[Non-healing wounds]. / Een wond aan het been die niet geneest.

The incidence of non-healing wounds is increasing. Identification of the underlying cause of a wound is of essential importance for adequate treatment. In this article, we present three female patients aged 50, 65 and 85 years with respectively pyoderma gangraenosum, livedoid vasculopathy and Martorell hypertensive ischaemic leg ulcer. All patients were treated with local wound care for weeks without a valid diagnosis. In retrospect it can be concluded that several warning signals had not been recognised. Severe pain, atypical location or appearance, insufficient healing and progression of the wound despite adequate wound care should all be considered red flags. Patients with non-healing wounds require prompt referral and more extensive diagnostic investigation. Our cases also show that a multidisciplinary wound care team ensures and accelerates consensus on diagnosis and treatment plan. Such a team can ensure and coordinate follow-up in the home environment.

Multifaceted approach to a rare clinical case of calciphylaxis in a renal transplant recipient.

Calcific uremic arteriolopathy (CUA) is a highly morbid condition usually found in ESRD patients that has rarely been reported after renal transplantation and renal function restoration. Furthermore, little is known about the optimal management of CUA in this setting. Herein, we report on the clinical case of AB, a 70-year-old woman who developed CUA after renal transplantation and renal function restoration. However, other risk factors for CUA such as diabetes and warfarin treatment, due to mechanical aortic valve implantation, were present. Thirty-eight months after renal transplantation she developed erythema and livedo reticularis in both legs and a gradually enlarging skin ulcer in the right leg. A skin biopsy of the ulcer showed features compatible with the CUA, such as sub-intimal calcification and luminal obstruction of the small dermal arterioles, tissue ischemia and signs of adipocytes degeneration. A multidisciplinary approach was adopted, including medical and non-medical treatments such as surgical debridement and vacuum-assisted closure therapy. Medical treatments included a five weeks course of once a week intravenous infusion of pamidronate and intravenous sodium thiosulfate (STS) at increasing doses. Four months after beginning the therapy with STS, a complete healing of the ulcer on the right leg and the disappearance of the livedo reticularis on the left leg was noted. In conclusion, although rare CUA may develop also in renal transplanted patients, a timely and combined therapeutic approach is essential for its resolutive treatment. Sodium thiosulfate therapy has proven to be effective and tolerated.

Livedoid Vasculopathy Presenting in a Patient With Sickle Cell Disease.

We report a case in which a 43-year-old African American male with medical history of sickle cell disease (SCD) presented with a nonhealing ulcer. Biopsy revealed features of livedoid vasculopathy. Previously, livedoid vasculopathy had only been described in a patient with sickle cell trait, but never in a patient with SCD. Livedoid vasculopathy most commonly affects the distal lower extremities and is characterized by irregular, punched-out, painful ulcers that heal with stellate white scars of atrophie blanche. Histologically, it reveals segmental hyalinizing vessels, focal thrombosis, and endothelial proliferation. The etiology is currently unclear, but it has been shown to be related to procoagulant states and a diagnosis of livedoid vasculopathy should prompt a thorough hypercoagulable workup, including testing for SCD in high-risk patients.

Treatment for Livedoid Vasculopathy: A Systematic Review.

Importance: Livedoid vasculopathy is a painful, ulcerative condition of the lower extremities for which no established treatment exists. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series. Objective: To systematically review the treatment for livedoid vasculopathy and synthesize the available clinical data. Evidence Review: A systematic review of the literature using Ovid MEDLINE (covering the period January 1, 1946, through June 9, 2017) and Ovid EMBASE (covering January 1, 1947, through June 9, 2017) databases was performed with a broad and inclusive search strategy along with a subsequent search of the references of retrieved articles. All case series reports published in the English language and in a peer-reviewed journal discussing the treatment for livedoid vasculopathy diagnosis were included. Findings: A total of 29 case series reports published in the English language and in a peer-reviewed journal discussed the treatment for livedoid vasculopathy. These reports represented a total of 339 patients, of whom 230 (68%) were female and 69 (20%) were male; sex was not stated for 40 patients. Treatment with anticoagulants, antiplatelets, anabolic steroids, thrombolytics, hyperbaric oxygen, intravenous immunoglobulins, vitamin supplementation, UV light, and a combination of 1 or more of these among other therapies had a favorable outcome. Anticoagulants were the most commonly used monotherapy, achieving a favorable response in 62 of 63 patients (98%). Anabolic steroids, intravenous immunoglobulins, and antiplatelets were the second, third, and fourth most commonly used treatments, respectively. All of these therapies were associated with good clinical outcomes. Adverse events were observed in 44 patients (13%). Conclusions and Relevance: A variety of treatments with varying degrees of success have been used to treat livedoid vasculopathy. Randomized clinical trials should be performed in the future to better establish these treatments in clinical practice.

Chemical lumbar sympathectomy in the treatment of idiopathic livedo reticularis.

OBJECTIVE: Livedo reticularis (LR) is a reticulated discoloration of the skin, particularly on lower extremities. Few treatment options are reported. This study investigated the efficacy and safety of chemical lumbar sympathectomy (CLS) in idiopathic LR. The key technique points of CLS are also illustrated in detail. METHODS: Patients with idiopathic LR with a strong desire for treatment were recruited during a 2.5-year period. L3-4 CLSs were performed with 5% phenol (2 mL) in each injection site. The needle tip extends to approximately one-third of the vertical dividing line of the vertebral body. The contrast along the psoas muscle fibers indicates targeting on gray rami communicans instead of the sympathetic trunk. The primary efficacy variable was achieving "clear or almost clear" of LR lesions staying >1 hour in a 24°C air-conditioned room. Safety assessments included monitoring and recording of all adverse events and tolerability to treatment. The follow-up period was 2.5 to 4.7 years. RESULTS: Ten women (median age, 22 years) were enrolled. Seven patients achieved "clear or almost clear" of LR lesions after CLS. The postoperative skin surface temperature increase was 7.4°C ± 2.6°C. Two patients achieved "major partially resolved," and one patient achieved "minor partially resolved." Two of the seven with "clear or almost clear" results reported recurrence ≤1 year during the follow-up, CLS was repeated, and they then achieved "clear or almost clear" again. Two patients reported mild pain localized to the thigh area, which resolved spontaneously by the second day. CONCLUSIONS: This study showed CLS provides a valid option for the treatment of idiopathic LR. The efficacy of CLS can be long-lasting, and CLS can be repeated if LR recurs. Targeting at gray rami communicans, rather than the sympathetic trunk, is comparably effective and safer for sympathetic interruption.

Sensory ganglionopathy with livedoid vasculopathy controlled by immunotherapy.

INTRODUCTION: Livedoid vasculopathy is a rare dermatological condition characterized by painful ulceration, atrophic scarring, and persistent livedo reticularis. The pathogenesis is unclear. METHODS: We report a patient with biopsy-proven livedoid vasculopathy who developed a progressive sensory ganglionopathy with profound sensory ataxia. Serial nerve conduction assessments were undertaken. RESULTS: Combined treatment with prednisolone and mycophenolate mofetil failed to control the ganglionopathy. After addition of rituximab, both symptoms and nerve conduction studies showed stabilization. CONCLUSIONS: Sensory ganglionopathies associated with autoimmune and inflammatory conditions may be characterized by a sub-population of "sick" dorsal root ganglia that can be rescued with aggressive immunotherapy.

Atrophie blanche: is it associated with venous disease or livedoid vasculopathy?

PURPOSE: The purpose of this learning activity is to provide information about the etiology and treatment of atrophie blanche. TARGET AUDIENCE: This continuing education activity is intended for physicians and nurses with an interest in skin and wound care. OBJECTIVES: After participating in this educational activity, the participant should be better able to:1. Discuss the pathophysiology of atrophie blanche.2. Explore treatment options for livedoid vasculopathy. ABSTRACT: Atrophie blanche (AB) is a porcelain-white scar that may be seen at the base of a healed ulcer or in association with livedoid vasculopathy (LV). The term AB originally had been used synonymously with LV, whereas LV is a noninflammatory thrombotic condition presenting as either a primary or secondary event (often associated with coagulation).

Livedo reticularis heralding hypercalcaemia of malignancy.

The term livedo reticularis is used to describe net-like purple rash usually on the lower limbs. It is an important clinical sign with diverse aetiologies. Hypercalcaemia is an uncommon but important clinical entity, sometimes associated with livedo reticularis. Generally, hypercalcaemia of renal failure and secondary hyperparathyroidism has been reported with this condition. We report a case of livedo reticularis heralding onset of hypercalcaemia of malignancy.

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 - a thrombotic disease.

Livedoid vasculopathy is a rare, chronic occlusive disease of vessels supporting the upper layers of the skin. It is characterized by purpuric maculae and recurrent painful ulcerations mostly affecting the lower leg. These ulcerations occur episodically especially in summer time and heal slowly, leaving characteristic porcelain-white scars called ’atrophie blanche’.This review is focused on the current knowledge on livedoid vasculopathy and modern therapy strategies resulting from its etiopathogenetic associations with prothrombotic states. Livedoid vasculopathy and its pathophysiology are clearly distinguished from inflammatory vasculitis and thus require a different therapeutic approach. The prevention of irreversible residual scarring and improving the quality of life of patients in this often misdiagnosed disease is one of the main treatment goals.

Aortopathies: etiologies, genetics, differential diagnosis, prognosis and management.

Aortic root and ascending aortic dilatation are indicators associated with risk of aortic dissection, which varies according to underlying etiologic associations, indexed aortic root size, and rate of progression. Typical aortic involvement is most commonly seen in syndromic cases for which there is increasing evidence that aortic aneurysm represents a spectrum of familial inheritance associated with variable genetic penetrance and phenotypic expression. Aortic root and ascending aortic dimensions should be measured routinely with echocardiography. Pharmacologic therapy may reduce the rate of progression. Timing of surgical intervention is guided by indexed aortic size and rate of change of aortic root and ascending aorta dimensions. Lifelong surveillance is recommended.

Livedoid vasculopathy successfully treated with hyperbaric oxygen.

Livedoid vasculopathy is a painful, ulcerating condition of the lower legs, ankles and feet with the typical histological feature of hyalinising vascular change of dermal blood vessels with minimal inflammation. Therapeutic interventions have been diverse and varyingly successful. We report a biopsy-proven case in a 27-year-old male, which responded rapidly and completely to hyperbaric oxygen therapy. A few such cases have been reported previously, but only in dermatological journals, not in the hyperbaric medicine literature.

Intravenous immunoglobulins in difficult-to-treat ulcerated livedoid vasculopathy: five cases and a literature review.

BACKGROUND: Livedoid vasculopathy (LV) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports suggest the successful use of intravenous immunoglobulins (IVIG) in LV. METHODS: Outcomes in five patients treated with IVIG for treatment-resistant ulcerated LV were retrospectively analyzed. RESULTS: Treatment with IVIG induced complete remission (based on clinical evaluation and a pain-related visual analog scale) in four patients but was ineffective in one patient. Three patients relapsed; the median time to relapse was 10.7 months. Re-treatment with IVIG in these three patients was successful. CONCLUSIONS: These cases confirm previous reports that IVIG seems to be a rapid, effective, and safe treatment for patients with idiopathic refractory ulcerated LV. However, a placebo-controlled study is mandatory to confirm these results.

Livedoid vasculopathy as a coagulation disorder.

Livedoid vasculopathy is an occlusive cutaneous disease of the dermal vessels with pauci-inflammatory or non-inflammatory histopathology findings. It is characterized by the presence of macules or papules, erythemato-purpuric lesions located on the legs, especially on the ankles and feet, which produce ulcerations that are intensely painful and originate ivory atrophic scars called "atrophie blanche". In this review article, studies on LV from the literature are analyzed, and their etiopathogenic associations, particularly those related to the thrombophilic states, as well as the pathologic findings and therapeutic approaches applied in the difficult clinical management of these cases, are evaluated.

[A 65-year-old man with history of claudication, palpable purpura and livedo reticularis]. / Claudicatio intermittens, palpable Purpura und Livedo reticularis bei einem 65-jährigen Patienten.

HISTORY AND ADMISSION FINDINGS: A 65-year-old man was admitted with history of claudication symptoms and painful skin lesions of the lower legs. Physical examination showed palpable purpura of the lower legs and livedo reticularis, most marked at the forefoot and toes. INVESTIGATIONS: Computed tomography (CT) showed an aortic mass 2 cm above the bifurcation. This was treated after angiography with a covered stent. Biopsy of the skin lesions showed no sign of vasculitis and no cholesterol crystals. TREATMENT AND COURSE: The patient was discharged and remained symptom-free for 9 months. He was readmitted at that time with recurrent complaints. CT revealed a subtotal stenosis of the aortic stent. A skin biopsy showed CD31-positive tumor cells in small arteries. Biopsy of a new osteolytic lesion in the ileum confirmed the diagnosis of angiosarcoma of the aorta. The patient decided in favor of palliative care and was discharged from the hospital. CONCLUSION: Primary tumors of the aorta, although they are rare, should be considered in the presence of an intravascular mass with stenosis to blood flow. A skin biopsy is easy to conduct and often leads to the final diagnosis.