Livedoid Vasculopathy with Severe Debilitating Neuropathy in a Prior Professional Athlete.

Livedoid vasculopathy (LV) can be a challenging diagnosis with an interesting pathophysiology. LV is an uncommon diagnosis that can be easily mistaken for more common skin conditions, especially in a person of color who may be underrepresented in pathology images used in medical education. LV has an average of five years from initial presentation to diagnosis, possibly due to providers not having it on their differential for lower extremity ulcerations. Prolonged time to diagnosis can potentially lead to life-changing complications. We present a case of a former professional sprinter who became debilitated by neuropathy secondary to complications from LV. He was seen multiple times and had an extensive work-up exploring a broad differential including autoimmune etiologies, hypercoagulable disorders, neuropathies, and other vascular disorders before reaching the diagnosis. This case emphasizes the importance of early diagnosis and treatment with a multidisciplinary team to help prevent the progression of these symptoms. We break down an extensive work-up that involves a multidisciplinary team including dermatology, hematology, neurology, rheumatology, and vascular surgery. This case will also highlight examples of LV in a patient with a dark skin complexion, which can be challenging to find in current literature. We additionally show images that demonstrate many of the classic pathologic findings associated with LV and how those can help lead to the diagnosis along with detailed descriptions of those findings. Classic physical exam findings including atrophic blanche and lower extremity ulcerations are highlighted. We also review LV's history, diagnosis, and treatment to help readers achieve a better understanding of the disease.

Punch grafting for the treatment of ulcerated atrophie blanche.

Case report: A 79-year-old woman presented with a large painful ulcer on the lateral aspect of her left leg over a 6-month period and was diagnosed of ulcerated atrophie blanche. On an outpatient basis punch grafting was performed and 3 weeks after, complete epithelization was achieved. Discussion: Ulcerated atrophie blanche is a misdiagnosed disorder with painful lesions and, consequently, a high impact on quality of life. Atrophie blanche describes porcelain-white colored, red-dotted atrophic plaques on legs or feet. It may be due to multiple causes, usually associated with alterations in the microcirculation. All causes of atrophie blanche can be included in the term livedoid vasculopathy, a type of occlusive vasculopathy without vasculitis. Many patients with atrophie blanche and livedoid vasculopathy have also chronic venous insufficiency. Etiological treatment should be prescribed in order to avoid progression of the lesions. In case of chronic venous insufficiency, control of venous hypertension is essential. Without anti-edema measures, superficial, very painful, and resistant ulcers may appear. These ulcers can be considered a wound on scar tissue; therefore, it must be treated as a hard-to-heal wound. As we show in this case, punch grafting is an effective therapeutic alternative for wound closure and pain reduction of ulcerated atrophie blanche.

Adalimumab in the Treatment of Recalcitrant Livedoid Vasculopathy.

Livedoid vasculopathy is a rare condition affecting the cutaneous vasculature. Patients typically develop bilateral lower limb ulcers that tend to recur and do not heal. Edema, discomfort, and itching are linked to ulcers. The patient's quality of life is negatively impacted by this. Atrophie blanche, a stellate, porcelain-white scar, is typically left behind once these ulcers heal. Livedoid vasculitis, livedo reticularis with ulcerations, atrophie blanche, segmental hyalinizing vasculitis, and painful purpuric ulcers with a reticular pattern on the lower limbs are some of the terminologies used to describe livedoid vasculopathy. This condition has been treated using various techniques, including intravenous immunoglobulins, steroids, anticoagulants, antibiotics, immunosuppressive drugs, and antiplatelets. Here, we report the case of a 24-year-old male who presented with a red-colored, painful, and itchy lesion over his right calf for one year. He had recurring lesions over his right foot for the past 18 years. He had received multiple treatment courses over 18 years but had no relief. He was treated with eight doses of adalimumab injection (40 mg/0.8 mL) administered subcutaneously at an interval of 15 days. He had near-complete healing of the ulcer and complete remission of symptoms.

A comprehensive review on pathogenesis, associations, clinical findings, and treatment of livedoid vasculopathy.

Livedoid vasculopathy (LV) is a thrombo-occlusive vasculopathy that involves the dermal vessels. Clinically, it is characterized by the presence of painful purpuric ulcers on the lower extremities. Histopathologically, it shows intraluminal fibrin deposition and thrombosis, segmental hyalinization, and endothelial proliferation. It is important to notice that the term "atrophie blanche" is descriptive and it includes not only patients with LV but also patients with a combination of vasculitis and vasculopathy, that is, LV and medium-sized vasculitis such as cutaneous polyarteritis nodosa (PANc). Diagnosis is based on a proper clinicopathological correlation, excluding the main differential diagnosis and considering vasculitis as a mimicker or concomitant diagnosis. Coagulation disorders must also be studied although they are not found in all LV. Its frequency is reviewed as well. Treatment of LV is challenging, and different therapies have been attempted. Among them, pain management, wound care, control of cardiovascular risk factors, and both antiplatelets and anticoagulants, mostly rivaroxaban, are the main therapies used. These different therapies as well as their degree of evidence are reviewed.

Clinical, Laboratory, Histopathological and Therapeutic Profile of Livedoid Vasculopathy: A Case Series of 17 Patients.

Livedoid vasculopathy is a rare disorder clinically presenting with triad of livedo reticularis, leg ulcerations, and atrophie blanche. We present a case series of 17 patients with clinical and/or histopathologically confirmed livedoid vasculopathy from a single tertiary centre in India with female-to-male ratio of 1.5:1 and mean age of 36.12 ± 12.02 years. Presentation with burning pain around ankles was seen in 83.33% of patients, while 100% had atrophie blanche/scarring and 76.47% had retiform ulcers. Hypercholesterolemia was seen in four patients, while systemic lupus erythematosus (SLE), anti-phospholipid antibody with SLE, dermatomyositis and hyper-homocysteinemia were seen in one patient each. The most common histopathology finding was hyaline thrombi within dermal vessels in 94.11%. On treatment with dual anti-platelet therapy, 70.58% of patients could achieve significant improvement in their Visual Analog Scale, Dermatology Life Quality Index and reduction in ulcer scores without serious adverse events. Out of 17 patients, 11 experienced flare in their disease course over one year period of follow-up. This cohort aims to contribute to Indian literature of this underreported entity.

Atrophie blanche complicated with lower limb infection and maggot growth: A case report.

Background: Atrophie blanche (AB) is a thrombotic vascular disease, also known as venous vasculitis or segmental hyaline vasculitis, characterized by chronic recurring painful ulcers on the lower legs, especially the ankles. AB is a clinically rare condition, affecting 1%-5% of the population, specifically middle-aged women with an average age of 45 years, and cases of AB in children are rare. Following recovery, ivory-white atrophy spots accompanied by pigmentation and telangiectasia remain in patients. One of the complications of AB is the parasitic growth of microorganisms infecting the ischemic soft tissue undergoing necrosis in the lower limbs. Furthermore, although infection combined with microbial parasitism is a type of surgical site infection, myiasis is particularly rare, which may warrant limb amputation or may even be life-threatening. Understanding the complications of AB may help in early and timely surgical debridement as well as wound repair. Summarizing the knowledge and treatment strategies of AB and formulating clinical strategies and guidelines for AB management with insights from relevant cases are important. Case summary: A 59-year-old woman was hospitalized due to repeated ulceration of the skin of the right lower leg for 3 years, aggravation, and maggot growth for 3 days. In the previous 3 years, the skin and soft tissue of the right calf had become ischemic, necrotic, and infected, but the patient did not seek any medical treatment. Subsequently, 2 years ago, she was diagnosed with AB at the dermatology department of our hospital. After hormone treatment, her right leg improved. However, 1 year ago, the skin and soft tissue of the right leg again became ischemic, necrotic, and infected. This time, the patient did not seek medical treatment and applied musk on her wound. The wound deepened, resulting in the exposure of the tendon and some bones. In addition, a large number of maggots and microorganisms grew in and infested the wound for 3 days before the patient came to our department for treatment. Debridement of the necrotizing infected site on the right lower leg combined with negative pressure vacuum sealing drainage were performed twice within 16 days after admission. Simultaneously, antibiotics were given systemically. On the 17th day after admission, the wound appeared clean, myiasis had resolved, and the growth and coverage of the granulation tissue on the wound were satisfactory. Subsequently, debridement of the infected site on the right leg, removal of skin of the right thigh, and autologous free skin grafting were performed. After 10 days, the wound was clean, all skin grafts had survived, and wound repair was satisfactory. Finally, the patient was discharged after 38 days of hospitalization. Conclusion: Although AB is rare, leukodystrophy requires specialized treatment and regular follow-up. If lower limb infection and maggot growth occur simultaneously, self-treatment should be avoided and medical attention must be sought immediately. Early implementation of wound debridement and anti-infective treatment combined with wound repair, which should be performed after cleaning the wound, is advised.

Comparative Efficacy of Rivaroxaban and Immunoglobulin Therapy in the Treatment of Livedoid Vasculopathy: A Systematic Review.

Livedoid vasculopathy (LV) is an uncommon chronic coagulation disorder whose underlying etiology is not yet fully understood. It predominantly affects females, especially those in late adolescence. There is currently limited research on treatment options for those with this diagnosis. The present systematic review aims to compare the efficacy of rivaroxaban and intravenous immunoglobulin (IVIG) therapy in the treatment of livedoid vasculopathy. A detailed search was conducted from April 20, 2022, to May 1, 2022, using four databases: Elsevier, Medline Complete, Medline Ovid, and PubMed. Out of these, 20 relevant articles were used, and the data was extracted and analyzed. Both rivaroxaban and IVIG were shown to be effective treatment options with similar treatment response times. However, future large-scale clinical trials are needed to determine an established treatment regimen for these patients.

Efficacy of an anti-TNF-alpha agent in refractory livedoid vasculopathy: a retrospective analysis.

BACKGROUND: Livedoid vasculopathy is a recurrent thrombo-occlusive vasculopathy of cutaneous blood vessels and its standard or first-line therapy is still controversial. Besides hypercoagulability, inflammatory factors may also play a secondary role in the pathogenesis of this disease. Monotherapy of thrombolytics cannot achieve satisfactory results because of concomitant inflammation. OBJECTIVE: This pilot study aimed to determine the efficacy of an anti-TNF-alpha agent in patients with refractory livedoid vasculopathy. METHODS: We studied five patients with livedoid vasculopathy who were resistant to steroids, antiplatelets, or danazol therapy, and were treated with etanercept 25-50 mg once a week for 12 consecutive weeks. We assessed clinical characteristics, laboratory findings, and etanercept's efficacy on skin lesions, pain, and quality of life. RESULTS: Etanercept therapy resulted in fast relief of pain in a mean time of 2 weeks. The median duration for the disappearance of erythema and ulcer healing was 8.8 weeks and 10.6 weeks, respectively. There was a reduction in pain by 34.3% after 12 consecutive weeks of etanercept treatment. Disease severity and quality of life significantly improved. CONCLUSIONS: In refractory livedoid vasculopathy patients, etanercept therapy is efficient for skin lesions and pain, and improvement of quality of life, especially in rapid relief of pain.

Recognition and Management of Cutaneous Polyarteritis Nodosum Versus Livedoid Vasculitis: A Case Report.

Cutaneous polyarteritis nodosum (CPAN) is a vasculitis of small and medium-sized muscular arteries of the dermis and subcutaneous tissue with no associated systemic involvement. A common presentation of CPAN can be misinterpreted as a non-invasive form of livedoid vasculitis, synonymous with the "atrophie blanche" which similarly presents as ivory-white stellate-shaped scars. Although hyperpigmentation can also be present, as seen in our 47-year-old female patient, cutaneous polyarteritis nodosum is unique due to the etiology of the inflammatory illness which requires a deep, segmented skin biopsy for diagnosis in order to identify the vessel inflammation. In this case report, we discuss a patient with a 20-year history of painful, recurrent ulcerations and polyneuritis with previous ulcer eruptions that healed as ivory-white stellate scarring. AB cutaneous forms of polyarteritis nodosum (PAN) may be only one manifestation of the disease, with other presentations in association with multi-organ system disease. This report will discuss the necessity of a high index of clinical suspicion with a clinical presentation similar to that of our patient. We will discuss the importance of early recognition and diagnosis of cutaneous vasculitis, such as CPAN, based on clinical presentation and history in hopes of limiting morbidity and the risk of progression to systemic forms of the disease.

Aetiology and management of atrophie blanche in chronic venous insufficiency.

The presence of chronic venous insufficiency results in venous hypertension, which can lead to the development of venous leg ulceration. Patients often present with oedema of the lower limb and skin changes, for example, the presence of haemosiderin and lipodermatosclerosis, as well as ulceration. In some instances, patients can also develop atrophie blanche (AB)-white fibrotic areas on the skin adjacent to the ulcer. AB remains an ambiguous term owing to the use of many synonyms. Hence, health professionals need to be aware of the clinical presentation of AB and should be able to clearly differentiate between scarring caused by previous ulcers and that caused by the presence of AB. In this article, the authors discuss the underlying diseases associated with AB and explore the treatment of AB in patients with chronic venous insufficiency.

Efficacy of intravenous immunoglobulins in livedoid vasculopathy: long-term follow-up of 11 patients.

BACKGROUND: Evidence for the efficacy of various therapies of livedoid vasculopathy (LV) is limited. OBJECTIVE: We sought to determine efficacy and tolerability of 2 g/kg of intravenous immunoglobulins (IVIG) every 4 weeks in patients with LV. METHODS: This was a long-term follow-up study of 11 patients with LV treated with 2 g/kg of IVIG assessing the clinical characteristics, disease course, and quality of life. RESULTS: The treatment regimen led to complete remission of ulcerations and pain in 17 of 29 disease episodes (59%) after 3 cycles and in 25 of 29 episodes (86%) after 6 cycles. Two disease episodes showed remission after 7 and 8 cycles, resulting in a total number of remissions of 27 (93%). Subscore analysis showed resolution of pain in 80% after 2 IVIG cycles. Disease severity and quality of life were significantly improved after 6 cycles. Median duration of remissions was 26.7 months after initial and 7.5 months after subsequent disease episodes. LIMITATIONS: This was a retrospective study that did not include the comparison of IVIG efficacy and its impact on quality of life with treatment options. CONCLUSIONS: In our patients with LV, high-dose IVIG led to fast and complete resolution of pain and ulcerations and to substantial improvement in quality of life.

Unilateral livedoid vasculopathy associated with involutional phase of cutaneous infantile hemangioma: the connection to coagulation disorders.

Livedoid vasculopathy is a bilateral painful and recurrent cutaneous ulcerative disorder of the legs that leads to atrophie blanche, atrophic white-porcelain scars, and is associated with disorders of fibrinolysis and/or coagulation. We present a young boy with an association between livedoid vasculopathy in the area of a previous involuted cutaneous hemangioma. We found 4 uncommon abnormalities associated with thrombo-occlusive events: heterozygous 20210 A→G genotype of prothrombin, reduced activity of anticoagulation proteins C and S, and elevated lipoprotein (a).

Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach.

Livedoid vasculopathy (LV) is a noninflammatory thrombotic condition presenting in a primary idiopathic or secondary subtype associated with abnormal coagulation factors. Different from atrophie blanche (AB), which is a clinical manifestation of certain scars, LV may have AB in combination with recurrent livedo reticularis with chronic and painful skin ulcers particularly around the ankle region, and at the back of the feet. Histology is characterized by segmental hyalinizing changes at the subintimal region of small dermal vessels with thrombotic occlusions. LV skin ulcers resolve with stellate, porcelain-white scars that need to be distinguished from similar changes seen with venous insufficiency. "Atrophie blanche" was originally used synonymously with "livedoid vasculopathy." AB describes spontaneously occurring porcelain-white skin areas with red dots that typically occur in the context of skin changes attributed to chronic venous insufficiency. The 2 forms of AB--(1) the LV-AB complex and (2) AB in the context of chronic venous insufficiency--are unrelated and require separate diagnostic and therapeutic approaches. Using a modified Delphi method, we have developed an international consensus document on the diagnosis and management of LV. Individual sections of this document provide advice on diagnosis and management of LV.

Livedoid vasculopathy managed with hyperbaric oxygen therapy.

Livedoid vasculopathy is an uncommon condition resulting in painful lower extremity ulceration and scarring. This condition presents as purpuric macules and papules that progress to painful, irregular ulcers of the lower legs and dorsal feet. These ulcerations are often recurrent and chronic with spontaneous remissions and exacerbations that may be seasonal. The first case, a 22-year-old female presented with three-year history of recurrent multiple non-healing ulcers involving feet and ankles. The ulcers were associated with severe debilitating pain and paraesthesia, as a result of which she was unable to walk without support. Patient was administered HBOT at pressure of 2.5ATA for 1 h daily, six days a week. After ten sittings of HBOT, patient reported a drastic reduction in the pain along with reduction in the dose of analgesic by half and a definite improvement in her walking. The second case was a 49-year-old male who also had history of recurrent ulceration on the dorsum of feet and ankles associated with severe pain. With HBOT, the patient felt an improvement in pain and ambulation by the 8th sitting and complete relief from pain by the 17th sitting. HBOT is a recognized modality of treatment of various problem wounds and non-healing ulcers due to various etiologies. The above two cases show that it can be a useful treatment modality for livedoid vasculopathy where other treatment modalities have failed and therefore could be given to a larger number of patients in hospitals where it is available.

Drug-associated livedoid vasculopathy

Livedoid vasculopathy is reported in a 38-year-old white female, who first presented with spontaneous skin lesions in the left ankle, at 17 years old. For the last fifteen years she used low-dose oral contraceptive (gestodene and ethinylestradiol) and persisted asymptomatic for a long period. Nevertheless, painful red papules and dark spots reappeared in the same area and progressed to an intensely tender and irregular shallow ulcer, during summer. Skin biopsy samples showed dermal vessels with subintimal fibrinoid necrosis and intraluminal thrombosis, without clear inflammation. Cutaneous lesions have improved faster after topical betamethasone was added to treatment. Data from the present case suggests a drug-associated livedoid vasculopathy.

Relata-se caso de vasculopatia livedóide em uma mulher branca de 38 anos, que inicialmente apresentou lesões cutâneas espontâneas no tornozelo esquerdo aos 17 anos de idade. Durante os últimos quinze anos, fez uso de contraceptivos orais (gestodene e etinilestradiol). Permaneceu assintomática por longo período; entretanto, no último verão, pápulas avermelhadas dolorosas e manchas escuras reapareceram na mesma área e formaram uma úlcera rasa irregular intensamente dolorosa. O exame das amostras da biópsia de pele mostrou vasos dérmicos com necrose fibrinóide e trombose intraluminar, sem reação inflamatória. As lesões cutâneas apresentaram cicatrização mais rápida após acréscimo de betametasona tópica ao esquema terapêutico. Os dados do presente caso sugerem vasculopatia livedóide associada ao uso de droga.

A Case of Atropie Blanche Accompanied by Cryoglobulinemia / 대한피부과학회지

We report a case of atrophie blanche which was accompanied by cryoglobulinemia. The patient, 17-year-old female, have had recurrent painful ulcerations and ivory-white atrophic scars with telangiectases surrounded by hyperpigmentation. On histological examination, vessel wall thickening and hyalinization of the intima, partial vascular occlusion, vascular proliferation and mild perivascular chronic inflammatory cells infiltration and hemorrhage in the upper dermis were noted. The patient had been treated with dipyridarnol(Persantin) and acetylsalicylic acid hut the lesions had been waxed and waned.