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Dermoscopy of early non-ulcerated livedoid vasculopathy.

Introduction

Livedoid vasculopathy is an uncommon disease and is a purely cutaneous form of ischemia. The disease is particularly painful due to vascular occlusion, which commonly progresses to ulceration and subsequently heals slowly over weeks or months. It does not show a complete clearing of the lesion, but gives rise to pearly atrophic scars (white atrophy), punctate telangiectasia, and brownish pigmentation accompanied by a racemous livedo (1).

The disease can be accompanied by atrophie blanche (AB), a porcelain-white scar that may be seen at the base of a healed ulcer. Livedoid vasculopathy is responsible for about 1% of leg ulcers (2). The lower legs, in particular the dorsum of the feet and ankles, are most often affected.

The disease is more common in patients over 45. Venous stasis can also be associated with this disease. Other comorbidities are arterial hypertension and treatment with anticoagulants. A recent study in Germany estimated the percentage of leg ulcers in those patients to be as high as 14.3% (3).

Patients and methods

We report the use of dermoscopy to further characterize such lesions in patients with early, non-ulcerated livedoid vasculopathy without clotting disorders. The duration of these complaints was between 6 and 12 months.

Dermoscopic examination was performed using a DermLite camera with polarized light (10x magnification; 3Gen, Dana Point, CA). The DermLite cam contains polarization filters, and therefore immersion liquids do not need to be applied during skin examination.

Five consecutive patients (four females and one male between 43 and 50 years old) with painful livedoid vasculopathy of the ankles without ulcerations and without venous insufficiency were evaluated.

Results

Although our patients had no ulceration, all five showed variable amounts of ivory-white areas surrounded by erythematous lesions. The erythematous lesions had glomerular-like vessels in all five patients (Figs. 1 and 2). In three patients, some telangiectatic linear vessels were noted in the periphery of the lesions (Fig. 2). Pigmentary changes were completely absent.

Discussion

Our study has two major limitations: a) the small number of patients and b) the lack of histology. Nevertheless, it is of interest because there is not much information available on this subject. Here we present dermoscopic features of early livedoid vasculopathy without ulcerations. There is only one other study by Hu et al. reporting on the use of dermoscopy in livedoid vasculopathy. They investigated nine female patients with ulcerated livedoid vasculopathy (4). The ivory-white atrophic scar-like areas they noted were also seen in our patients. Because they had no history of ulceration, ulceration does not seem to be a conditio sine qua non. We suggest that these ivory-white lesions are a result of tissue fibrosis. Hu et al. observed some hyperpigmentation in a reticular pattern in the periphery of the lesions (4). This seems to be a secondary change either due to ulceration with pronounced inflammation or due to concomitant venous insufficiency. In our study, hyperpigmentation was not visible. The major finding in our study and in the study by Hu et al. was the formation of glomerular vessels (4). We also observed some telangiectatic linear vessels in the periphery of the lesions as described elsewhere (4). We suggest that these vascular formations might be later symptoms.

Glomerular vessels are not specific to livedoid vasculopathy. They have been described in chronic sun-damaged skin with precancerous lesions (5-7), in the periphery of hyperkeratotic Bowen's disease (8) and basal cell carcinoma (9), in poromas (10), in psoriasis (11), and in cutaneous leishmaniasis (12, 13). In contrast to other tumorous or non-tumorous cutaneous lesions, the glomerular vessels in early livedoid vasculopathy show a more regular and even distribution over the entire lesion, which might be a more characteristic feature.

Livedoid vasculopathy is caused by a hypercoagulable state, leading to obstruction of small dermal blood vessels by intraluminal thrombi. In addition, the platelets may be characterized by increased aggregation ability (Table 1). The histopathology of livedoid vasculopathy is characterized by intraluminal thrombosis, proliferation of the endothelium, and segmental hyalinization of dermal vessels. Treatment of choice is with oral rivaroxaban (14).

References

(1.) Kerk N, Goerge T. Livedoid vasculopathy--current aspects of diagnosis and treatment of cutaneous infarction. J Dtsch Dermatol Ges. 2013;11:407-10.

(2.) Jockenhofer F, Gollnick H, Herberger K, Isbary G, Renner R, Stucker M, et al. Aetiology, comorbidities and cofactors of chronic leg ulcers: retrospective evaluation of 1000 patients from 10 specialised dermatological wound care centers in Germany. Int Wound J. 2016;13:821-8.

(3.) Renner R, Dissemond J, Goerge T, Hoff N, Kroger K, Erfurt-Berge C. Analysis of the German DRG data for livedoid vasculopathy and calciphylaxis. J Eur Acad Dermatol Venereol. 2017;31:1884-9.

(4.) HuSC, Chen GS, Lin CL, Cheng YC, Lin YS. Dermoscopic features of livedoid vasculopathy. Medicine (Baltimore). 2017:96:66284.

(5.) Zalaudek I, Giacomel J, Schmid K, Bondino S, Rosendahl C, Cavicchini S, et al. Dermatoscopy of facial actinic keratosis, intraepidermal carcinoma, and invasive squamous cell carcinoma: a progression model. J Am Acad Dermatol. 2012; 66:589-97.

(6.) Warszawik-Hendzel 0, Olszewska M, Maj M, Rakowska A, Czuwara J, Rudnicka L. Non-invasive diagnostic techniques in the diagnosis of squamous cell carcinoma. J Dermatol Case Rep. 2015;9:89-97.

(7.) Manfredini M, Longo C, Ferrari B, Piana S, Benati E, Casari A, et al. Dermoscopic and reflectance confocal microscopy features of cutaneous squamous cell carcinoma. J Eur Acad Dermatol Venereol. 2017;31:1828-33.

(8.) Payapvipapong K, Tanaka M. Corona of glomerular vessels: a diagnostic marker of hyperkeratotic Bowen's disease. Dermatol Surg. 2013;39:1395-8.

(9.) MicantonioT, GuliaA, Altobelli E, Di CesareA, Fidanza R, Riitano A, etal. Vascular patterns in basal cell carcinoma. J Eur Acad Dermatol Venereol. 2011;25:358-61.

(10.) Minagawa A, Koga H. Dermoscopy of pigmented poromas. Dermatology. 2010; 221:78-83.

(11.) Kim GW, Jung HJ, Ko HC, Kim MB, Lee WJ, Lee SJ, et al. Dermoscopy can be useful in differentiating scalp psoriasis from seborrhoeic dermatitis. Br J Dermatol. 2011;164:652-6.

(12.) Llambrich A, Zaballos P, Terrasa F, Torne I, Puig S, Malvehy J. Dermoscopy of cutaneous leishmaniasis. Br J Dermatol. 2009;160:756-61.

(13.) Dobrev HP, Nocheva DG, Vuchev DI, Grancharova RD. Cutaneous leishmaniasis--dermoscopic findings and cryotherapy. Folia Med (Plovdiv). 2015;57:65-8.

(14.) Weishaupt C, Strolin A, Kahle B, Kreuter A, Schneider SW, Gerss J, et al. Anticoagulation with rivaroxaban for livedoid vasculopathy (RILIVA): a multicentre, single-arm, open-label, phase 2a, proof-of-concept trial. Lancet Haematol. 2016;3:e72-9.

Uwe Wollina (1[??])

(1) Department of Dermatology and Allergology, Dresden Academic Teaching Hospital, Dresden, Germany. ([??]) Corresponding author: wollina-uw@khdf.de

Received: 1 February 2018 | Returned for modification: 26 February 2018 | Accepted: 16 March 2018

doi: 10.15570/actaapa.2018.29
Table 1 | Important procoagulant factors in livedoid vasculopathy.

Factor                     Description

Protein C deficiency       Protein C is a vitamin K-dependent
                           anticoagulant protein that inactivates
                           coagulation factors Va and Villa, and its
                           deficiency (< 55%) results in a thrombophilic
                           state.
Protein S deficiency       Protein S is a cofactor of protein C and
                           inactivates the coagulation factors Va and
                           Villa; type 1 and type 3 are known as
                           quantitative defects, whereas type 2 is a
                           qualitative defect that has been
                           infrequently observed.
Factor V Leiden (FVL)      FVL thrombophilia is caused by a mutation
mutation                   in the gene for factor V(F5G1691A mutation
                           resulting in the amino acid substitution
                           FVR506Q).
Antithrombin (AT) III      Hereditary AT deficiency is rare; the
deficiency                 autosomal dominant trait typically reduces
                           functional AT levels to 40 to 60% of normal
Prothrombin gene mutation  Prothrombin G20210A mutation.
Hyperhomocysteinemia       May occur isolated, but is also a feature of
                           inherited metabolic disorders, including
                           homocystinuria, due to mutation in the CBS
                           gene, and N(5,10)-methylenetetrahydrofolate
                           reductase deficiency, caused by mutation in
                           the MTHFR gene (607093); homocysteinemia/
                           homocystinuria and megaloblastic anemia can
                           result from defects in vitamin B12
                           (cobalamin; cbl) metabolism.
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Author:Wollina, Uwe
Publication:Acta Dermatovenerologica Alpina, Pannonica et Adriatica
Date:Jul 1, 2018
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