Are You Confident of the Diagnosis?
Characteristic findings on physical examination
Mid-dermal elastolysis (MDE), first described by Shelley & Wood in 1977, is a skin condition of the elastic tissue predominantly manifesting on the trunk and proximal extremities. Most commonly observed skin changes include patches of well-circumscribed fine wrinkles (type I) and/or perifollicular papular protusions (type II, Figure 1). The disease may also occur with persistent reticular erythema and fine wrinkles (type III).
Expected results of diagnostic studies
The critical histopathologic feature of MDE is the selective loss of elastic fibers in the mid-dermis. Further histopathologic findings of MDE include mild dermal lymphohistiocytic infiltrates, elastophagocytosis of elastic fibers, and occasionally multinucleate giant cells. Special stains that accentuate elastic fibers may be helpful in making the diagnosis (Figure 2).
Differential diagnoses include anetoderma, annular elastolytic giant cell granuloma, cutis laxa, and pseudoxanthoma elasticum-like papillary dermal elastolysis.
Who is at Risk for Developing this Disease?
MDE is a rare acquired skin condition. Up to 2010, about 80 cases of MDE have been reported in the medical literature. MDE usually affects Caucasian females at a median age of about 40 years. Pregnancy, intake of contraceptives, smoking and increased ultraviolet exposure have frequently been associated with the occurrence of MDE. Preexisting and/or associated skin conditions such as urticaria, atopic dermatitis, granuloma annulare, and phototoxic dermatitis have been described in MDE patients.
What is the Cause of the Disease?
The precise pathogenesis of MDE is unclear. Increased activity of elastases (matrix metalloproteinases, cathepsin G etc.) seems to result in elastin degradation. An altered elastin re-synthesis system may also be a causative factor of permanent elastic fiber loss in MDE.2-5
Systemic Implications and Complications
MDE was observed to be associated with systemic conditions such as Hashimoto’s thyroiditis, Graves disease, lupus erythematosus, Sweet syndrome and autoimmune phenomena (eg, elevated antinuclear antibodies). However, these conditions are likely coincidental in MDE.
There are no proved treatment options for patients with MDE. Anecdotically, topical tretinoin or dapsone led to some improvement of MDE.
Optimal Therapeutic Approach for this Disease
So far, there is no optimal therapeutic approach for patients with MDE. Because of the likely coincidental association with other autoimmune conditions, a specific workup for these disorders (autoimmune thyroid disease, lupus, etc.) is not necessary unless there are other clinical features of these disorders.
The patient should be informed that MDE is noninherited and of benign nature, though the condition may cause significant cosmetic and psychological impairment. The condition usually takes a chronic course. Ultraviolet overexposure and smoking should be avoided. Consultation with a psychologist and or psychiatrist may help with psychosocial issues.
Unusual Clinical Scenarios to Consider in Patient Management
MDE has been observed to be associated with systemic conditions such as Hashimoto’s thyroiditis, Graves’ disease, lupus erythematosus, Sweet syndrome and autoimmune phenomena (eg, elevated antinuclear antibodies). However, these conditions are likely coincidental in MDE.
What is the Evidence?
Shelley, WB, Wood, MG. “Wrinkles due to idiopathic loss of mid-dermal elastic tissue”. Br J Dermatol. vol. 97. 1977. pp. 441-5. (First report on MDE in the world literature.)
Gambichler, T. “Mid-dermal elastolysis revisited”. Arch Dermatol Res. vol. 302. 2010. pp. 85-93. (Comprehensive analysis of MDE cases previously reported in the literature.)
Patroi, I, Annessi, G, Girolomoni, G. “Mid-dermal elastolysis: a clinical, histologic, and immunohistochemical study of 11 patients”. J Am Acad Dermatol. vol. 48. 2003. pp. 846-51. (So far, largest case series of MDE patients, including immunohistological studies.)
Gambichler, T, Stücker, M, Kreuter, A, Matip, R, Gaifullina, R, Scola, N. “Expression of extracellular matrix proteins in reticular variant of mid-dermal elastolysis”. J Eur Acad Dermatol Venereol. vol. 24. 2010. pp. 1481-84. (Extensive sudies on a patient with MDE using immunohistochemistry and real-time RT-PCR.)
Lewis, KG, Bercovitch, L, Dill, SW, Robinson-Bostom, L. “Acquired disorders of elastic tissue: part II”. decreased elastic tissue. J Am Acad Dermatol. vol. 51. 2004. pp. 165-85. (Comprehensive review on acquired diseases of the elastic tissue characterized by loss of elastic fibers such as MDE, anetoderma, annular elastolytic giant cell granuloma etc.)
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