Forum for Nordic Dermato-Venereology Nr 1, 2018 | Page 5

Educational Review Livedoid Vasculopathy N icolas K luger , R egional E ditor , F inland Helsinki University Central Hospital and University of Helsinki, Dermatology, Allergology and Ve- nereology, FI-00029 Helsinki, Finland. E-mail: [email protected] Livedoid vasculopathy (LV) is a thrombo-occlusive cutaneous disease of the dermal vessels with pauci-inflammatory or non-inflammatory histopathology findings (1). LV was first described by Millian in 1929 (2) under the term atrophie blanche en plaques and then better characterized by Bard & Winkelman in 1967 (3). It has been reported in the literature under various terms that make it difficult to recognize: livedoid vasculitis, PURPLE (painful purpuric ulcers with reticular pattern of the lower extremities) (1), idiopathic atrophie blanche (4), segmental hyalinizing vasculitis, and livedo reticularis with summer ulcerations (5). The current official denomination is livedoid vasculopathy (6). The term atrophie blanche (AB) should be used only to describe the typical smooth, ivory-white plaque-like area on the lower extremities surrounded by hyper- pigmented border and telangiectatic blood vessels (6). However, AB may be associated with conditions other than LV (6). LV is characterized by chronic and extremely painful ulcerations of the feet and legs in relation to a vaso-occlusive phenomenon due to intraluminal thrombosis of the dermal venules. Primary (“idiopathic”) and secondary forms of LV are distinguished. Epidemiology LV has an estimated incidence of 1/100,000 inhabitants/year. LV is typically a disease of the young. Patients are mainly aged between 15 and 50 years, and women are particularly affected, with a female to male ratio of 3:1 (1). Clinical manifestations The presentation of LV is quite typical, and diagnosis can be made by the dermatologist. A telangiectatic purpura leads to asymmetrical, irregular, painful, “punched-out” and recurrent ulcers of the foot, ankles and lower legs, with a slow healing and AB scarring, defined as white smooth ivory- or porcelain scars, surrounded by telangiectasia and livedoid brownish hyperpigmentation (Fig. 1a, b) (1). The lesions are usually bilateral. Active lesions are purpura, bruises, and painful ulcers, while inactive disease corresponds to scars, hyperpigmenta- tion, and AB (Fig. 1c, d) (5). Of note, livedo racemosa (defined as a livedo with opened meches) reminiscent of Sneddon’s syndrome can be seen in patients with LV (Fig. 2). This can be located on the lower or upper limbs. Finally, mononeuropathy may occur in relation to thrombosis of the vasa vasorum (1). LV is exceedingly painful and the impact on quality of life is significant (7). Histopathology The biopsy must include dermo-hypodermis to rule out cutaneous polyarteritis nodosa (PAN). Where possible, mul- tiple biopsies are recommended, and they should avoid the immediate margins of the ulcers (granulation tissue, inflam- mation secondary to tissue repair). The main characteristics are the occlusion of dermal blood vessels by fibrin deposition Fig. 1. (A) Active lesions of livedoid vasculopathy (LV): punched out ulcers of the external side of the ankle, inflammation and purpura. Atrophie blanche (AB) on the upper part with white ivory scars and hyperpigmentation on the border. (B) Active flare of LV on the dorsum of the foot in an 88-year-old woman with activated protein C resistance. (C) Painful ulcers in a 44-year-old woman: punched-out ulcers, inflammation, small AB scars and lack of chronic venous insufficiency. (D) AB scars on both lower legs in a 24-year-old woman with no hypercoagulable condition. Forum for Nord Derm Ven 2018, Vol. 23, No. 1 3