A Case of Fibroelastolytic Papulosis on the Neck of a Young Man

Fibroelastolytic papulosis of the neck (FEPN) encompasses a spectrum of two disorders that were previously reported as pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). The clinical presentation of FEPN is asymptomatic to mildly pruritic whitish-yellow papules that may coalesce into cobblestone patterned plaques that resemble pseudoxanthoma elasticum (PXE). The histology is characterized by a decrease or loss of elastic fibers in the papillary dermis and this is sometimes accompanied by a minimal or nodular increase of dermal collagen fibers. We report here on a 28-year-old Korean man with asymptomatic, multiple, skin-colored to slightly yellowish, match-head sized, cobblestone-patterned papules on the neck, and these were histologically consistent with FEPN and the papules showed slightly increased dermal collagen associated with decreased and fragmented elastic fibers, elastin and tropoelastin. The pathogenesis of FEPN in this case might have been related with mild dermal inflammation, followed by fragmentation, elastolysis and increased dermal collagen.     

Pseudoxanthoma elasticum-like papillary dermal elastolysis

Elastolysis refers to a loss of elastic fibers. It can affect the papillary dermis, the reticular dermis, or both. Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a clinicopathological entity first described by Rongioletti and Rebora in 1992 (1). It is an acquired elastolytic disorder that mainly affects the papillary dermis. Clinically it resembles pseudoxanthoma elasticum (PXE), but it differs histologically and there is no systemic involvement, in contrast to PXE.     

Pseudoxanthoma elasticum-like papillary dermal elastolysis developed in early middle age

Pseudoxanthoma elasticum-like papillary dermal elastolysis is a rare acquired elastolytic disorder characterized by papules that resemble pseudoxanthoma elasticum, and it typically affects elderly women. Histopathological examination shows atrophic epidermis and band-like loss of elastic tissue in the papillary dermis. The pathogenesis is assumed to be related to intrinsic aging because it affects elderly people and shows the loss of elastic tissue. We report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis in early middle age presenting typical clinical and histopathological findings. The patient was a 41-year-old woman who had had her lesions for 10 years. We propose that younger patients, hitherto unknown, can be affected by this disorder and suggest that mechanisms other than intrinsic aging are involved in its pathogenesis.     

Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum‐like papillary dermal elastolysis?

There are numerous acquired elastic tissue disorders, several of which present cutaneously with small yellow-to-white papules resembling plucked chicken skin. Differential diagnoses depend on the abnormalities within the network of elastic tissues. We report a case with distinct histologic features, which may represent a unique elastic tissue disorder or a variant of pseudoxanthoma elasticum-like papillary dermal elastolysis. Our patient's clinical presentation includes scattered 1–2 mm white-to-yellow papules without surface change on the upper back and neck region. Histology is characterized by foci of clumped, granular elastic tissue, which have replaced the oxytalan and elaunin fibers, alternating with foci of decreased concentrations of normal-appearing elastic fibers within the papillary dermis. Given its characteristics, we have termed this novel entity 'papillary dermal elastosis'.     

Acquired idiopathic cutaneous elastolysis of the papillary and mid-dermis: distinct but related conditions. Report of two cases

Among the acquired idiopathic cutaneous elastolytic disorders, pseudoxanthoma elasticum-like papillary dermal elastolysis (PDE) and mid-dermal elastolysis (MDE) are conditions not frequently reported in the literature.
We report 2 cases: the first an older woman with typical papular and plaque-like lesions on both sides of her neck and shoulders and the second a 42-year-old woman with fine wrinkles scattered all over the Hunk, shoulders and abdomen. Histological examination of the first case revealed loss of the elastic fibers limited to the papillary dermis, instead in the second case the loss was observed in the mid-dermis.
We discuss the possibility that PDE and some cases of mm-inflammatory MDE may represent distinct but related entities in a "spectrum" of acquired elastolysis and that they may be an expression of intrinsic aging.     

弹性假黄瘤样真皮乳头层弹性纤维溶解症国内首例报告

报告国内首次发现的弹性假黄瘤样真皮乳头层弹性纤维溶解症1例。患者女,38岁。因多发性肌炎10月余,颈部起黄色斑丘疹2月就诊。双侧颈部、锁骨上方有数十个绿豆至黄豆大淡黄色柔软丘疹或斑块,呈鹅卵石样。临床及实验室检查符合多发性肌炎,无眼及心血管改变。组织病理检查以韦杰尔特染液染色,可见真皮乳头层弹力纤维完全消失,真皮网状层内弹性组织轻度减少,von-kossa染色未见钙化。透射电镜真皮乳头层内弹力纤维消失,在真皮网状层上部可见大量未成熟的弹力纤维,胶原纤维结构正常。     

Pseudoxanthoma elasticum-like papillary dermal elastolysis: A report of two cases

We report 2 women, aged 83 and 63 years, who presented with multiple asymptomatic, slowly progressive, coalescing, skin-colored papules affecting the sides of the neck and lower abdomen. Incisional biopsies obtained from both patients revealed elastolysis in the papillary dermis. These 2 cases represent pseudoxanthoma elasticum-like papillary dermal elastolysis, a recently described entity, the etiology of which remains unclear.     

Papillary dermal elastolysis similar to pseudoxanthoma elasticum

Papillary dermal elastolysis similar to pseudoxanthoma elasticum is an elastolytic disorder characterized by cutaneous lesions on the neck and in the supraclavicular region that are clinically similar to pseudoxanthoma elasticum, with no systemic complications. The histological examination shows a loss of elastic fibers in the papillary dermis. We report a case in a 76-year-old woman with typical lesions on the neck.     

Mid-dermal elastolysis revisited

The clinical as well as histological data of 79 mid-dermal elastolysis (MDE) patients reported in the literature were evaluated. MDE is an acquired skin condition of the elastic tissue predominantly manifesting on the trunk and proximal extremities of young women. Most commonly observed skin changes include patches of well-circumscribed fine wrinkles (type I) and perifollicular papular protrusions (type II). Rarely, MDE may also occur with persistent reticular erythema and wrinkling (type III). The critical diagnostic histopathological feature of MDE is the selective loss of elastic fibres in the mid-dermis. Mild lymphohistiocytic infiltrates, elastophagocytosis of elastic fibres by macrophages, and even multinucleate giant cells are occasionally observed in MDE lesions. Immunohistological studies and cell culture experiments indicate that dysbalances in elastin turnover are associated with pathological degradative processes including increased elastolytic activity that finally lead to loss of elastic fibres in the mid-dermis. First-line differential diagnoses may include closely related conditions such as anetoderma, annular elastolytic giant cell granuloma, cutis laxa acquisita and pseudoxanthoma elasticum-like papillary dermal elastolysis. Future therapeutic approaches in MDE patients should focus on agents that are able to block increased elastase activity and induce elastin synthesis.     

Fibroelastolytic papulosis: histopathologic confirmation of disease spectrum variants in a single case

Fibroelastolytic papulosis is a rare, acquired fibroelastolytic disorder that presents clinically as white‐to‐yellow papules and plaques most commonly occurring on the neck of elderly patients. The term fibroelastolytic papulosis encompasses two closely related conditions previously described as pseudoxanthoma elasticum‐like papillary dermal elastolysis (PDE) and white fibrous papulosis of the neck (WFPN). Here we present a case of a 78‐year‐old white female with a several‐year history of numerous, asymptomatic 2–3 mm yellowish, non‐follicular papules distributed symmetrically over the posterior neck, axillae, arm and antecubital fossae. Histopathologic examination revealed thickened and clumped elastotic fibers admixed with thick, sclerotic appearing collagen bundles in the mid and deep reticular dermis. Rare melanophages, loss of vertically oriented elastic fibers and scattered elastotic globes were noted in the papillary dermis. Based on the shared clinicopathologic features showed in this case, strong consideration should be made for the additional inclusion of papillary dermal elastosis as existing along the disease continuum of fibroelastolytic papulosis. This occurrence of fibroelastolytic papulosis shows unique histopathologic findings of pseudoxanthoma elasticum‐like PDE, papillary dermal elastosis and WFPN, further supporting the theory that these entities exist as variants along the fibroelastolytic papulosis spectrum.     

White fibrous papulosis of the neck in three Sicilian patients

Three patients, two females and one male, presented with white fibrous papulosis of the neck. This condition is characteristically located on both sides of the neck; however, it also appears on the upper sternal region, with a necklace-like configuration, in the two female patients, aged 72 and 78 years, respectively. A differential diagnosis was carried out with respect to other dermatoses that show a similar skin aspect, and to the pseudoxanthoma elasticum-like papillary dermal elastolysis. This is because this condition, as well as fibrous papulosis, can be interpreted as a clinical-histological variant of the same process of cutaneous ageing. However, environmental factors can play a role in the aetiopathogenesis of fibrous papulosis in the Sicilian population.     

Upper dermal elastolysis: A comparative study with mid-dermal elastolysis

We encountered a patient who complained of many small papules on the neck, shoulders, upper chest and upper back. Biopsy specimens showed complete loss of elastic fibers in the upper dermis including papillary dermis, whereas those of the mid dermis were intact. Electron microscopy revealed that assembly of component fibrils of elastic fibers was loose, and electron dense substance was aggregated in the spaces between these loosely bound subunit Fibrils or along the periphery of abnormal fibers. Dermal phagocytes engulfed abnormal as well as normal elastic fibers. Upper dermal elastolysis is a clinical and histopathological entity different from mid-dermal elastolysis. Ultrastructural changes of the former are essentially similar to those of the latter but much more severe. It is suggested that activated elastophagocytosis of dermal phagocytes may play a role in this disease.
Hashimoto K, Tye MJ. Upper dermal elastolysis: A comparative study with mid-dermal elastolysis.     

Acquired disorders of elastic tissue: Part II. decreased elastic tissue

Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood owing to the paucity of reported cases. Several acquired disorders in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis. Learning objective At the conclusion of this learning activity, participants should have an understanding of the similarities and differences between acquired disorders of elastic tissue that are characterized by a loss of elastic tissue.     

Pseudoxanthoma elasticum-like papillary dermal elastolysis: immunohistochemical study using elastic fiber cross-reactivity with an antibody against amyloid p component

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis is a rare disorder clinically characterized by multiple, asymptomatic nonfollicular papules, yellow or flesh colored, grouped to form plaques with cobblestone appearance. These plaques are arranged symmetrically on the neck and supraclavicular areas of adult or elderly women, closely resembling the lesions of PXE. Histopathologically, the lesions are characterized by a band-like area of elastolysis along the papillary dermis. We report 2 cases of PXE-like papillary dermal elastolysis, in which the anomalies of the elastic tissue were immunohistochemically investigated with an antibody to the amyloid P component. This immunostaining demonstrated a band-like loss of elastic tissue along the papillary dermis. No clumping, fragmentation, or calcification of the elastic tissue was seen. We also review the literature about this rare process and discuss the differential diagnosis with other elastic tissue disorders that may show similar clinical and/or histopathologic findings.     

Papulosis fibroelastolítica del cuello. ¿Es la elastólisis de la dermis papilar un hallazgo histopatológico característico?

—Two patients, a 69-year-old and a 70-year-old women, presented multiple symmetrical, confluent, whiteyellowish papules on the lateral and dorsal aspects of the neck. The lesions were symptomless and had appeared several years before consultation. Histopathological features disclosed a reduced amount or a complete loss of elastic fibers in the papillary dermis in both involved and non-involved perilesional skin and a non-constant thickening of collagen fibers in the papillary and reticular dermis in involved skin.Morphological similarities and a possible relationship between reports of white fibrous papulosis of the neck (WFPN) and pseudoxanthoma-elaticum-like papillary dermal elastolysis (PEL-PDE) are discussed. Both entities share similar clinical features and under both concepts identical lesions have been reported. The observation of papillary dermal elastolysis in both non-involved and involved skin seems to suggest that this finding should not be considered a diagnostic characteristic feature.Probably both entities should be considered as a unified disorder (fibroelastolytic papulosis of the neck). These entities probably correspond, as has been previously proposed, to morphological variants that reflect cutaneous intrinsic aging (fibroelastolytic patterns of intrinsic skin aging).     

Elastolysis in lichen ruber planus.

The dermal elastic fiber network was studied in specimens from five patients with lichen ruber planus, using a standard elastin staining procedure (orcein), results being compared with those for the elastin-associated microfibrillar network stained using anti-fibrillin antibodies in an immunofluorescence and an avidin-biotin-peroxidase complex technique. Additional specimens of healed apparently normal skin were taken from two of the patients. Orcein-stained fibers were scarce or absent in the inflammatory zone in all the lesions. In contrast, an extensive fibrillin immunoreactive network was present in the papillary zone in all the specimens, in a pattern similar to that of normal skin. In specimens from healed lesions of lichen ruber planus, dermal orcein-stained fibers were present in the papillary dermis. The findings indicate that the amorphous component of elastic fibers is destroyed during the acute phase of lichen ruber planus. Hypothetically, the elastolysis is caused by elastases released from macrophages known to be present in the lichenoid infiltrate. In contrast, the fibrillin fiber network seems to be less or not at all affected by proteolytic events during the inflammatory phase of lichen ruber planus.     

Pseudoxantoma elasticum-like dermal elastolysis: a case report

Elastic fibers are components of dermal connective tissue that can be affected in several acquired disorders. Recently, a new entity known as pseudoxanthoma-like papillary dermal elastolysis has been described. We present a case in a 61-year-old woman.     

Mid Dermal Elastolysis: Case Report and Review of the Literature

A new case of typical mid dermal elastolysis was studied with electron microscopy. Elastic fibers were irregularly branched or lumpy, but subcomponents of these fibers were often normal. In some fibers, loose assembly of skeleton fibrils and aggregation of dense substances were observed. Phagocytosis of normal and abnormal elastic fibers by dermal macrophages was observed.