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Background

This is a rare disorder of the connnective tissue. It is an elastic tissue disorder with selective loss of elastic fibres occurring in the mid-dermis. Patients present with fine wrinkling of the epidermis and perifollicular protrusion which gives the skin an aged appearance. Occasionally, a clinical history of a prior inflammatory event such as urticaria may be found but most cases are idiopathic. The cause of the disease is unknown although theories about macrophage activation and UV radiation have been raised.

OUTLINE

Disease Associations  
Pathogenesis  
Laboratory/Radiologic/Other Diagnostic Testing  
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/Immunohistochemistry/Electron Microscopy  
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

 

DISEASE ASSOCIATIONS CHARACTERIZATION
GRANULOMA ANNULARE  

Colocalization of granuloma annulare and mid-dermal elastolysis.

Adams BB, Mutasim DF.

Department of Dermatology, University of Cincinnati College of Medicine, Cincinnati, OH 45267-0592, USA.

J Am Acad Dermatol. 2003 Feb;48(2 Suppl):S25-7 Abstract quote

Mid-dermal elastolysis is an elastic tissue disorder of unknown causation. Various elastic tissue abnormalities have been described in granuloma annulare.

To our knowledge, we report the first case illustrating the clinical and histologic colocalization of granuloma annulare and mid-dermal elastolysis.

HASHIMOTO'S THYROIDITIS  


Mid-dermal elastolysis associated with Hashimoto's thyroiditis.

Gambichler T, Linhart C, Wolter M.

Department of Dermatology, J.W. Goethe University, Frankfurt/Main, Germany.

J Eur Acad Dermatol Venereol. 1999 May;12(3):245-9. Abstract quote

We report the case of a 38-year-old Caucasian female presenting asymptomatic plaques of fine wrinkling and perifollicular papular protrusions especially on the trunk.

Histological examination evidenced loss of elastic fibers in the mid-dermis due to elastophagocytosis, with giant cells and granuloma formation. Moreover, elevated titers of thyroid autoantibodies were detected and thyroid ultrasound revealed echo-poor tissue.

These findings met the diagnoses of mid-dermal elastolysis and Hashimoto's thyroiditis. This association has not been reported before.

We present a comprehensive overview of the literature and discuss the pathogenetic aspects of mid-dermal elastolysis and the significance of the association with Hashimoto's thyroiditis.

 

PATHOGENESIS CHARACTERIZATION
AUTOIMMUNE  


Features of an autoimmune process in mid-dermal elastolysis.

Kirsner RS, Falanga V.

Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, FL 33136.

J Am Acad Dermatol. 1992 Nov;27(5 Pt 2):832-4. Abstract quote

Mid-dermal elastolysis is a rare disorder that is manifested clinically by wrinkling and histologically by the selective absence of elastic fibers in the mid dermis.

We describe a young woman who developed abnormal wrinkling after augmentation mammoplasty with silicone implants. Histologic examination confirmed the diagnosis of mid-dermal elastolysis. In addition, a positive antinuclear antibody titer and a false-positive Lyme titer were subsequently detected. In a previously reported case a patient who had mid-dermal elastolysis also had an unusual false-positive pattern for Lyme disease. Silicone mammoplasty has been associated with connective tissue diseases.

This report adds mid-dermal elastolysis to the list of diseases associated with augmentation mammoplasty and, along with the presence of autoantibodies, supports autoimmune involvement in mid-dermal elastolysis.

ULTRAVIOLET LIGHT  


The clinical spectrum of mid-dermal elastolysis and the role of UV light in its pathogenesis.

Snider RL, Lang PG, Miaze JC.

Department of Dermatology, Medical University of South Carolina, Charleston.

 

J Am Acad Dermatol. 1993 Jun;28(6):938-42 Abstract quote

BACKGROUND: We observed four patients with mid-dermal elastolysis (MDE) that was either precipitated or aggravated by UV light (UVL) exposure or was primarily confined to areas of UVL exposure.

OBJECTIVE: Our purpose was to report four cases of MDE occurring after significant UVL exposure and to demonstrate why we suspect that MDE in some instances may be photoinduced or photoaggravated. We also wish to demonstrate the varied clinical presentation of this disorder.

METHODS: Because all our patients had MDE involving skin exposed to UVL, biopsies were performed on clinically uninvolved sun-exposed and sun-protected skin in one patient to elucidate further the role of UVL in this process.

RESULTS: On routine histopathologic examination we found that uninvolved sun-exposed skin but not sun-protected skin demonstrated early MDE. Ultrastructural examination revealed a spectrum of elastic fiber changes in involved and clinically uninvolved sun-exposed skin. Phagocytosis of elastic fibers was not present.

CONCLUSION: We conclude that UVL exposure was a major causative or aggravating factor of MDE in our patients and that the clinical appearance of this disorder is variable.

 

LABORATORY/RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  


Mid-dermal elastolysis.

Streams BN, Williams JM, Moschella SM.

Department of Dermatology, Lahey Clinic, Harvard Medical School, Burlington, Massachusetts, USA

Cutis. 2003 Apr;71(4):312-4. Abstract quote

Mid-dermal elastolysis (MDE), which presents as fine wrinkling of the skin or perifollicular papules, is extremely rare. This entity is distinguished from other elastolytic disorders by its characteristic bandlike loss of elastic fibers limited to the mid dermis.

We report a case of MDE that developed gradually in an otherwise healthy woman without prior cutaneous disease. The current theories on the pathogenesis of MDE also are discussed.


Wrinkling due to mid-dermal elastolysis: two cases and literature review.

Rao BK, Endzweig CH, Kagen MH, Kriegel D, Freeman RG.

Department of Dermatology, New York Presbyterian Hospital-Cornell University Medical College, New York, NY, 10021, USA.

J Cutan Med Surg. 2000 Jan;4(1):40-4. Abstract quote

BACKGROUND: Mid-dermal elastolysis is an acquired disorder of elastic tissue clinically characterized by diffuse fine wrinkling, most often of the trunk and arms. Histologically, a clear band of elastolysis is present in the mid-dermis.

OBJECTIVE: Although examples of diffuse elastolysis are well known, only a small number of patients with mid-dermal elastolysis have been reported to date. We present two patients with clinical and histological evidence of mid-dermal elastolysis, review the literature, and summarize the salient features of some common disorders of elastic tissue.

METHODS: The first patient presented with fine wrinkles and papules over the upper arms, upper chest, and axillae, and demonstrated increased laxity of the eyelids. The second patient had striking wrinkles extending in a band-like pattern on her arms, upper chest, back, and abdomen. Neither one of our patients had a previous history of skin inflammation, urticaria, or any other underlying diseases related to their skin changes. Skin biopsies were taken from lesional and perilesional skin of both patients, and were stained with hematoxylin and eosin, and with elastic tissue stain. In addition, a tissue sample from Patient 1 was fixed for electron-microscopy.

RESULTS: Hematoxylin and eosin stains did not demonstrate specific changes or diagnostic patterns. However, elastic tissue stains revealed a band-like loss of elastic tissue in the mid-dermis. Elastic tissue in the remaining superficial and deep dermis stained normally. Electron-microscopy was consistent with these findings and revealed significant loss of elastic tissue limited to the mid-dermis.

CONCLUSION: We have presented two cases of mid-dermal elastolysis and reviewed the literature. To date, the pathophysiology of mid-dermal elastolysis had not been elucidated and no definitive therapy exists.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL Loss of elastic fibers in the mid-dermis due to elastophagocytosis, with giant cells and granuloma formation

Mid-dermal elastolysis preceded by acute neutrophilic dermatosis.

Lewis KG, Dill SW, Wilkel CS, Robinson-Bostom L.

Department of Dermatology, Brown Medical School, Rhode Island Hospital, Providence, RI, USA, Departments of Dermatology and Pathology, Brown Medical School and Boston University Medical Center/Roger Williams Medical Center, Providence, RI, USA and Department of Pathology, Brown Medical School, Rhode Island Hospital, Providence, RI, USA.
J Cutan Pathol. 2004 Jan;31(1):72-76 Abstract quote.  


BACKGROUND: Mid-dermal elastolysis is a rare idiopathic elastic tissue disorder that is characterized by localized patches of finely wrinkled skin and a 'band-like' loss of elastic tissue in the mid-reticular dermis. Lesions may be preceded by erythema and/or urticaria, and histological examination of inflamed lesional skin may demonstrate lymphohistiocytic dermal infiltration.

CASE REPORT: We report a case of mid-dermal elastolysis in a 31-year-old woman who developed multiple erythematous and urticarial plaques on the arms and trunk. Histologic examination of a representative lesion revealed a neutrophilic infiltrate and a normal pattern of elastic tissue. Several months later, the erythema and urticaria was noted to have resolved, leaving soft, pendulous plaques with overlying finely wrinkled skin. A follow-up biopsy at this time showed minimal lymphocytic inflammation but almost complete absence of elastic tissue in the mid-reticular dermis.

CONCLUSIONS: To our knowledge, acute neutrophilic dermatosis resulting in mid-dermal elastolysis has not been previously described. This observation lends support to an emerging theory that the pathogenesis of mid-dermal elastolysis may be inflammatory.


Mid-dermal elastolysis: a pathological and ultrastructural study of five cases.

Neri I, Patrizi A, Fanti PA, Passarini B, Badiali-De Giorgi L, Varotti C.

Department of Dermatology, University of Bologna, Italy.


J Cutan Pathol. 1996 Apr;23(2):165-9. Abstract quote

The aim of this study was to evaluate the presence of inflammatory phenomena and elastic fiber phagocytosis in mid-dermal elastolysis.

The pathological and ultrastructural features of 5 Caucasian female patients (ranging from 26 to 40 years) with acquired diffuse asymptomatic areas of skin wrinkling have been reviewed. The clinical features of all cases were characteristic of this condition and only in one patient were erythematous urticaria-like, non pruriginous patches also observed. In 4 cases a history of prolonged sun bathing was present and in 3 cases there was a short history of oral contraception.

The pathological study confirmed the typical absence of elastic fibers in the midreticular dermis. In two cases elastic fibers were still detectable in the periadnexal dermis. Hematoxylin and eosin sections showed a mild perivascular infiltrate in two cases, while in three patients histiocytes were scattered among collagen bundles. Multinucleated giant cells containing fragmented elastic fibers were detectable in one patient.

Ultrastructural analysis revealed large mononuclear cells with phagocytic aspects toward elastic fibers in all cases.

Mid dermal elastolysis: case report and review of the literature.

Agha A, Hashimoto K, Mahon M.

Department of Dermatology & Syphilology, Wayne State University School of Medicine, Detroit, MI 48201.

J Dermatol. 1994 Oct;21(10):760-6. Abstract quote

A new case of typical mid dermal elastolysis was studied with electron microscopy. Elastic fibers were irregularly branched or lumpy, but subcomponents of these fibers were often normal.

In some fibers, loose assembly of skeleton fibrils and aggregation of dense substances were observed.

Phagocytosis of normal and abnormal elastic fibers by dermal macrophages was observed.

 

SPECIAL STAINS/IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  


Mid-dermal elastolysis: A clinical, histologic, and immunohistochemical study of 11 patients.

Patroi I, Annessi G, Girolomoni G.

Istituto Dermopatico dell'Immacolata, Istituto di vicerero e cura a carrattere scientifico.

 

J Am Acad Dermatol. 2003 Jun;48(6):846-51 Abstract quote

BACKGROUND: Mid-dermal elastolysis is a rare entity defined by the selective loss of elastic tissue in the mid dermis. Many cases appear induced or aggravated by ultraviolet (UV) light exposure. Pathogenesis is still uncertain.

OBJECTIVE: Our purpose was to report on the clinical and histologic features of 11 patients with mid-dermal elastolysis. Moreover, we analyzed by immunohistochemistry leukocyte subsets and expression of metalloproteinase (MMP) with the potential to degrade elastic tissue in 7 cases.

RESULTS: All patients were women with a mean age of 31.4 years. Disease duration ranged from 4 months to 17 years. Affected areas included the trunk, neck, and upper aspect of limbs. Two patients also had Hashimoto's thyroiditis and uterine carcinoma, respectively, whereas 1 patient had undergone silicone mammoplasty. In all patients, disease onset was associated with intense UV light exposure. Moderate leukocyte infiltration in the dermis was observed mostly in recent lesions and was composed of CD3(+) T cells and some CD68(+) macrophages with a normal number of factor XIIIa(+) dermal dendritic cells. Elastin, but not fibrillin-1 immunoreactivity disappeared from the mid dermis. MMP-9 was detected in epidermal keratinocytes and in the cytoplasm of large, angulated, multinucleated cells located in lesional dermis. These cells were negative for leukocyte, dendritic cell, macrophage, and T-cell markers and were absent in old lesions. Staining for MMP-7 and MMP-12 did not differ from control skin.

CONCLUSION: Onset of mid-dermal elastolysis appears strongly associated with UV exposure, which may induce fibroblast-like cells to express MMP-9 that in turn could be involved in the degradation of elastic fibers.

ELECTRON MICROSCOPY  


Mid-dermal elastolysis: an ultrastructural and biochemical study.

Fimiani M, Mazzatenta C, Alessandrini C, Paola M, Paola C, Andreassi L.

Istituto di Dermatologia e Venereologia, Universita degli Studi di Siena, Policlinico le Scotte, Italy.

 

Arch Dermatol Res. 1995;287(2):152-7. Abstract quote

Mid-dermal elastolysis (MDE) is a particular elastic tissue disorder in which selective loss of elastic fibres occurs in the mid-dermis. It is clinically characterized by the appearance of fine wrinkling of the epidermis and perifollicular protrusion which gives the skin an aged appearance. It is sometimes associated with an inflammatory event such as urticaria while other cases are regarded as idiopathic. The pathogenesis of MDE is still obscure. Some authors have underlined the role of macrophage activation and others have imputed UV radiation.

We report here a typical case of MDE arising after several attacks of solar urticaria. Electron microscopic and biochemical studies were carried out. Ultrastructural examination showed active elastophagocytosis by macrophages and mast cells, often degranulated, near phagocytosing cells. Biochemical studies demonstrated that fibroblasts derived from lesional skin of the MDE patient produced high levels of elastase and cathepsin G compared with fibroblasts from a healthy sex- and age-matched control. Phagocytosis of morphologically normal elastic tissue is a noticeable characteristic feature of MDE.

In our case mast-cell activation and the abnormal synthesis and/or release of fibroblast elastolytic enzymes seemed to play a role in the pathogenesis of the MDE.

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
UPPER DERMAL ELASTOLYSIS  


Upper dermal elastolysis: a comparative study with mid-dermal elastolysis.

Hashimoto K, Tye MJ.

Department of Dermatology and Syphilology Wayne State University School of Medicine, Detroit, MI 48201.

J Cutan Pathol. 1994 Dec;21(6):533-40. Abstract quote

We encountered a patient who complained of many small papules on the neck, shoulders, upper chest and upper back. Biopsy specimens showed complete loss of elastic fibers in the upper dermis including papillary dermis, whereas those of the mid dermis were intact.

Electron microscopy revealed that assembly of component fibrils of elastic fibers was loose, and electron dense substance was aggregated in the spaces between these loosely bound subunit fibrils or along the periphery of abnormal fibers. Dermal phagocytes engulfed abnormal as well as normal elastic fibers.

Upper dermal elastolysis is a clinical and histopathological entity different from mid-dermal elastolysis. Ultrastructural changes of the former are essentially similar to those of the latter but much more severe. It is suggested that activated elastophagocytosis of dermal phagocytes may play a role in this disease.

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Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


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Last Updated 2/2/2004

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