Solitary lesion in papular elastorrhexis: A diagnostic challenge.

Sir,

Papular elastorrhexis is a rare acquired disorder of elastic tissue, which is characterized by multiple, nonfollicular, skin colored to white papules that predominantly affects trunk and upper extremities. The papules commonly appear in adolescence without any prior inflammation or trauma and affects females more than males. Histologically it is characterized by fragmentation and decreased elastic fibers in reticular dermis with or without alteration in collagen fibers.[1] Here in we report a case of papular elastorrhexis in a 16-year-old male having a solitary lesion.

A 16-year-old male came to our outpatient department with complaint of a pea-sized, soft, white-colored lesion over his right shoulder since 6 months. On examination single, well-defined, soft, hypopigmented, nonfollicular papule of 0.5 cm in diameter with wrinkled surface was present over right shoulder [Figure 1]. Lesion was compressible on palpation. There was no history of any skin lesion or inflammation or trauma over affected site prior to occurrence of the lesion. Histopathological examination was done considering differential diagnoses of anetoderma and papular elastorrhexis, which on 40X revealed mild hyperkeratosis and epidermal acanthosis with underlying unremarkable dermis [Figure 2], whereas Verhoeff–van Gieson (VVG) staining on high resolution (200X) revealed markedly reduced elastic fibers in superficial dermis [Figure 3] and fragmentation of elastic fibers in lower dermis (400X) [Figure 4]. Based on clinical and histopathological findings, final diagnosis of papular elastorrhexis was made.

Figure 1

Solitary, hypopigmented, nonfollicular, papule with wrinkled surface present over right shoulder

Figure 2

Photomicrograph showing mild hyperkeratosis and epidermal acanthosis with underlying unremarkable dermis (H and E × 40)

Figure 3

Photomicrograph showing markedly reduced elastic fibers in superficial dermis (VVG ×200)

Figure 4

Photomicrograph showing fragmentation of elastic fibers in lower dermis (VVG ×400)

Papular elastorrhexis, first reported by Bordas et al. in 1987,[2] is a rare acquired elastic tissue disorder. Till date only 31 cases have been reported of which 11 were males and 20 were females, thus showing a female predominance.[1] Lesions usually occur over trunk and upper extremities, however, rarely lesions may also be seen on scalp, face, shoulders, axillae, thighs, neck, and retroauricular region.[1] Solitary lesion as in our case has not been yet described in literature. Important histological feature is loss or decreased and fragmented elastic fibers with or without any changes in collagen bundles.[1]

Exact etiology of papular elastorrhexis is not known. It is considered as a variant of nevus anelasticus[2] or abortive form of Buschke–Ollendorff syndrome.[3] It has also been suggested that nevus anelasticus, papular elastorrhexis and collagenoma represent a single disease or disease spectrum and should be included under papular elastorrhexis.[4]

Apart from various connective tissue disorders, anetoderma is an important clinical differential diagnosis of papular elastorrhexis. Histopathologically, anetoderma shows marked to total loss of elastic fibers in dermis,[5] whereas papular elastorrhexis has loss as well as fragmentation of elastic fibers.

Till date there is no proven treatment for papular elastorrhexis. However, intralesional triamcinolone injection can improve the condition to some extent.

The purpose of this report is to highlight the occurrence of solitary lesion in papular elastorrhexis and importance of histopathological examination in diagnosis of papular elastorrhexis and its differentiation from anetoderma. We believe that papular elastorrhexis is an underdiagnosed entity due to its subtle and benign nature and its close clinical resemblance to various other connective tissue pathologies.

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Conflicts of interest

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