Papillary Dermal Elastolysis Research Articles

Bumps on the neck? Pseudoxanthoma elasticum-like papillary dermal elastolysis

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is an acquired disorder of dermal elastic tissue. Unlike pseudoxanthoma elasticum which it mimics, PXE-PDE has no systemic involvement. PXE-PDE typically affects postmenopausal women and is characterised clinically by tan to yellow coloured papules on the neck, supraclavicular regions, axilla, and flexural surfaces that often coalesce into cobblestone plaques. The prevalence is thought to be underestimated as it is a relatively asymptomatic condition of minor aesthetic concern.

34806 Pseudoxanthoma elasticum-like papillary dermal elastolysis: A histopathologic diagnosis

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare acquired dermatologic disorder of the elastic tissue that mimics genetic pseudoxanthoma elasticum (PXE). PXE-PDE is characterized clinically by flesh-colored to yellow papules that often coalesce into cobblestone plaques on flexural sites predominately in postmenopausal women. PXE-PDE can be distinguished from true PXE on a histopathologic basis only, although the clinical presentation of PXE-like lesions without associated systemic complications makes a diagnosis of PXE-PDE more likely. The pathogenesis of PXE-PDE is unclear and has been suggested to be multifactorial, involving intrinsic, extrinsic, and genetic factors. Treatment of this disease has proven to be difficult, with various treatments having less than adequate outcomes. To our knowledge, there have been around 60 cases of PXE-PDE reported in the literature. Here, we present a case of pruritic PXE-PDE of the neck and antecubital fossae with intent to raise awareness of the most common clinical and histopathologic features.

28144 Cobblestoned skin on neck: The differential

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-like PDE) is a benign cutaneous condition that mimics pseudoxanthoma elasticum (PXE). PXE is a hereditary distortion and calcification of elastic fibers, not only in cutaneous tissue, but in cardiac and ocular tissues. Histopathology is an important diagnostic tool to distinguish between the two and can save the patient from an extensive work-up if misdiagnosed as PXE. We present, herein, a 62-year-old healthy female of with an asymptomatic papular rash on her neck for 8 months. She wears a hijab, and reports frequent heat and sweat. The patient failed topical steroid creams given by her primary care provider. Exam showed multiple skin-colored to yellow smooth papules on the bilateral and posterior neck, and bilateral antecubital fossae. Biopsy from her left neck showed abrupt loss of elastic fibers in the papillary dermis with a Verhoeff-van Gieson stain. A von Kossa stain did not demonstrate calcium deposition, ruling out PXE. PXE-LPDE is likely an underdiagnosed acquired condition that occurs most commonly in women after the sixth decade of life and has overlapping clinical and histopathologic features with white fibrous papulosis, another benign condition that can be argued is the same entity. We favored PXE-LPDE in our patient due to the band-like loss of elastic fibers in the papillary dermis. Although chronic sun exposure has been implicated in its pathogenesis, our patient has been sun protected for most of her life. Unfortunately, no specific therapy has been shown to be successful in the treatment of PXE-like PDE.

Pseudoxanthoma elasticum-like papillary dermal elastolysis in an elderly male

Pseudoxanthoma elasticum-like papillary dermal elastolysis in an elderly male

Pseudoxanthoma elasticum–like papillary dermal elastolysis: A mimicker of genetic pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE)–like papillary dermal elastolysis (PDE) is an acquired, rare, elastic tissue disorder that presents with multiple asymptomatic, nonfollicular yellowish or normochromic papules, coalescing into symmetrical cobblestone plaques, with a predilection for the neck of postmenopausal women. The condition develops slowly, in months to years, and, although clinically similar to PXE, it is devoid of any systemic implication, being only of esthetic concern. The etiology is unknown, but it is speculated that there is a multifactorial pathogenesis, with the contribution of intrinsic aging, exposure to ultraviolet radiation, abnormal elastogenesis, and genetic factors. The diagnosis is confirmed by histopathology, with special stains specific for elastic fibers revealing loss of elastic plexus in the papillary dermis and the presence of melanophages. Its prevalence is probably underestimated, which reinforces the importance of better clinical and histologic identification to distinguish it from inherited PXE and to avoid extensive systemic investigations and unnecessary medical visits for the patient.

Cutaneous Elastic Tissue Anomalies.

After a review of the physiology in the formation and degradation of cutaneous elastic tissue, we describe the clinicopathologic disorders characterized by increased and decreased cutaneous elastic tissue. Cutaneous disorders characterized by increased and/or abnormal elastic tissue in the dermis include elastoma, also named nevus elasticus, dermatosis lenticularis disseminata, pseudoxanthoma elasticum, late-onset focal dermal elastosis, linear focal elastosis, elastoderma, elastofibroma dorsi, and elastosis perforans serpiginosa. In some of these conditions, the specific histopathologic diagnosis may be rendered with hematoxylin-eosin stain, whereas in other ones special elastic tissue stains are necessary to demonstrate the anomalies. Cutaneous disorders characterized by decreased dermal elastic tissue include nevus anelasticus, papular elastorrhexis, perifollicular elastolysis, anetoderma cutis laxa, postinflammatory elastolysis and cutis laxa, white fibrous papulosis of the neck, pseudoxanthoma elasticum-like papillary dermal elastolysis, and mid dermal elastolysis. In most of these conditions, the histopathologic anomalies are only seen with elastic tissue stains, and cutaneous biopsies of these processes stained with hematoxylin-eosin show appearance of normal skin. The diagnosis of some of these disorders characterized by increased or decreased elastic dermal tissue should be followed by general exploration of the patient to rule out associated severe systemic anomalies, and in some cases, a genetic counseling should be offered to the family.

Papular elastorrhexis: clinical perspectives.

First described by Bordas in 1987, papular elastorrhexis (PE) is a rare elastic fiber disorder of the skin characterized by multiple, discrete, asymptomatic, firm, nonfollicular, monomorphous, 1–5 mm, circumscribed, hypopigmented, oval to round papules, symmetrically distributed on the chest, abdomen, back, shoulders, arms, and thighs. The onset of the condition is usually in the first or second decade of life. PE appears to be an exceedingly rare entity, with 33 cases reported in the literature until now. However, the disorder might be underestimated probably because of its subtlety, asymptomatic course, and benign nature of clinical alterations, which can easily be confused with other dermatoses such as acne scars. Clinical and histopathological differential diagnosis of PE is broad and includes papular acne scars, eruptive collagenoma, disseminated lenticular dermatofibrosis (as a component of Buschke–Ollendorff syndrome), white fibrous papulosis of the neck, pseudoxanthoma elasticum, pseudoxanthoma elasticum-like papillary dermal elastolysis, middermal elastolysis, and perifollicular elastolysis. Treatment of PE is a matter of debate and no reliable curative option exists.

The spectrum of fibroelastolytic papulosis: a retrospective case series.

Introduction: There is an overlap between clinical and pathological features of the entities known as pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). Although the term fibroelastolytic papulosis has been used to encompass both entities as spectrum variants of a one and only disease, many authors still differentiate the two.Methods: Cases of fibroelastolytic papulosis confirmed by histopathologic examination were retrospectively reviewed within a time frame of 2.5 years.Results: Five cases of fibroelastolytic papulosis were identified. All patients were females aged between 63 and 78 years, presenting with an asymptomatic eruption of isolated or coalescing white to yellow papules on the neck. Two of the patients also had involvement of other anatomical areas. In all cases histology demonstrated a significantly decreased or absent papillary dermal elastic plexus, a mild superficial perivascular lymphocytic infiltrate and sparse melanophages in the papillary dermis, without interface changes. A decrease in upper reticular dermal elastic fibers was noted in four cases and thickening of dermal collagen bundles in three cases. Dilated superficial dermal vessels were present in 3 cases and mild epidermal atrophy in one case.Conclusion: We find the differentiation of PXE-PDE and WFPN to be somewhat theoretical, often confusing, and based on non-significant subtle histological and clinical differences. In our opinion, we should not continue to use these terms, but favor the common term of fibroelastolytic papulosis.

Atrophying pityriasis versicolor as an idiosyncratic T cell–mediated response to Malassezia: A case series

Atrophying pityriasis versicolor (PV), first described in 1971, is a rare variant in which lesions appear atrophic. We sought to determine the pathophysiology of atrophying PV. A retrospective chart review identified 6 cases of atrophying PV. In all cases, routine light microscopy, an elastic tissue stain, and immunohistochemical assessment for the expression of CD3, CD4, CD8, GATA3 and CXCR3 was performed. All cases demonstrated hyperkeratosis with intracorneal infiltration by pathogenic hyphal forms as well as epidermal attenuation and papillary dermal elastolysis. A supervening, mild-to-moderate, superficial lymphocytic infiltrate was noted and characterized by a focal CD8+ Tcell-mediated interface dermatitis along with a mixed T-cell infiltrate composed of GATA3+ and CXCR3+ Tcells. Small sample size and the loss of some patients to follow-up. Atrophying PV represents the sequelae of a mixed helper T-cell (TH1 and TH2) idiosyncratic immune response to Malassezia and can present as a protracted dermatosis that may clinically mimic an atypical lymphocytic infiltrate. TH1 cytokines can recruit histiocytes, a source of elastases, and upregulate matrix metalloproteinase activity, which may contribute to epidermal atrophy.

A Case Report of Pseudoxanthoma Elasticum-like Papillary Dermal Elastolysis

Pseudoxanthoma elasticum-like papillary dermal elastolysis is a relatively rare disorder within the literature. Increased awareness of this entity will broaden the differential diagnoses among dermatologists encountering such a condition. Pseudoxanthoma elasticum-like papillary dermal elastolysis tends to occur predominantly in elderly females. Clinically, the lesions present as papules and cobblestone plaques on the neck, resembling pseudoxanthoma elasticum. Histologically, there is absence of elastic fibers in the papillary dermis, and no calcification of the remaining fibers. Pseudoxanthoma elasticum-like papillary dermal elastolysis differs from Pseudoxanthoma elasticum by the absence of systemic manifestations, hence patients are reassured and no further workup is warranted. As of now, no effective treatments are available for this entity. Pseudoxanthoma elasticum-like papillary dermal elastolysis should be in the differential diagnosis of a dermatologist when encountering a clinical presentation of Pseudoxanthoma elasticum in an elderly patient localized to the neck, with absence of fragmented and calcified elastic fibers on histologic examination.

Does clinical pseudoxanthoma-like papillary dermal elastolysis require typical elastolysis on histology? A case report and controversy

Does clinical pseudoxanthoma-like papillary dermal elastolysis require typical elastolysis on histology? A case report and controversy

Fibroelastolytic papulosis in an elderly woman with about 30-year history: Overlapping between pseudoxanthoma elasticum–like papillary dermal elastolysis and white fibrous papulosis of the neck

Fibroelastolytic papulosis in an elderly woman with about 30-year history: Overlapping between pseudoxanthoma elasticum–like papillary dermal elastolysis and white fibrous papulosis of the neck

ELASTÓLISE DA DERME PAPILAR SEMELHANTE A PSEUDOXANTOMA ELÁSTICO - UMA ENTIDADE SUBDIAGNOSTICADA

A elastólise da derme papilar semelhante a pseudoxantoma elástico (EDP-PXE) é uma entidade clinico- -patológica adquirida, de etiologia desconhecida, caracterizada por ausência parcial ou total de fibras elásticas na derme superficial. Reportamos o caso clínico de uma doente caucasiana de 62 anos, fototipo III, com uma dermatose localizada nas regiões posterior e laterais do pescoço caracterizada por múltiplas pápulas amareladas, monomórficas, arredondadas, com 2-3mm de diâmetro, assintomáticas, presentes desde há cerca de 2 anos. O exame histopatológico, com a coloração de orceína, revelou ausência quase total de fibras elásticas na derme superficial, compatível com o diagnóstico de EDP-PXE. Consideramos que esta entidade, exclusivamente cutânea, está sub-diagnosticada por se tratar de uma patologia assintomática, cujo diagnóstico histopatológico está dificultado, na ausência de corretas hipóteses de diagnóstico.

Role of Steroid Therapy inPseudoxanthoma Elasticum-like Papillary Dermal Elastolysis

Pseudoxanthoma Elasticum-like Papillary Dermal Elastolysis (PXE-PDE) is a peculiar idiopathic elastolytic disorder that clinically resembles Psudoxanthoma elasticum(PXE). It is histologically characterized by a total or partial loss of elastic fibers in the papillary dermis. It more often affects elderly women and is characterized by asymptomatic and symmetrical yellowish papules localized predominantly on the neck, supraclavicular regions, and flexural areas. After analyzing a series of cases and the recent literature suggesting that glucocorticoids may down-regulate the elastin gene expression and elastin mRNA, in cultured human skin fibroblasts, we think that high dose and prolonged steroid therapy may contribute to the appearance of PXE-PDE lesions.

Pseudoxanthoma Elasticum–Like Papillary Dermal Elastolysis

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.

Papillary dermal elastosis

We recently came across the article "Papillary dermal elastosis" by Newlove et al published in the October 2011 edition of Dermatology Online Journal. In this paper, the authors described what they interpreted to be the second case of papillary dermal elastosis, an entity we had first identified in 2009. Upon further reading of their histopathologic and clinical description, we believe this case is more consistent with the diagnosis of pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE).

Papillary Dermal Elastolysis with a Twist

Pseudoxanthoma elasticum–like papillary dermal elastolysis (PXE-like PDE) is a rare, acquired, complete absence of oxytalan and elaunin elastic fibers

Pseudoxanthoma elasticum–like papillary dermal elastolysis: A large case series with clinicopathological correlation

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is a rare acquired elastic tissue disorder. To date, less than 20 cases have been reported. We report a case series of 17 patients presenting with PXE-like PDE and discuss the clinicopathological correlation. Seventeen cases of PXE-like PDE were collected prospectively and evaluated for common demographic, clinical, and histopathological features. All patients were women with a mean age of 61.8 years. The lateral sides and back of neck were the most common sites of involvement (100%), followed by the supraclavicular region (41.2%) and the axilla (35.3%). Systemic involvement was absent in all cases, and in 7 patients the discovery of PXE-like PDE was an incidental finding. The main histopathologic features included complete loss (82.4%) or marked reduction (17.6%) of elastic fibers in the papillary dermis and the presence of melanophages in the same zone (88.2%). Our results require validation with a larger series. Our findings help to differentiate PXE-like PDE from similar elastic tissue disorders based on the selective elastic tissue elimination in the papillary dermis and the presence of melanophages in the same zone as a possible consequence of subclinical junctional photodamage. PXE-like PDE is likely underdiagnosed rather than rare, and dermatologists should be aware of its similarity to inherited PXE to spare unnecessary investigations because of the lack of systemic involvement. Clinicopathologic correlation is critical as hematoxylin-eosin staining is nonspecific and elastic tissue stains are necessary to make the correct diagnosis.

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.

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.