Komplikationen bei Pseudoainhum

A 39 year old man presented with recurrent episodes of leukocytoclastic vasculitis and a large ulcer at the distal part of the stump of his right leg. Since birth the patient showed a clinical picture of pseudoainhum as part of the amniotic band syndrome (Simonart syndrome). Radiologic examination of the right foot showed only an hypoplastic talus and calcaneus which had been designated as a "stump":, which have been detected by x-ray. On both upper limbs there were missing phalanges, syndactyly and some constrictive fibrotic bands. Since the ulcer worsened with therapy, the patient underwent the amputation of the stump just below under the knee. Since then no further vasculitic lesions have been observed. We consider this case remarkable both because pseudoainhum is so rare and because of these equally unusual complications.     

老年下肢静脉性皮肤溃疡1例

 报道老年下肢静脉性皮肤溃疡1例。患者女，84岁，因双下肢溃疡伴疼痛1月就诊。皮肤科检查：右足背红肿，见11 cm×4 cm大小溃疡，有脓苔及黑色结痂，内踝见20 cm×5 cm大小溃疡，左小腿散在结痂面。D 二聚体4.81 mg/L。皮损组织病理示：表皮缺失，溃疡面坏死、渗出，毛细血管周围大量炎性细胞，肉芽肿形成。下肢静脉彩超：右小腿见数条肌腱静脉增宽，最宽处约4.8 mm，未见明显血流信息。诊断：右下肢静脉性皮肤溃疡。予低分子量肝素钠针、血栓通冻干粉针抗凝治疗14天及对症处理，出院时溃疡好转但血栓未通；出院后内服中药活血理气汤3周，溃疡恢复，下肢静脉彩超血栓消失。     

Skin manifestations of refractory anemia with excess of blasts (RAEB)

The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)     

Erfolgreiche Therapie eines Ulcus cruris rheumaticum mit systemischen Immunglobulinen

Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disease. Leg ulcers in rheumatoid arthritis may be caused by vasculitis and are an interdisciplinary therapeutic challenge. A 69 year old women with rheumatoid arthritis and many other medical problems presented with widespread vasculitis-induced therapy-resistant ulceration on her right lower leg. Since previous therapeutic efforts had a negative effect on wound healing, we administered intravenous immunoglobulins which led to complete healing of the ulcer was achieved. Both the serological and rheumatologic features of her rheumatoid arthritis also improved. Intravenous immunoglobulins represented an effective therapeutic option with fewer side effects in the therapy of vasculitis-induced leg ulceration in this patient with rheumatoid arthritis.     

Initial cutaneous manifestations associated with histopathological leukocytoclastic vasculitis in two patients with antiphospholipid antibody syndrome

Antiphospholipid antibody syndrome (APS) is a multisystem disorder associated with a variety of circulating autoantibodies that target different phospholipid protein complexes. APS is sometimes lethal as a result of severe sequelae, which may be primary or secondary to the underlying disease. We report two women who presented histopathologically with leukocytoclastic vasculitis as the first cutaneous manifestation and were subsequently diagnosed with APS associated with systemic lupus erythematosus (SLE). Patient 1 presented with widespread cutaneous necrosis (WCN) with rapidly spreading pain down the lower extremities. Skin biopsy specimens from her leg purpura and WCN revealed perivascular infiltrates with neutrophils consistent with leukocytoclastic vasculitis and thromboses of small-sized dermal vessels. Patient 2 exhibited livedo reticularis, painful cutaneous nodules with necrosis, ulcer, and erythematous macules on her lower extremities, shoulder, and face. Skin biopsies of her right knee showed intravascular thrombosis of small dermal vessels and infiltration of perivascular tissues with necrotizing granulomatous vasculitis in the dermis. We found that these various cutaneous manifestations with leukocytoclastic vasculitis were present at an early stage of APS. Although progression to leukocytoclastic vasculitis in patients with APS is uncommon, our data suggest that the association between microvascular occlusions and cutaneous vessel vasculitis has a predictive value for the pathogenesis. It is important for dermatologists to recognize these cutaneous signs to permit early and accurate diagnosis and treatment.     

2 basaliomas of the lower leg in a 60-year-old female--one of them simulating ulcus cruris

We report on a 60-year-old female patient with an ulcer on the left lower leg, which was treated for 2 years without success. Histologically, a biopsy specimen showed a partly fibrotic and partly solid basal cell epithelioma. When all of the patient's skin was examined, a small skin-colored nodule was found on her right lower leg that histologically also turned out to be a basal cell epithelioma. The patient had no history of predisposing factors like exposure to arsenic or actinic skin damage. Our case is compared with the literature and briefly discussed.     

Infiltrating basal cell carcinoma in the setting of a venous ulcer

A 77-year-old man was referred with a 5-year history of an intermittently painful, nonhealing right medial ankle ulcer. The ulcer had not responded to multiple treatment modalities, including Unna boots, compression therapy, sclerotherapy, and split-thickness skin grafting. The past medical history was significant for a deep venous thrombosis in the right leg 30 years earlier (treated with warfarin for 3 months) and a history of greater saphenous vein harvesting for coronary bypass grafting 28 years previously. After the vein stripping, the patient had suffered from increasing right leg edema and stasis changes in the right leg. His history was also remarkable for coronary artery disease, dyslipidemia, and lymphoma treated with chemotherapy 8 years before presentation, with no evidence of recurrence. He had stopped smoking approximately 20 years earlier. Medications included atenolol, simvastatin, nicardipine, nitroglycerin, and aspirin. Skin examination revealed a 3.0 x 3.5-cm ulcer adjacent to the medial malleolus. The edges of the ulcer appeared raised and rolled (Fig. 1). Centrally, there was granulation tissue, which appeared healthy. There were surrounding dermatitic changes. Dorsalis pedis and the posterior tibial pulses were normal. Noninvasive vascular studies revealed severe venous incompetence of the right popliteal and superficial veins. Arterial studies and transcutaneous oximetry were normal. Computed tomographic scan of the pelvis did not reveal any adenopathy, and radiographic imaging did not reveal any bony changes suggestive of osteomyelitis. Biopsy of the ulcer edge and base showed infiltrating basal cell carcinoma (Fig. 2). Mohs' micrographic surgery required three layers; the final extent of the ulcer was 7.8 x 6.9 cm. A split-thickness skin graft was placed.     

Successful treatment of a leg ulcer occurring in a rheumatoid arthritis patient under leflunomide therapy

Objective We report the case of a leg ulcer in a rheumatoid arthritis (RA) patient under treatment with leflunomide, discuss the influence of the drug on the aetiopathogenesis of the ulcer and describe its successful treatment. Case summary A 68‐year‐old woman with a 12‐year history of RA developed a leg ulcer after 4 months of leflunomide treatment. Other ulcerogenic factors were ruled out. There were some clinical hints for rheumatoid vasculitis. The ulcer was resistant to ambulant conservative phase adapted wound bed preparation and a split skin transplantation failed. After omission of leflunomide and washout procedure with cholestyramine a second split skin transplantation resulted in complete healing. Discussion Leflunomide inhibits the division of activated T cells and thus inhibits among others the production of proinflammatory cytokines and the adhesion of cells to the endothelium. These mechanisms may partly explain the possible influence of leflunomide on the perpetuation of the ulcer. Until now, occurrence of vasculitis and leg ulcers has been described in one case each for the novel immunomodulator leflunomide. No successful treatment of a leg ulcer under leflunomide has been described yet. Omission of leflunomide and a washout treatment in our case led to a complete healing. This may indicate a critical role of leflunomide in the maintenance of this slow healing ulcer. Conclusions An association between leflunomide intake, occurrence of leg ulcers in RA patients and delayed wound healing should be considered.     

Churg‐Strauss syndrome presenting as scar reactivation: histopathologic features and an illustration of ‘locus minoris resistentiae’

We report a 33‐year‐old female with cutaneous involvement by Churg‐Strauss syndrome confined to surgical scars that were obtained 13 years before. She presented to the emergency department with 2‐day history of fever, night sweats, right‐sided weakness, hoarseness and worsening asthma symptoms. She was found to have an eosinophilia and two sub‐5‐mm pulmonary nodules. The patient also reported that the scars on her right thumb, inner wrist and back had been swollen, red and painful for 2 days. Examination revealed tender, erythematous, well‐healed edematous scars studded with small skin colored papules. She had no clinical findings that were classic for cutaneous vasculitis. A skin biopsy of a scar revealed perivascular and palisading granulomatous inflammation consisting of histiocytes and neutrophils with leukocytoclasia. Focal vascular injury was identified. Scattered tissue eosinophils were seen. Special stains were negative for infection. Thereafter, she was started on intravenous steroids, at which point the fever, pulmonary and cutaneous symptoms subsided. Although scar sarcoidosis is a well‐described phenomenon, granulomatous inflammation and vasculitis seen in Churg‐Strauss syndrome exclusively manifesting in well‐healed surgical scars highlights the unique features seen in this case and draws attention to the concept of locus minoris resistentiae. This case also highlights how a skin biopsy in the setting of suspected systemic vasculitis can confirm the presence of vasculitis and/or granulomatous inflammation and obviate the need for more invasive, higher risk procedures such as lung biopsy.     

Red fingers syndrome and recurrent panniculitis in a patient with chronic hepatitis C

Cases of red fingers syndrome and nodular vasculitis in patients with hepatitis C have rarely been described. Both red fingers syndrome and nodular vasculitis are characterized by microscopic vasculitis, and it has been hypothesized that hepatitis C plays a role in the pathogenesis of cutaneous disorders such as vasculitis, especially in cryoglobulinaemic vasculitis. We describe the case of a 75-year-old woman diagnosed with chronic hepatitis C who presented with nodular lesions on her right thigh and red fingers syndrome. A skin biopsy taken from a nodule showed infiltration of the vessel walls by mononuclear cells and septal and lobular panniculitis. The diagnosis of nodular vasculitis was established. In our opinion, red fingers syndrome and nodular vasculitis might be related to a vascular reaction of immune-mediated mechanisms induced by hepatitis C.     

CHILD综合征长期随访一例

【摘要】 患儿女,87 d龄,出生后因右侧躯体片状红色斑块伴同侧肢体畸形于2012年1月就诊。皮肤科检查：右下腹、右臀部、右侧会阴部、右腿及右足部见明显红色斑块,表面附着黄色鳞屑,边界清楚,无明显渗出及异味;右下颌、右颈部、右腋下及右手第1 ~ 4指指背见淡黄色疣状增生;右足第2、3、4趾只有1个趾间关节且甲板缺如。右足X线片显示右足畸形及骨质缺损。诊断：CHILD综合征。患儿间断口服阿维A胶囊,外用凡士林、他卡西醇软膏、弱效糖皮质激素软膏6年余,用药时皮损可缓解,停药易反复,后改外用辛伐他汀软膏2年。皮损随年龄增长等比例增大,患肢较健侧肢体生长速度稍快。至2020年1月患儿右下颌、右颈部及右手第1 ~ 4指皮损消退,右手第5指出现新发带状疣状增生性斑块,右小腿皮损较前稍好转;无其他系统受累表现。     

The lived experience of recurrence prevention in patients with venous leg ulcers: An interpretative phenomenological study

Aim of the studyTo explore patient understanding of why they develop a venous leg ulcer and how they can prevent recurrence.MethodThe methodological framework of the hermeneutic phenomenological approach was used. Semi-structured interviews were conducted with seventeen participants living with a venous leg ulcer from May 2017 to November 2018. Data were analysed using Smith's interpretative hermeneutic analysis.ResultsThe results are categorised into three main themes: “Trauma due to accident” (initial venous leg ulcer) and “Prevention of ulcer recurrence” (compression); “Trauma due to compression therapy” (venous leg ulcer recurrence). The findings demonstrate active venous leg ulcers are often caused by acute incidents while carrying out an activity in people with underlying chronic venous insufficiency. After a complete healing, preventive measures, such a compression stockings are initiated by the patient or health care provider. Trauma due to adherence to compression stockings caused skin breakdown beneath compression that caused subsequent ulcer recurrence.ConclusionThis study contributes to understanding the lived experience of patients with venous leg ulcers who develop a venous leg ulcer and their understanding of how they can prevent recurrence. Patients with VLUs would benefit from early preventive strategies, such as such a compression stockings fitting and application, integrated into daily care plan of primary care and community settings.     

Successful treatment of chronic venous leg ulcers with lyophilized cultured epidermal allografts

The case of a 67-year-old woman with chronic venous leg ulcers and severe gonarthrosis is described. In spite of intensive therapy, the leg ulcers had persisted for 4 years and made the intended orthopaedic operation of the right knee impossible. The patient was treated with lyophilized cultured epidermal allografts and her leg ulcers healed within 40 days. Lyophilized cultured epidermal allografts represent a modern type of active wound dressing that leads to rapid healing of chronic venous leg ulcers and enables patients to undergo surgical intervention.     

The role of low resistance blood flow pathways in the pathogenesis and healing of venous leg ulcers

In an attempt to clarify the pathophysiology of haemodynamics in legs with venous ulcer we investigated the effect of a single intermittent pneumatic compression treatment on the peripheral resistance of leg arteries and the cutaneous laser Doppler flux in the leg. Eight patients with venous leg ulcers and 10 subjects with healthy legs were investigated. Doppler waveforms of the leg arteries and laser Doppler flux of the leg skin were recorded before and after a single intermittent pneumatic compression treatment with the subjects in a recumbent position. In the legs with venous ulcer, the peripheral resistance of the arteries was lower and the laser Doppler flux was greater, compared with healthy legs (p = 0.003 and p = 0.002, respectively). A single intermittent pneumatic compression treatment raised the peripheral resistance in the arteries of legs with ulcer and laser Doppler flux of the skin more in ulcer legs than in healthy legs (p = 0.046 and p = 0.034, respectively). These findings suggest that removal of oedema causes redistribution of skin blood flow in the legs with venous ulcer favouring the superficial capillary perfusion. This could explain why compression treatment promotes the healing of venous leg ulcers.     

Homozygous factor V Leiden mutation in a patient with traumatic leg ulceration

Factor V Leiden mutation is a well-established risk factor for venous thrombosis and ulceration. We report a 20-year-old male patient who presented with a traumatic left leg ulcer that responded poorly to therapy. He had no previous medical history. Following investigation it was discovered that the patient had extensive venous thrombosis of the left leg veins and was homozygous for the factor V Leiden gene mutation. This case demonstrates the need for detailed history taking and thorough investigation of any atypical ulcer in young adults.     

Leg ulcers and hydroxyurea: report of three cases with essential thrombocythemia

CASE 1: A 65-year-old woman with essential thrombocythemia (ET) had been taking oral hydroxyurea (HU), 1,000 mg daily, for 7 years. Six months ago, she developed an ulcer on the outer part of her left ankle, which healed spontaneously within 2 months. She presented with a new, tender, shallow ulcer, 2 cm x 2 cm in size, at the same site. Doppler examination revealed thrombosis of the left common femoral vein and a calcified atheroma plaque of the left common femoral artery. The dosage of HU was decreased to 500 mg daily when the platelet counts were found to be within normal levels. The ulcer completely healed within 2 months with occlusive wound dressings, and has not recurred within the follow-up period of 1 year. CASE 2: A 56-year-old women presented with multiple, painful, leg ulcers of 1 year duration. She had been diagnosed as having ET and had been on HU therapy, 1,500 mg/day, for the past 5 years. Interferon-alpha-2b was started 3 months ago, in addition to HU, which was tapered to 1,000 mg daily. She had suffered from hypertension for 20 years treated with nifedipine and enalapril, and had recently been diagnosed with diabetes mellitus which was controlled by diet. Examination revealed three ulcers located on the lateral aspects of both ankles and right distal toe. Arterial and venous Doppler examinations were within normal limits. Histopathology of the ulcer revealed nonspecific changes with a mixed inflammatory cell infiltrate around dermal vessels. The ulcers completely healed within 10 weeks with topical hydrocolloid dressings. After healing, she was lost to follow-up. A year later, it was learned that she had developed a new ulcer at her right heel, 3 months after her last visit (by phone call). This ulcer persisted for 8 months until HU was withdrawn. CASE 3: A 64-year-old woman with ET presented with a painful leg ulcer of 6 months' duration. She had been taking oral HU for 5 years. She had a 20-year history of hypertension treated with lisinopril. Examination revealed a punched-out ulcer of 2 cm x 2 cm over the right lateral malleolus. Doppler examination of the veins revealed insufficiency of the right greater saphenous and femoral veins. Angiography showed multiple stenoses of the right popliteal and femoral arteries. As her platelet count remained high, HU was continued. During the follow-up period of 13 months, the ulcer showed only partial improvement with local wound care.     

Unklare akrale Nekrosen an den Fingern

A 63-year old woman presented with dry necrotic areas on the distal and medial phalanges of her right index and middle finger caused by burns while smoking. Differential diagnostic considerations included occlusive arterial disease, vasculitis, endangiitis obliterans, peripheral embolisms and exposure to chemicals. The patient had the habit of smoking her cigarettes until the last possible drag, putting her skin in direct contact with the burning tobacco.     

Therapie maligner Tumoren auf venösen Beinulzera

Malignant changes in persistent venous leg ulcers are a grave complication of chronic impaired wound healing. In our case, a venous leg ulcer had persisted on the right calf for 30 years. Exophytic tumors in the ulcer with frequent bleeding prompted biopsies. A squamous cell carcinoma was found, but only in the second biopsy. The surgical procedure was planned so that in a single session both the tumor and the underlying causes of the chronic venous insufficiency in the leg could be treated appropriately. Extirpation of the enlarged lymph nodes in the groin was combined with crossectomy and removal of the long saphenous vein, followed by circular radial excision of the ulcer scar fascia (fasciotomy). The excised tissue was examined histologically. Muscle biopsies were taken from the site of suspicious adhesions of the fascia to the calf muscle. The large, circular defects on the lower leg were covered with the appropriate dressing to condition the wound bed. After three weeks the well-granulated area was covered with meshed split skin grafts. During the operation and in the post-operative phase, machine-assisted and manual decongestion was performed, an established therapy for lymphedema, chronic venous insufficiency and chronic venous ulcers.     

Nokardioseinfektion einer Gefäßendoprothese

A 61-year-old woman presented with purple-red subcutaneous nodules on her right leg. She had experienced fever up to 40 degrees C for the past 10 days and felt generally weak over the last two months. Four months earlier, a vascular graft had been implanted in her right femoral artery. Based on the diagnosis of skin infection due to implantation of an infected prosthesis, she was hospitalized and treated with an antibiotic regime. During the initial antibiotic treatment, the symptoms deteriorated, and she developed joint, hepatic and CNS abscesses. Finally, a microbiologic culture with an extended incubation time revealed the diagnosis of an infection with the slowly growing bacterium Nocardia ssp. Hereupon the patient was treated purposefully with Meropenem over six months. This treatment resulted in complete recovery.     

Case of hypereosinophilic syndrome with cutaneous necrotizing vasculitis

A 25-year-old male presented with pruritic papules over his trunk and extremities. The pruritic eruption had appeared on the arms 5 months before and gradually spread over the trunk. Blood tests showed a white blood cell count of 13 760/microL with 42.2% eosinophils. A skin biopsy from the leg revealed necrotizing vasculitis in the upper dermis with prominent eosinophil infiltration in the dermis and subcutis. Liver dysfunction in accordance with eosinophilia was also seen. The underlying disorder causing the eosinophilia was not detected. We diagnosed this case as hypereosinophilic syndrome. Although systemic corticosteroid therapy using prednisolone was effective, the symptoms recurred during a reduction of prednisolone. The addition of cyclosporine resulted in improvement of his symptoms. Both drugs were successfully tapered without relapsing symptoms. Hypereosinophilic syndrome with cutaneous necrotizing vasculitis is a rare condition, and this case suggests that cyclosporine may be a useful therapeutic adjunct in facilitating steroid tapering.