Dermatology Online Journal

Papillary dermal elastosis
Tracey Newlove MD, Julia Tzu MD, Shane Meehan MD
Dermatology Online Journal 17 (10): 12

Department of Dermatology, New York University, New York, New York

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Abstract

There are numerous acquired disorders of elastic tissue that are distinguished by a combination of clinical appearance, location, gender, age of onset, and characteristic histopathologic findings. We present a case of a 36-year-old man with multiple confluent, hypopigmented papules that coalesced into plaques with prominent follicular ostia over the dorsal aspects of the forearms, shoulders, upper chest, and upper back. Histologically there was selective loss of papillary dermal elastic fibers. The clinical and histopathologic findings in this case are consistent with an acquired disorder of elastic tissue which we believe represents the second reported case of papillary dermal elastosis.

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History

A 27-year-old man presented to the Manhattan Campus of the VA New York Harbor Healthcare System in 2001 for evaluation and treatment of inflammatory facial acne. On examination of his upper back and arms, a cutaneous eruption was noted. On further questioning, the patient reported this eruption had been present for at least three years. He reported a history of severe inflammatory acne on his upper back previously but no prior acneiform or inflammatory eruptions on the arms. He denied pruritus or other symptoms preceding the onset of the current eruption. The patient also denied a history of sunburns, frequent sun exposure, or tanning bed use. A punch biopsy was performed from lesional skin.

Past medical history included severe nodulocystic acne on his face and trunk, insulin-controlled diabetes mellitus, and depression. The diagnosis of diabetes mellitus was made in 2010, greater than ten years after the onset of the cutaneous eruption. Current medications include insulin, aspirin, citalopram, and lisinopril. A review of systems with emphasis on ocular, cardiovascular, pulmonary, and rheumatologic symptoms was negative. He reports former tobacco use for 25 years.

Physical examination

Figure 1	Figure 2

Multiple, confluent, hypopigmented papules that coalesed into plaques with prominent follicular ostia were present over the dorsal aspects of the forearms, shoulders, upper chest, and upper back. Several hyperpigmented atrophic patches also were noted. There was no erythema, crust, or scale.

Laboratory data

A complete blood count and comprehensive metabolic panel were normal except for glucose of 207 mg/dL.

Histopathology

Figure 3

There is loss of elastic fibers throughout the papillary dermis and fragmentation of elastic fibers in the upper reticular dermis as demonstrated by an EVG stain. Photolichenoid drug eruption: There is psoriasiform epidermal hyperplasia with mild spongiosis, hypergranulosis, and compact orthokeratosis. There is a perivascular and focally lichenoid lymphocytic infiltrate with some eosinophils.

Discussion

There are numerous acquired disorders of elastic tissue that are differentiated by a combination of clinical appearance, location, gender, age of onset, and characteristic histopathologic findings. The clinical and histopathologic findings in this case are consistent with an acquired disorder of elastic tissue, which we believe represents the second reported case of papillary dermal elastosis. In 2008, a 33-year-old woman with confluent, white-to-yellow papules on the upper back and neck with foci of clumped, granular elastic tissue, alternating with foci of decreased papillary dermal elastic fibers was reported [1]. The differential diagnosis of this unique entity includes several disorders of elastic tissue such as mid-dermal elastolysis, pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE), white fibrous papulosis of the neck (WFPN), late-onset focal dermal elastosis, anetoderma, and cutis laxa.

Mid-dermal elastolysis (MDE) is a rare, acquired condition that is characterized by well-circumscribed or large, diffuse areas of fine wrinkling (type 1) as well as discrete perifollicular papules with prominent follicular indentation (type 2) on the trunk, lateral aspects of the neck and upper extremities with selective loss of elastic fibers in the mid-dermis. First described in 1977 [2], approximately 80 cases have been reported in the literature with most cases occuring in middle-aged Caucasian women [3]. Pathogenesis is unknown, but proposed factors include exposure to natural or artificial ultraviolet (UV) radiation [4, 5] as well as a variety of inflammatory dermatoses, such as urticaria, granuloma annulare, and Sweet syndrome [6, 7].

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN) are related clinical entities that together have been termed fibroelastolytic papulosis (FEP) [8]. PXE-PDE presents as multiple, yellow or skin-colored non-follicular cobblestone-appearing papules in flexural areas that may clinically resemble type 2 MDE. First described in 1992 [9], this condition has thus far only been documented in women. Most cases have presented after the fifth decade, with only one case reported in a 26-year-old woman [10]. Histopathologic features include partial or total band-like elastolysis of the papillary dermis. WFPN, which is observed in both sexes, is characterized by isolated, two-to-three-mm, nonfollicular, white or skin-colored papules that are symmetrically scattered on the upper neck. The main histopathologic feature is thick collagen bundles in the papillary to mid-dermis [11], with loss of elastic fibers in the papillary and reticular dermis [12].

Late-onset focal dermal elastosis presents clinically with scattered, yellow papules that preferentially involve the neck, upper back, and axillae, with onset typically after the sixth decade of life. The histopathological pattern consists of increased normal-appearing elastic tissue that is located predominately in the mid and deep reticular dermis [13].

Anetoderma presents as discrete, well-circumscribed, pouch-like areas of flaccid skin that preferentially affect the trunk and proximal aspects of the extremities. Two clinical presentations are distinguished by the presence or absence of inflammatory lesions prior to onset. Histopathologic features include the condition is characterized by a loss of elastic fibers in both the papillary and reticular dermis [14].

Acquired cutis laxa is characterized by loosely hanging skin folds that are often preceded by an inflammatory eruption, such as eczema, urticaria, or erythema multiforme as well as loss of elastic fibers throughout the papillary and reticular dermis [15]. There is an appreciable risk of systemic elastolysis affecting the gastrointestinal, urogenital, pulmonary, and cardiovascular systems.

References

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10. Lee G, et al. A case of pseudoxanthoma elasticum-like papillary dermal elastolysis. Kor J Dermatol 2002;40:274

11. Shimizu H, et al. White fibrous papulosis of the neck: a new clinicopathologic entity? J Am Acad Dermatol 1989;20:1073 [PubMed]

12. Balus L, et al. Fibroelastolytic papulosis of the neck: a report of 20 cases. Br J Dermatol 1997;137:461 [PubMed]

13. Higgins J, Whitworth M. Late-onset focal dermal elastosis: a case report and review of the literature. Cutis 2010;85:195 [PubMed]

14. Venencie PY, Winkelmann RK. Histopathologic findings in anetoderma. Arch Dermatol 1984;120:1040 [PubMed]

15. Nanko H, et al. Acquired cutis laxa (generalized elastolysis): light and electron microscopic studies. Acta Derm Venereol 1979;59:315 [PubMed]

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