate clinical awareness of its risks by showing non-specific symptoms of agranulocytosis. We also highlight the need for laboratory test monitoring patients treated with DDS in order to favor the early treatment of this adverse effect, thus enhancing patient prognosis. q On dermoscopy, there were multiple normochromic papules and linear vessels (Figure 2). REFERENCESConsidering the diagnosis of pseudoxanthoma elasticum, the patient was referred for ophthalmologic evaluation, when no typical angioid streaks were seen. A biopsy was taken from the cervical region, and histopathology with Verhoeff stain revealed loss of elastic fibers in the papillary dermis (Figure 3).Described in 1992 by Rongioletti and Rebora, PXE-PDE is an acquired, rare disease that affects exclusively elderly women, usually after the fifth decade of life. 1Etiology is unknown, but is believed to be due to aging, exposure to ultraviolet radiation and abnormal elastogenesis. On dermoscopy, besides non-follicular papules, linear and arborizing vessels were described, corresponding to telangiectases seen in the papillary dermis on histology. 4,5 The differential diagnosis should be made with pseudoxanthoma elasticum, white fibrous papulosis of the neck and mid-dermal elastolysis. 2 Pseudoxanthoma elasticum is an inherited disorder characterized by calcification and fragmentation of elastic fibers that affects the skin, retina (with the appearance of angioid streaks) and cardiovascular system. Unlike pseudoxanthoma elasticum, PXE-PDE has no systemic manifestations.

Pseudoxanthoma elasticum-like papillary dermal elastolysis in an elderly male

“…In addition, elastic fibers in the mid-dermis were shortened. Kossa stain did not show calcification [2]. Fragmentation of elastic fibers is usually absent, whereas rarely observed in PXE-PDE, suggesting that elastolytic change can be seen not only in the papillary dermis but also in the subpapillary dermis.…”

Case Report: Pseudoxanthoma elasticum

“…At an early stage, the skin lesions are found on the right, left and rear sides of the neck and flexion creases, including armpits, inguinal region, popliteus region, and periumbilical area, as seen in this patient. The lesions may also affect the genital area and oral mucosa 2,4,5 .…”