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Fig. 2 | Orphanet Journal of Rare Diseases

Fig. 2

From: Gastrointestinal Kohlmeier–Degos disease: a narrative review

Fig. 2

Chronologic pathology of cutaneous K-D as occurs in the microvasculature of the dermis: A Early inflammatory phase characterized by a vasocentric lymphocytic infiltrate permeative of the vessel wall with variable fibrin deposition and red cell extravasation consistent with a lymphocytic vasculitis (H&E, ×40). B Due to the targeted endothelial cell injury there is endothelial cell necrosis, subsequent denudement followed by vascular thrombosis with some inflammatory residuum eventuating into a relatively pauci-inflammatory thrombogenic vasculopathy (H&E, ×40). C Repetitive episodes of endothelial cell injury cause basement membrane zone thickening while the lining endothelium has a mummified anucleated appearance (H&E, ×40). D Eventually the vessels disappear and there is ensuing ischemic driven fibrosis corresponding clinically to the depressed porcelain white center (H&E, ×4). E Enhanced interferon expression as demonstrated by the extent of myxovirus A (MXA) deposition within the vessel wall, amidst inflammatory cells and endothelium (MXA, ×40). F There are extensive microvascular deposits of C5b-9 (C5b-9, ×10)

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