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Mid-dermal elastolysis termed a female-centric condition

Mid-dermal elastolysis (MDE), while rare, has been determined to be a female-centric disease, according to a case report and updated review of the literature published in the International Journal of Women’s Dermatology (Aug. 2015; 1(3):126–130).

A 45-year-old female was referred to Dr. Richard M.Haber, with the Division of Dermatology at the University of Calgary, for a second opinion regarding what was initially thought to be a case of generalized atrophoderma by the referring dermatologist. The patient had widespread papules and plaques with wrinkling and mild protrusion from the surface of the skin predominantly over her abdomen, trunk, and proximal arms and legs.

The patient’s past medical history was unremarkable (of note, she was nulliparous), and she had no recent travel. She was active outdoors and often exercised without sun protection. Dr. Haber diagnosed MDE, and became interested in opportunities to further investigate this condition.

“I have seen the occasional cases [of MDE] presented at meetings, but it is the first one that [my colleagues and I] have diagnosed,” said Dr. Haber. “We thought it was a bit unusual and we thought we would perhaps look at the fact that it more common in females—it has been reported before, but it has not been the emphasis of cases in the past,” hes aid.

Possible role of UV

German dermatologist Dr. Thilo Gambicheler reported an 83.5% female prevalence in his review of MDE (Arch Dermatol Res 2010;302(2):85–93). “Interestingly, of the publications since 2009, six of the 10 were reports of Type III MDE. Unlike Type I and Type II MDE, type III demonstrates a strong male predominance,with only three reported cases affecting females,” the authors state in the report.

“Therefore if the six recent reports of Type III MDE are removed, three of the four cases of Type I and II MDE since 2009 have been females.”

The authors report that while ultraviolet radiation or hormonal roles remain elusive in MDE, “they could potentially explain the unequivocal proportion of female patients, as well as the significant history of sun tanning bed exposure amongst them.” The investigators note that in almost half of the cases reported sun exposure was implicated.

“It is unusual that [the condition could be] sun-induced because it rarely affects the face, ” said Dr. Haber. “However, there are other sun-induced conditions where the face is spared, for example . . . polymorphous light eruption [in which the face] may be involved, but it may be spared. Therefore [this feature] does not preclude [MDE] from beings un-induced.”

No effective treatment

Dr. Haber said that there is currently no evidence for effective treatments. According to the literature, topical tretinoin is most widely used and while there is some evidence that it reduces the winkles, it does not alter the natural history of the disease. The use of tretinoin has been found to be more effective in more mild cases.

“Other patients, where they have signs of inflammation, there have been reports of using anti-inflammatory drugs to prevent this from [developing],”said Dr.Haber.“However, most of the time you do not see a lot of inflammation in this condition. [If the patient] presents with redness and itching, it is in this situation that treatment with anti-inflammatory agents—such as dapsone—has been used.”

The authors note that there is a potential role for soybean extract in enhancing elastic fiber network and inhibition of elastases, though it has so far only been tested on mice.

“[My case] patient just wanted to know what she had. She had been to numerous doctors in the past and was not diagnosed,” said Dr. Haber. “She was certainly happy to have at least a diagnosis and she was able to go read about it . . . That is always a good thing, but having a diagnosis unfortunately does not mean that we can actually . . . treat it, which is very frustrating in a number of cases.”

Psychosocial implications Dr. Haber said it was important to be able to reassure his patient that the condition was unlikely to appear on the face, since the cosmetic implications of MDE can be life-altering for patients.

The researchers noted that clinicians should not diminish the severity of a diagnosis because there are no systemic manifestations. They noted that clinicians should not neglect the psychosocial impact of this diagnosis.

“We are hoping just by making clinicians more aware of this condition then perhaps they will recognize [MDE] and hopefully we can get a series of these patients. [Up until now there have only] been isolated cases, so it has been almost impossible to do any evidence-based trial for any type of therapies,” said Dr. Haber.

“And there are occasionally serendipitous [incidents] where patients have taken a drug for other reasons and the condition gets better.”

Greater awareness of MDE, according to Dr. Haber, is critical because the clinician must know to prompt the pathologist to pursue orcein or van Gieson staining.

“Biopsies sent for hematoxylin and eosin staining are commonly reported as normal,” Dr. Haber pointed out.

Article original appeared in The Chronicle of Skin & Allergy (Feb. 2016; 22(1):1,10)

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