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https://doi.org/10.14300/mnnc.2016.11077

The clinical case of the lipoid necrobiosis in patient with the connective tissue dysplasia

[Notes from practice]
Galina Nechaeva; Olga Drokina; Vitaliy Ochlopkov; Alexey Kononov; Vladimir Konev; Ekaterina Loginova;

Differentiated connective tissue dysplasia (CTD), including Ehlers-Danlos syndrome, Marfan syndrome, etc., is accompanied by diagnostically significant skin disorders. At the same time, interpretation of the pseudo-tumor formations of skin in patients with undifferentiated forms of the CTD (nCTD) is still debated.
We have described a clinical case of lipoid necrobiosis in a patient with the nCTD, the difficulties of differential diagnosis due to atypical clinical picture of skin disease.
The best management of patients with skin disorders and CTD is a multi-disciplinary approach, coordinated efforts of various specialists, including general practitioner, dermatologist, pathologist.

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Keywords: connective tissue dysplasia, lipoid necrobiosis, pseudo-tumor formations of skin