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Papillary dermal elastolysis has been described in the setting of experimental combination nivolumab and cabiralizumab immunotherapy. We report a third patient with distinctive, generalized atrophic macules that developed after a morbilliform eruption during a clinical trial for treatment of metastatic pancreatic adenocarcinoma. Histopathological findings demonstrated diminished elastic fibers in the papillary dermis, associated with a histiocyte-rich infiltrate and increased dermal mucin, features that should clue the dermatopathologist to this condition.

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.

Pseudoxanthoma elasticum-like papillary dermal elastolysis is an acquired elastic tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic involvement. Histopathologically, special staining of elastic fibers demonstrates a total or partial band-like loss of elastic fibers in the papillary dermis. Although ultraviolet radiation seems to be one of the main etiological factors in this entity, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck of a woman who wore hijab.

Multiple cutaneous side effects have been reported with the use of immunotherapies including programmed cell death protein 1 inhibitors. We report 2 patients who presented with papillary dermal elastolysis presenting as multiple, skin-colored, wrinkled papules and atrophic macules following an inflammatory eruption in the setting of combination chemotherapy with nivolumab and cabiralizumab. These two cases highlight a novel finding, elastolysis in the setting of chemotherapy with nivolumab and cabiralizumab.

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.

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis and focal dermal elastosis are acquired noninflammatory elastic tissue disorders. The skin lesions clinically resemble PXE without systemic involvement. We report the clinicopathological features of five cases and discuss the pathogenesis and differential diagnosis. Increased awareness of this newly described entity will broaden the differential diagnosis for dermatologists when encountering a similar case scenario.

Elastic tissue disorders are heterogeneous and are characterized by increased or decreased dermal elastic tissue. The disorders are further defined by characteristic clinical morphologies and distributions, presence or absence of extracutaneous associations, and distribution and morphology of the elastic tissue alteration. Pseudoxanthoma elasticum (PXE) is an inherited elastic tissue disorder characterized by yellowish papules coalescing into cobblestone plaques on the neck and flexural skin, with increased calcified clumped elastic tissue on biopsy and associated cardiac and ophthalmologic abnormalities. PXE-like disorders that lack systemic associations and calcification of elastic fibers may mimic PXE. These include PXE-like papillary dermal elastolysis (PXE-PDE), white fibrous papulosis of the neck (WFPN), and papillary dermal elastosis (PDE), all of which show elastic tissue loss in the papillary dermis (elastolysis), with variably increased dermal collagen. These are collectivly referred to as fibroelastolytic papulosis (FEP) disorders and are briefly covered in the elastolysis chap. 13 as well. Late-onset focal dermal elastosis (LOF9DE) also presents with PXE-like papules but shows increased reticular dermal elastic fibers. FEP disorders may also have increased elastic tissue; thus, the term fibroelastolytic and elastotic papulosis (FEEP) acknowledges this histologic variability and allows the inclusion of LOFDE in this clinical-pathologic spectrum of elastic tissue disorders. Linear focal elastosis, also characterized by increased dermal elastic fibers, is distinguished by cutaneous horizontal linear bands across the lower and mid spine. This chapter will discuss the clinical and histopathologic features of these disorders.

Acquired elastolytic disorders comprise a complex spectrum of rare skin diseases that are characterized by loss of elastic tissue. In this review, acquired elastolytic skin disorders are briefly introduced, including acquired cutis laxa, anetoderma, annular elastolytic giant cell granuloma, mid-dermal elastolysis, papular elastorrhexis, perifollicular elastolysis, sporadic acrokeratoelastoidosis, and a relatively new condition named fibroelastolytic papulosis with its subtypes pseudoxanthoma-like papillary dermal elastolysis and white fibrous papulosis of the neck. Given the paucity of reported patients and limited research in this field, the pathomechanisms of acquired elastolytic skin diseases are poorly understood and effective therapeutic approaches are absent.

Fibroelastolytic papulosis of the neck (FEPN) consists of two disorders: white fibrous papulosis of the neck (WFPN) and pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE). The neck and supraclavicular areas are frequently involved; however, axillary involvement is significantly more rare, especially for white fibrous papulosis. Herein, we present an unusual case of white fibrous papulosis of the axillae, in addition to the neck, in a Caucasian woman.

Mid-dermal elastolysis is a rare acquired elastic tissue disorder with about 100 cases reported in the literature. It is characterized by localized patches of finely wrinkled skin on the shoulder and upper extremities and a band-like loss of elastic tissue in the mid-dermal layer on biopsy. Some patients may have symptoms of discomfort, erythema, and/or pruritis. Mid-dermal elastolysis is predominantly seen in young to middle-aged Caucasian females and extensive skin involvement may lead to cosmetic concerns. Furthermore, it is important to rule out other disorders of elastic fiber that are associated with systemic involvement. We present a case of MDE, discuss the differential diagnosis, and describe characteristic clinical features and histology findings of each condition.

Pseudoxanthoma elasticum (PXE) is a disease characterized by aberrant mineralization of soft tissue and fragmentation of elastic fibres. It is often difficult to distinguish PXE clinically from pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-like PDE). However, we have identified that the dermoscopic findings in PXE include coalescing and reticulated yellow-white clods on a light purple-red background, whereas the dermoscopic findings in PXE-like PDE lack such a coloured background. To our knowledge, this is the first detailed description of dermoscopic differences between PXE and PXE-like PDE.

Objective Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare skin condition seen predominantly in elderly females. These asymptomatic lesions are brought to the dermatologist's attention due to patient's displeasure in their appearance. We report a case of a 28-year-old female with PXE-PDE on the right neck treated with nonablative fractional resurfacing (NAFR). Case The patient on examination had yellow-to-skin-colored papules that coalesced into a cobblestone-appearing plaque on her lateral neck. The area was treated with NAFR to improve texture and cosmesis. After three treatments, the patient noted at least a 50% improvement of appearance and texture in the affected skin. Adverse effects were mild without development of serious side effects, such as scarring and/or permanent dyspigmenation. Conclusion Established therapies for elastin disorders like PXE-PDE have yielded unsatisfactory results by patient and clinician standards. Positive results with NAFR, illustrated in our PXE-PDE case, further provides a role for NAFR in elastin disorders.

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.

Background: Pseudoxanthoma elasticum–like papillary dermal elastolysis (PXE-PDE), a rare acquired elastolytic disorder, occurs in postmenopausal, elderly women and is characterized by soft, white-yellow papules that localize on the neck and supraclavicular regions and often coalesce into cobblestone plaques. It has no systemic involvement and is histologically distinct from the clinically similar inherited pseudoxanthoma elasticum. Case Summary: A 64-year-old Caucasian woman presented with a 2-year history of multiple, asymptomatic soft yellow 1- to 2-mm papules on her neck, which were increasing in number. On histopathology, haemotoxylin and eosin stain showed a normal-appearing papillary dermis; however, Verhoeff–van Gieson elastic stain showed absent elastic fibres in the papillary dermis. Papular elastorrhexis was suggested, but given its clinical picture, PXE-PDE was also considered. There was no resolution after 2 courses of intralesional triamcinolone acetonide (0.1 mL of 5 mg/mL). Conclusion: PXE-PDE, which is characterized by complete loss or significant reduction of elastic fibres in the papillary dermis, was consistent with our patient’s presentation and histologic findings. Papular elastorrhexis, a paediatric cutaneous disorder, was initially considered, but clinicopathologic correlation led to a diagnosis of PXE-PDE. Intralesional corticosteroids was tried as a treatment but was ineffective at improving the condition. This report documents the importance of histopathology and clinicopathologic correlation when differentiating the overlapping variants of fibroelastolytic disease.

Mid-dermal elastolysis is an acquired skin condition affecting the elastin fibers of the dermis, resulting in laxity of the skin. We report a case of mid-dermal elastolysis for which novel treatment with mycophenolate mofetil was successful.

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.

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.

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare acquired non-inflammatory disorder characterized by papules that clinically resemble PXE and histologically by a total or partial loss of elastic fibers in the papillary dermis. It typically affects elderly women. Here, we present a 60-year-old woman diagnosed with PXE-PDE which is a rarely encountered condition.

A 55-year-old healthy Caucasian female, on no medication, was seen by a dermatologist because of a patchy, slightly indurated and violaceous eruption involving her neck and trunk. The clinical impression was of granuloma annulare (GA). Over a period of several months the violaceous lesions became atrophic with loss of colour and eventual wrinkling of lesional skin. Sequential skin biopsies were obtained, which revealed a spectrum of changes. Those from early violaceous lesional zones displayed perivascular lymphocytic infiltrates and interstitial granulomatous inflammation, characteristic of interstitial GA. Samples from atrophic lesional areas appeared normal on routine sections but an Orcein-Giemsa (OG) stain, prompted by the clinical history of atrophy, revealed absence of elastic fibers in the mid-reticular dermis. The combined clinicopathologic findings pointed to development of mid-dermal elastolysis (MDE) at involutional sites of GA. Owing to consideration of a cutaneous T-cell lymphoma in the differential diagnosis, genotyping in search of T-cell monoclonality was performed and yielded a negative result. Our case supports the existing but scant evidence in the literature that the rare, enigmatic condition termed MDE is an end-result of inflammatory destruction of dermal elastic fibers. GA is one form of dermatitis capable of culminating in this entity, but others have also been implicated.

Mid-dermal elastolysis is a rare disorder characterised by focal loss of elastic tissue in the mid-dermis. Most patients present with lesions of fine wrinkling (type I) or perifollicular papules (type II). The reticular variant (type III) has been described less often in the literature. We report a case of a young woman with type III mid-dermal elastolysis.

An 89-year-old Japanese woman had pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck and linear focal elastosis on the flexor aspects of the legs and thighs. The lesions of both diseases had similar histologic patterns-loss of elastic fibers in the papillary dermis and accumulation of normal-appearing elastic fibers in the subpapillary or mid dermis. Immunohistochemical analysis showed elastin, and microfibril-associated proteins (fibrillin 1 and 2 and microfibril associated glycoprotein 1 and 4) were absent or decreased in the papillary dermis and present in the accumulated elastic fibers in both lesions. The association of 2 diseases on different areas of the same patient and histologic and immunohistologic similarities between the diseases suggest that, despite the clinical differences, both diseases are closely related. (J Am Acad Dermatol 2002;47:S189-92.)

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.

Mid-dermal elastolysis (MDE) is an uncommon and probably underdiagnosed disorder of the elastic tissue. Clinical suspicion and histopathological confirmation are essential for the diagnosis. We report the case of a young woman who presented with this disorder after an inflammatory process during pregnancy and we review the main characteristics of this rare entity.

A 56-year-old white man with multiple, discrete nonfollicular papules on the neck is presented. Clinical and histopathologic features were compatible with the entity of white fibrous papulosis of the neck (WFPN). Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and WFPN are further clinicopathologic patterns of intrinsic aging. Clinically, WFPN is characterized by isolated, whitish papules, whereas those of PXE-PDE are yellowish and often coalesce to form "cobblestone" plaques. Our case showed clearly marginated whitish papules. The major histopathologic feature of WFPN is superficial dermal fibrosis with scant elastolysis; in PXE-PDE, there is papillary dermal elastolysis but no sign of fibrosis. No recurrence was performed in the 3 years' follow-up in our case. Surgical treatment may be considered in such cases with well-circumscribed lesions.

Background: Mid-dermal elastolysis is an acquired disorder of elastic tissue clinically characterized by diffuse fine wrinkling, most often of the trunk and arms. Histologically, a clear band of elastolysis is present in the mid-dermis. Objective: Although examples of diffuse elastolysis are well known, only a small number of patients with mid-dermal elastolysis have been reported to date. We present two patients with clinical and histological evidence of mid-dermal elastolysis, review the literature, and summarize the salient features of some common disorders of elastic tissue. Methods: The first patient presented with fine wrinkles and papules over the upper arms, upper chest, and axillae, and demonstrated increased laxity of the eyelids. The second patient had striking wrinkles extending in a band-like pattern on her arms, upper chest, back, and abdomen. Neither one of our patients had a previous history of skin inflammation, urticaria, or any other underlying diseases related to their skin changes. Skin biopsies were taken from lesional and perilesional skin of both patients, and were stained with hematoxylin and eosin, and with elastic tissue stain. In addition, a tissue sample from Patient 1 was fixed for electron-microscopy. Results: Hematoxylin and eosin stains did not demonstrate specific changes or diagnostic patterns. However, elastic tissue stains revealed a band-like loss of elastic tissue in the mid-dermis. Elastic tissue in the remaining superficial and deep dermis stained normally. Electron-microscopy was consistent with these findings and revealed significant loss of elastic tissue limited to the mid-dermis. Conclusion: We have presented two cases of mid-dermal elastolysis and reviewed the literature. To date, the pathophysiology of mid-dermal elastolysis had not been elucidated and no definitive therapy exists.

Summary Mid-dermal elastolysis is an unusual process characterized by the absence of the elastic fibres within the mid-dermis. The disappearance of these fibres causes fine wrinkles (type 1) or perifollicular protrusions (type 2) affecting the trunk, neck and arms. There is a female predilection, and most patients have a history of intense sun exposure. Recently, some cases of mid-dermal elastolysis presenting as reticular erythema have been reported. We report a case of reticular erythema with mid-dermal elastolysis that occurred in a 70-year-old man after insertion of a pacemaker.

We report the case of a 38-year-old Caucasian female presenting asymptomatic plaques of fine wrinkling and perifollicular papular protrusions especially on the trunk. Histological examination evidenced loss of elastic fibers in the mid-dermis due to elastophagocytosis, with giant cells and granuloma formation. Moreover, elevated titers of thyroid autoantibodies were detected and thyroid ultrasound revealed echo-poor tissue. These findings met the diagnoses of mid-dermal elastolysis and Hashimoto's thyroiditis. This association has not been reported before. We present a comprehensive overview of the literature and discuss the pathogenetic aspects of mid-dermal elastolysis and the significance of the association with Hashimoto's thyroiditis.

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.

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.

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).