Continuing medical education
Acquired disorders of elastic tissue: Part II. decreased elastic tissue

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Abstract

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.

Section snippets

Definition

Nevus anelasticus is an elastic tissue nevus disorder characterized by perifollicular papules and fragmentation or absence of elastic tissue.

History

Lewandowsky in 1921 described a subset of connective tissue nevi that commonly presents as grouped perifollicular papules on the pectoral region and that, at histopathologic examination, primarily or exclusively involves elastic tissue. He subsequently coined the term nevus elasticus.1., 2. Staricco and Mehregan in 1961 proposed the term nevus anelasticus

Definition

Papular elastorrhexis is characterized by asymptomatic papules and fragmentation of dermal elastic fibers.

History

Papular elastorrhexis was described in 1987 by Bordas and colleagues.6 Their patient had a nonfollicular papular eruption over the abdomen, back, and chest, and the most prominent histopathologic change was intense fragmentation of elastic tissue that resulted in a speckled appearance.

Epidemiology

Since 1987 there have been reports of approximately 13 cases of papular elastorrhexis, the majority of

Definition

Perifollicular elastolysis is an elastolytic disorder characterized by elastic tissue loss immediately surrounding hair follicles and balloonlike protrusions over affected areas.

History

In 1968 Varadi first referred to this disorder as perifollicular macular atrophy and later reclassified it as perifollicular elastolysis to reflect the location and proposed mechanism of decreased elastic fibers.10

Epidemiology

There have been 3 reported cases of perifollicular elastolysis, occurring in women during the fourth decade

Definition

Anetoderma (Greek anetos, “slack”), also known as macular atrophy, is characterized clinically by small, atrophic papules that herniate inward at palpation and histopathologically by loss of dermal elastic tissue.

History

Anetoderma was described in 1892 by Jadassohn in an 18-year-old woman who presented with erythematous papules on the elbows that resolved with atrophy and wrinkling.13., 14. Pellizzari described a similar condition under a different name in 1884.14., 15.

Epidemiology

Several hundred cases of

Definition

Acquired cutis laxa is characterized by loose redundant skin folds, loss of dermal elastic tissue that results in “bloodhound-like” or lugubrious facies, and risk of systemic involvement.

History

Marshall, Heyl, and Weber in 1966 first proposed a system of classification for cutis laxa that is widely accepted in the literature.54 The system distinguishes between acquired and inherited forms of the disease.

Epidemiology

Acquired cutis laxa is a rare disorder that usually affects adults, although isolated cases in

Definition

Postinflammatory elastolysis and cutis laxa is characterized by an acute papular eruption that resolves, leaving finely wrinkled skin and loss of dermal elastic tissue.

History

Postinflammatory elastolysis and cutis laxa were described by Marshall, Heyl, and Weber in 1966 in a series of five South African children who presented with an acute erythematous, infiltrated papular eruption.54

Epidemiology

Postinflammatory elastolysis and cutis laxa is a rare disorder that typically presents during infancy or childhood (1.5

Definition

White fibrous papulosis of the neck is characterized by a papular eruption on the neck with histologic evidence of fibrosis and variable loss of dermal elastic tissue.

History

White fibrous papulosis of the neck was described by Shimizu, Nishikawa, and Kimura in 1985 in a series of elderly Japanese patients with asymptomatic white papules on the posterior portion of the neck.88

Epidemiology

Eighty-one percent of the initial series of 32 cases were in men.88 Subsequently, additional cases have been reported in persons

Definition

PXE-like papillary dermal elastolysis is an acquired disorder characterized by papules that resemble PXE clinically, and nearly complete loss of elastic tissue in the papillary dermis histopathologically.

History

Rongioletti and Rebora in 1992 described PXE-like papillary dermal elastolysis in 2 female patients who presented with nonfollicular cobblestone-appearing papules that were coalescing into plaques on the neck; histopathologic examination showed elastolysis in the papillary dermis.97 No

Definition

Mid-dermal elastolysis is characterized histopathologically by focal loss of elastic tissue in the midreticular dermis and is manifest clinically as patches and plaques of finely wrinkled skin.

History

Shelley and Wood104 in 1977 described middermal elastolysis in a patient with a history of urticaria and endometriosis treated with oral contraceptives in whom histopathologic examination revealed the hallmark selective absence of elastic fibers in the mid-dermis. Contrary to their clinical suspicions, no

Definition

Upper dermal elastolysis is characterized by a papular eruption and selective loss of elastic tissue in the papillary dermis.

History

Upper dermal elastolysis was described by Hashimoto and Tye126 in 1994 in a patient with no known history of urticaria or unusual sun or chemical exposure, who presented with pruritus and small papules.

Epidemiology

There has been one reported case of upper dermal elastolysis in an 86-year-old white woman.

Etiology and pathogenesis

It has been suggested that lysis of elastic tissue may be a primary process or a

Definition

Granulomatous slack skin is a rare type of cutaneous T-cell lymphoma characterized by pendulous plaques and dermal elastolysis.

History

Bazex, Dupre, and Chritol in 1968 described the disorder in a patient with hanging skin folds and histologic evidence of granuloma formation.127 In 1978 Ackerman proposed the term granulomatous slack skin.128

Epidemiology

Granulomatous slack skin is a rare condition that has been reported in approximately 40 white patients, ranging in age from 14 to 69 years. Three times as many

Definition

Acrokeratoelastoidosis (AKE) is a palmoplantar keratoderma characterized by a papular eruption and loss of elastic tissue.

History

AKE was described by Costa in 1953 in an 18-year-old healthy white Brazilian woman who presented with a 3-year history of small asymptomatic papules on the palms and soles.146., 147.

Epidemiology

AKE is a rare disorder that commonly presents during childhood or adolescence, although presentation in adulthood (ages 54–77 years) has also been reported.148., 149. There appears to be no

Conclusion

Little is known about the cause and pathophysiology of many acquired disorders of elastic tissue. In this second of a 2-part review, we have covered acquired disorders that are characterized by loss or fragmentation of dermal elastic tissue. The clinical and histopathologic findings are diverse. Nevus anelasticus, papular elastorrhexis, perifollicular elastolysis, PXE-like papillary dermal elastolysis, and white fibrous papulosis of the neck appear to follow a benign course and to be of

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      Citation Excerpt :

      WFP is located mainly on the nape and less frequently on the lateral sides.7 On histopathology, the distinctive features are thickening of the collagen fibers in the papillary and sometimes the middermis, along with decreased elastic fibers; however, there is a link between WFPN and PXE-PDE, because both conditions have been considered as different clinicopathologic patterns of intrinsic skin aging,9 with the coexistence of the two entities being known as fibroelastolytic papules of the neck.25,34 Such patients show an asymptomatic monomorphic eruption, consisting of isolated papules, ranging in color from white to yellow, that merge into yellowish plaques on the neck.

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    Funding sources: Supported in part by an unrestricted grant from PXE International, Inc.

    Conflicts of interest: None identified.

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