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Reflectance Confocal Microscopy Features of Degos Disease

Marine Cavalié1; Katerina Tsilika, MD1; Laura Sillard, MD1; Nathalie Cardot-Leccia, MD2; Thierry Passeron, MD, PhD1; Jean-Philippe Lacour, MD1; Philippe Bahadoran, PhD1,3,4
[+] Author Affiliations
1Department of Dermatology, University Hospital of Nice, Nice, France
2Department of Pathology, University Hospital of Nice, Nice, France
3INSERM U1065 C3M Team 1, Nice, France
4Cliniacal Research Center, University Hospital of Nice, Nice, France
JAMA Dermatol. 2014;150(1):96-97. doi:10.1001/jamadermatol.2013.4662.
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Degos disease, otherwise known as malignant atrophic papulosis, is a rare occlusive vasculopathy characterized by pathognomonic cutaneous lesions and frequently fatal systemic involvement. The cause of Degos disease is unknown, and there is currently no effective treatment. Cutaneous lesions of Degos disease have a typical histologic appearance consisting of wedge-shaped necrosis of the dermis. Reflectance confocal microscopy (RCM) is a new in vivo skin imaging technique. The resolution of emerging images is close to that of conventional microscopy (approximately 1 μm), with a penetration depth up to 200 μm allowing the morphologic observation of the normal1 and abnormal dermis.2 Our treatment of a patient with Degos disease prompted us to investigate the RCM features of his skin lesions.

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Figures

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Figure 1.
Cutaneous Examination

Small papules of the trunk with white centers surrounded by erythematous borders.

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Figure 2.
Dermoscopic Image

Telangiectatic ring.

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Figure 3.
Reflectance Confocal Microscopy Features

A, Mosaic image showing the difference between lesional dermis (circle) and perilesional dermis (square) (scale bar indicates 500 μm). B, Detail of the lesional dermis showing highly refractile mass of collagen fibers and loss of capillaries (scale bar indicates 100 μm). C, Detail of perilesional dermis showing normal collagen fibers and increased capillaries (scale bar indicates 100 μm).

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Figure 4.
Histopathologic Features

Wedge-shaped area of necrosis in the dermis (hematoxylin-erythrosin-saffron, original magnification ×25). The dotted line represents the frontier between normal dermis and necrosed dermis.

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