EXTRAORDINARY CASE REPORT

Elastoderma: Case Report and Literature Review Hala Adil, MD* and Sarah Walsh, MD†

Abstract: Elastoderma is a rare cutaneous condition, which clinically presents as an acquired laxity and decreased recoil of skin. It is histologically characterized by an increase of pleomorphic and fragmented elastic fibers in the superficial dermis. A case of a 61-year-old woman with wrinkled lax skin on the anterior and lateral aspects of her neck was reported. Microscopic examination revealed an accumulation of fragmented curled elastic fibers in the dermis. This is the fifth reported case of elastoderma. Key Words: elastoderma, disorders of elastic tissue (Am J Dermatopathol 2015;37:577–580)

A punch biopsy from the right lateral neck was taken. Histological examination with routine hematoxylin–eosin staining (Figs. 2A, B) and Verhoeff–van Gieson (Fig. 3A, B) staining revealed an increase of fragmented, curled, and thickened elastic fibers with clumping in the superficial dermis, reminiscent of pseudoxanthoma elasticum. However, Von Kossa staining (Fig. 4) showed no evidence of calcification. These findings are, in conjunction with the clinical presentation, consistent with the diagnosis of elastoderma.

DISCUSSION

Elastoderma is a rare condition that was first described by Kornberg et al1 in 1985 in an otherwise healthy 33-year-old

INTRODUCTION Elastoderma is a rare condition characterized by localized areas of lax skin. In the reported cases thus far, elastoderma typically occurs in young adults, both male and female, on the neck and extremities. Histologically, it has an increase of fragmented elastic fibers in the dermis without calcification. Various treatments have been tried without much success. In this case report, we describe a patient with localized lax skin on the neck. Microscopic examination showed an increase of fragmented curled elastic fibers in the dermis, consistent with elastoderma. To our knowledge, this is the fifth reported case of elastoderma. It is important to keep this rare condition in mind as to avoid incorrect diagnosis or treatments.

CASE REPORT A 61-year-old white woman presented to dermatology clinic with the complaint of wrinkling and thickening of skin over the neck and chest for the past 4 years. On physical examination, the patient was noted to have lax wrinkled skin on the anterior and lateral aspects of her neck and the superior chest (Figs. 1A, B). There was no history of trauma to the area. The remainder of physical examination was normal. The patient did not have a diagnosis of an autoimmune disease or connective tissue disorder. She did have a history of hypertension and hyperlipidemia, for which she was on olmesartan and atorvastatin. She denied ever having taken penicillamine. A laboratory workup, including a comprehensive metabolic panel, lipid panel, blood count, antinuclear antibody, rheumatoid factor, erythrocyte sedimentation rate, C-reactive protein, and acute hepatitis panel, was within normal limits. From the *Department of Dermatology, Saint Louis University, St. Louis, MO; and †Cutaneous Pathology, WCP Laboratories, Inc, St. Louis, MO. The authors declare no conflicts of interest. Reprints: Hala Adil, MD, Department of Dermatology, St. Louis University, 1755 South Grand Boulevard, St. Louis, MO 63104 (e-mail: [email protected]). Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.

Am J Dermatopathol  Volume 37, Number 7, July 2015

FIGURE 1. Physical examination remarkable for lax wrinkled patches on the anterior (A) and lateral (B) aspects of patient’s neck. www.amjdermatopathology.com |

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Adil and Walsh

FIGURE 2. Skin biopsy specimen (A, hematoxylin–eosin staining, magnification ·2; B, hematoxylin–eosin staining, magnification ·40) demonstrates increase in fragmented, curled, and thickened elastic fibers with clumping in the superficial dermis.

Am J Dermatopathol  Volume 37, Number 7, July 2015

FIGURE 3. Elastic stain (A, Verhoeff–van Gieson staining, magnification ·4; B, Verhoeff–van Gieson staining, magnification ·40) demonstrates increase of pleomorphic elastic fibers in the dermis.

woman with skin laxity localized on her elbow. Since then, 3 other cases have been reported with similar findings of focal areas of lax wrinkled skin on the neck, elbows, and knees with delayed recoil2 (Table 1). Histologically, this entity is characterized by increased and fragmented pleomorphic elastic fibers in the papillary and superficial dermis.5 There is no evidence of calcification.4 Transmission electron microscopy shows fibroblast-like cells with prominent rough endoplasmic reticulum and irregular deposits of elastic material at the periphery of elastic tissue fibers, with grape-like globular structures.3 Elastic fibers are a vital component of mammalian tissue, providing qualities of resilience and elastic recoil. Mature elastic fibers are composed of 90% elastin and are located in the mid and deep reticular dermis. The papillary

dermis contains oxytalan fibers, which run perpendicularly to the dermo–epidermal junction—they serve to connect the basal lamina to the underlying dermal elastic tissue and are composed of only microfibrils. Elaunin fibers contain a small amount of elastin and form a horizontal plexus in the upper reticular dermis. Cross-linked elastin is degraded by elastases, which are secreted by neutrophils, macrophages, platelets, and fibroblasts. This is found in all classes of proteinases, notably metalloproteinases (MMP). MMPs are thought to play a role in the elastolysis seen in anetoderma and acquired cutis laxa associated with inflammatory skin disease.3 In elastoderma, Kornberg et al1 postulated that the accumulation of elastin results from increased synthesis of elastin rather than degradation, supported by the electron microscopy findings of active fibroblasts with prominent rough endoplasmic reticulum. Inflammatory processes may be responsible for this

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Am J Dermatopathol  Volume 37, Number 7, July 2015

Elastoderma: A Case Report

FIGURE 4. Skin biopsy specimens are negative for calcification (Von Kossa staining, ·4).

process, as previously reported patients had erysipelas and dyshydrotic eczema2 and folliculitis4; however, our patient had no preceding inflammation or trauma to the affected area. The differential diagnosis of elastoderma includes other disorders of elastic tissue (Table 2). Most other disorders of increased elastic fibers have a clinical presentation different than elastoderma. These include elastoma, focal dermal elastosis, elastofibroma, and pseudoxanthoma elasticum. Elastoma is a type of connective tissue nevus that presents as ill-defined yellowish papules and nodules

and is characterized histologically by thickened tortuous elastic fibers in the dermis.3 Elastofibroma is a solitary slow-growing nodule or plaque commonly found in the subscapular region of older women and is thought to be a reaction to prolonged mechanical stress. Histologically, elastofibroma is characterized by fragmented and globular aggregates of elastic material in the dermis.3 Pseudoxanthoma elasticum clinically presents as yellow macules and papules on the neck, axillae, and groin and has a similar histological appearance as elastoderma with pleomorphic and fragmented elastic fibers in the superficial dermis. However, in pseudoxanthoma elasticum, there is calcification of the fragmented elastic fibers in the dermis, which can be confirmed with the Von Kossa stain. 3,6 Focal dermal elastosis has a similar clinical presentation to pseudoxanthoma elasticum; however, it histologically shows an increase of normal appearing elastic fibers in the dermis without calcification.6,7 Given the clinical laxity of the skin, cutis laxa, anetoderma, and mid dermal elastolysis are often considered; however, these are characterized by a decrease of elastic fibers. Cutis laxa is a rare disorder that clinically presents as loose pendulous skin. Histologically, it is characterized by a decrease of elastic fibers in the dermis; remaining fibers are often shortened and fragmented, and there is a variable inflammatory infiltrate.6,7 Anetoderma is a cutaneous disorder characterized by localized lesions with a wrinkled loose surface or a sac-like herniation of skin. This may be a primary process or may follow an inflammatory process. Histological

TABLE 1. Reported Cases of Elastoderma Site

Duration, yr

Gender

Age, yr

Clinical Findings

Kornberg et al1

Female

33

Right elbow

4

Inelasticity and pendulousness of the skin, incomplete and delayed recoil of skin

Yen et al3

Male

27

Anterior and posterior neck

2

Lax, extensible, wrinkled skin with areas of ulceration at site of old arthropod bite, incomplete and delayed recoil of skin

Vieira et al4

Male

16

Anterior neck

2

Circumscribed, 5 cm area of lax, extensible skin with delayed recoil

De Waal et al2

Male

25

Bilateral elbows and knees

Our case

Female

61

Anterior and lateral neck

10

Skin laxity of elbows and knees with delayed recoil

4

Lax, wrinkled skin on anterior neck and superior chest

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Histology Dense aggregates of pleomorphic elastic tissue in the dermis and subcutaneous adipose tissue, von Kossa stain negative for calcification Increased number of thin elastic fibers in the dermis

Accumulation of thin, irregular, intertwined elastic fibers within the dermis, von Kossa stain negative for calcification Increase in elastic tissue fibers in the reticular dermis with clumping and fragmentation, von Kossa staining negative for calcification Increase of fragmented, curled, and thickened elastic fibers with clumping in the superficial dermis, von Kossa stain negative for calcification

Treatment or Follow-up None provided

Ulcerations healed over 6 months, persistent laxity of skin. No treatment documented Partial excision of lesion; however, lesion reacquired original dimension Excision of excess skin, hyperlaxity of skin returned to lesser extent

Patient declined treatment

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Am J Dermatopathol  Volume 37, Number 7, July 2015

Adil and Walsh

TABLE 2. Elastic Tissue Disorders in the Differential Diagnosis of Elastoderma Disorder

Clinical Presentation

Elastoderma

Focal areas of lax wrinkled skin

Elastoma

A connective tissue nevus; Ill-defined yellowish papule(s) or nodule(s)

Focal dermal elastosis

Yellow papules on extremities

Elastofibroma

Solitary, slow-growing nodule or plaque on subscapular back Yellow macules and papules in the neck, axillae, and groin

Pseudoxanthoma elasticum

Penicillamine dermopathy

Fragility and wrinkling of skin may also see milia and purpura

Cutis laxa

Generalized or localized loose pendulous skin

Mid dermal elastolysis

Well-defined areas of fine wrinkling or perifollicular papules on the upper extremities, neck, and trunk, or persistent reticular erythema on the upper back Localized lesions with loose wrinkled surface or sac-like herniations of skin

Anetoderma

Histopathology Excessive accumulation of fragmented curled elastic fibers in the upper to mid dermis, Von Kossa stain negative for calcification Localized area of increased thickened tortuous, broad branching elastic fibers in the mid and lower dermis Increase in normal appearing elastic fibers in the mid and deep dermis Fragmented and globular aggregates of elastic material in the dermis Pleomorphic and fragmented elastic fibers in the superficial dermis, Von Kossa stain positive for calcification Excess pleomorphic elastic fibers with thickening and lateral budding similar to pseudoxanthoma elasticum, von Kossa stain negative for calcification Decrease or loss of elastic fibers in the dermis, with shortening and fragmentation Absence of elastic fibers in a welldefined horizontal band in mid dermis may see perivascular lymphocytic infiltrate with phagocytosis of elastic fibers Decrease to complete the absence of elastic fibers in superficial to the mid dermis

examination is remarkable for a decrease to the absence of elastic fibers in the superficial to mid dermis.6 Mid dermal elastolysis is characterized by widespread areas of fine wrinkling, most often on the upper extremities, neck, and trunk of young women. In almost half of the reported cases, there is preceding erythema and burning, suggesting an inflammatory process as an underlying cause. Microscopic examination shows an absence of elastic fibers in the mid dermis with mild perivascular lymphocytic infiltrates in earlier lesions; phagocytosis of elastic fibers may be present.6 The histological differential of elastoderma includes penicillamine dermopathy. Penicillamine is a copper chelating agent used to treat Wilson disease. Prolonged use has been shown to cause elastic fiber disorders in skin and visceral tissue.8–10 Penicillamine depletes extracellular copper interfering with lysyl oxidase, and thus prohibiting the formation of sufficient cross-links of collagen and elastin fibers.9 Clinically, penicillamine dermopathy is characterized by fragility and wrinkling of skin, as well as milia and purpura.11 Light microscopy of tissue reveals excess elastic fibers with thickening and lateral budding.9,10 This is similar to the appearance of elastic fibers in elastoderma and pseudoxanthoma elasticum; however, penicillamine-induced elastosis rarely demonstrates calcification.8 There is no standard therapy available for elastoderma. Previous cases have been partially excised; however, hyperlaxity of skin has been reported to return after surgery.2,4 The diagnosis

1. Kornberg RL, Hendler SS, Oikarinen AI, et al. Elastoderma—disease of elastin accumulation within in the skin. N Engl J Med. 1985;312:771–774. 2. De Waal AC, Blockx WAM, Seyger MMB, et al. Elastoderma: an uncommon cause of acquired hyperextensible skin. Acta Derm Venereol. 2012;92:328–329. 3. Yen A, Wen J, Grau M, et al. Elastoderma. J Am Acad Dermatol. 1995; 33:389–392. 4. Vieira AC, Vieira WT, Michalany N, et al. Elastoderma of the neck in a teenage boy. J Am Acad Dermatol. 2005;53:S147–S149. 5. Lewis KG, Bercovitch L, Dill SW, et al. Acquired disorders of elastic tissue: part 1. Increased elastic tissue and solar elastotic syndromes. J Am Acad Dermatol. 2004;51:1–21. 6. Weedon D. Skin Pathology. 3rd ed. New York, NY: Churchill Livingstone; 2010:332–351. 7. Mitra S, Agarwal AA, Das JK, et al. Cutis laxa: a report of two interesting cases. Indian J Dermatol. 2013;58:328. 8. Na SY, Choi M, Kim MJ, et al. Penicillamine-induced elastosis perforans serpiginosa and cutis laxa in a patient with Wilson’s disease. Ann Dermatol. 2010;22:468–471. 9. Rath N, Bhardwaj A, Sharma PK, et al. Penicillamine induced psuedoxanthoma elasticum with elastosis perforans serpiginosa. Indian J Dermatol Venereol Leprol. 2005;71:182–185. 10. Coatesworth AP, Darnton SJ, Green RM, et al, Antonakopoulos. A case of systemic pseudo-pseudoxanthoma elasticum with diverse symptomatology caused by long term penicillamine use. J Clin Pathol. 1998;51: 169–171. 11. Tang MBY, Chin TM, Yap CK, et al. A case of penicillamine induced dermopathy. Ann Acad Med Singapore. 2003;32:703–705.

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of elastoderma was discussed with our patient and possible treatment options including excision, and she has deferred. REFERENCES

Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.

Elastoderma: Case Report and Literature Review.

Elastoderma is a rare cutaneous condition, which clinically presents as an acquired laxity and decreased recoil of skin. It is histologically characte...
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