Mid-Dermal Elastolysis: A Case Report and Literature Review

Sir,

Mid-dermal elastolysis (MDE) is a rare, non-inflammatory acquired disease. It is more common in Caucasian women and less common in Asian populations. The clinical manifestations of MDE are flesh-colored, well-circumscribed, finely wrinkled skin papules that primarily affect the trunk, upper limbs, neck, shoulders, and thighs.[1]

A 10-year-old patient, without significant past medical history, presented to the dermatology clinic with a 10-month-history of progressive wrinkling on the trunk. No pruritus, pain, or other symptoms of discomfort were noted. Physical examination revealed diffuse well-defined papules and patches on the shoulders, waist, and abdomen. In addition, some of the epidermis appeared to have fine wrinkle-like changes [Figures 1a and b]. A skin biopsy was taken from the lesion. Histopathology examination showed a generally normal epidermis, with keratinization and more atypical eccrine gland ducts visible in the superficial dermis [Figure 2]. Elastic fiber staining revealed a decrease in elastic fibers in the mid-dermis. A diagnosis of mid-dermal elastolysis was made based on these characteristic findings. Since there is no effective treatment for mid-dermal elastolysis, the patient only requested observation and follow-up, not medication. She was followed up by telephone for 2 months and claimed no new lesion had arisen, but the current lesions were also unchanged.

Figure 1.

Diffuse well-defined papules and patches on the waist and abdomen

Figure 2.

Histology of hemotoxylin and eosin (H and E)-stained biopsy (4× magnification)

MDE can be classified into three types clinically: Type I lesions present as well-defined fine wrinkled patches, type II lesions as perifollicular papular protrusions, and type III with persistent reticular erythema and telangiectasia. Recently, Wang et al. reported a case of MDE with the clinical presentation of soft blisters, which may require more similar case reports in the future.[2]

The exact pathogenesis of mid-dermal elastolysis is unclear, with reports suggesting an association with autoimmune diseases such as Hashimoto's thyroiditis, antiphospholipid syndrome, systemic lupus erythematosus, and rheumatoid arthritis. Moreover, there are also risk factors, including pregnancy, UV exposure, oral contraceptives, and smoking. UV radiation is considered an important factor among these, as many patients have a history of excessive exposure to sunlight prior to the onset of lesions.[3] However, the lesions did not appear on exposed areas such as the face and there was no histopathological evidence of sun elastosis.

It is generally accepted that increased expression or activity of elastases causes degradation of elastin in MDE and associated diseases like etoderma. Elastases including, MMPs, can be released by various cell types, such as skin fibroblasts, monocytes/macrophages, neutrophils, lymphocytes, and multinucleated giant cells. Furthermore, it has been reported that MMP9 was highly expressed at the lesion site, while LOX2, fibulin-5, and fibulin-4 molecules involved in elastin production were downregulated in MDE patients.[4]

Histopathologic examination is indispensable for the diagnosis of MDE. A band-like loss of elastic fibers in the mid-dermis is the most prominent feature in diagnosing MDE. Early lesions show perivascular lymphocytic infiltration with scattered multinucleated giant cells and histiocytes, while old lesions have mildly thickened dermal collagen without inflammatory cells.[3] Elastic tissue staining, with Verhoeff-van Giesonor or lichens red, would show that all elastic tissue in the mid-dermis have disappeared, with a few elastic fibers remaining around the hair follicles and blood vessels. However, the elastic fibers of the dermal papillae and other components, such as collagen fibers, blood vessels, and fibroblasts, are normal.

In this case, the patient exhibited the typical features of type I MED. She was previously healthy, with no history of sun exposure and no obvious factors related to possible etiology. The differential diagnosis includes other elastic tissue disorders such as cutis laxa, perifollicular elastolysis, post-inflammatory elastolysis, patchy atrophy, and ray elastic fiber degeneration, which can be excluded by combining clinical manifestations and histopathology.

There is no effective treatment for this condition. Avoidance of light and the use of topical shades may reduce the onset. Medications, including corticosteroids, colchicine, tretinoin, clofazimine, and vitamin E, can be applied but have not shown significant efficacy. Emerging natural therapies, including soy extract and eicosapentanoic acid, can benefit MDE patients by preventing elastic tissue degeneration. Recently, Smithson et al. administered mycophenolate mofetil in the treatment of a 15-year-old female MED patient who achieved significant remission.[5]

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The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.