Dystrophic calcification in chronic leg ulcers--a clinicopathologic study.

BACKGROUND: Dystrophic calcification (DC) is a risk factor for conservative treatment failure in chronic leg ulcers of various pathologies. PATIENTS AND METHODS: We performed a retrospective noncontrolled trial of 212 patients with 362 chronic leg ulcers who underwent ulcer shave excision with subsequent skin grafting. The ulcers existed for at least 3 months, and no healing was achieved with good ulcer care. Tissue was subjected to histopathology (hematoxylin-eosin and van Kossa stains). RESULTS: DC was evident in 39 patients (18%). Metaplastic subcutaneous bone formation was observed in 15 patients (7%). Clinical symptoms associated with DC were resistance to good ulcer care, pain, and ineffective effects of compression therapy (in venous ulcers). Ulcers were treated with deep dermatome shaving of the ulcer bed and surgical removal of DC. In the same setting, defects were closed using mesh graft transplantation. The procedure achieved a complete take rate in 80% and a significant decrease of pain in 95% of cases. When comparing the take rates in patients with and without DC, DC had a negative effect on outcome (take rate: 91% without DC vs 80% with DC, p<.05). CONCLUSIONS: DC is resistant to conservative treatment. The first-line treatment is deep ulcer dermatome shaving and complete removal of calcifications whenever possible.

Deep ulcer shaving combined with split-skin transplantation in distal calciphylaxis.

Calciphylaxis is a cause of painful deep ulcers. There is controversy about best wound management in this disease. A retrospective study of inpatients during the 3 years was made. Seven calciphylaxis patients were identified. All patients suffered from various associated pathologies including diabetes mellitus type II and chronic renal insufficiency. Ulcers were treated by aggressive and deep shaving combined with autologous split-skin grafting in the same session. A 30% to 90% take rate of the grafts eventually with a complete ulcer healing in 6 of 7 patients was achieved. No patient developed a deep cutaneous infection or sepsis. All patients are still alive except one. The single death was related to cardiovascular complications. In distal calciphylaxis, aggressive ulcer surgery with defect closure offers a marked improvement in quality of life and prevents early deep skin infections and sepsis as major causes of mortality.

Tumescent suction curettage versus minimal skin resection with subcutaneous curettage of sweat glands in axillary hyperhidrosis.

BACKGROUND: Axillary hyperhidrosis is a common problem with a strong negative impact on professional and social life. Various types of surgical procedures have been developed for its treatment. OBJECTIVE: We want to compare efficacy and risk-benefit ratio of two local surgical procedures, i.e., the minimal skin excision with subcutaneous curettage (Method A) and tumescent liposuction curettage (Method B). METHODS: A total of 163 patients with primary axillary hyperhidrosis as assessed by positive iodine-starch test were included. The age range of patients was 16 to 61 years (mean 28 years), including 33 males and 129 females. A total of 125 underwent Method A, and 37 were treated by Method B. Both procedures were performed in tumescent anesthesia. The mean follow-up was 21 months (Method A) and 48 months (Method B). The outcome was evaluated by patient's global assessment and by Minor's starch test. Patient satisfaction was scored as "satisfied,""partially satisfied," or "dissatisfied." Adverse effects, complications, hospitalization time, and time to return to work were recorded and compared for both methods. In patients who underwent Method A, scar formation was assessed only for the first axilla (n=99). RESULTS: In Method A, the rate of residual sweating was 12.0%. The relapse rate was 1.0% of patients or 2% of axillae. In Method B, the relapse rate was 16.2% of patients or 14.5% of axillae within 12 months. If we consider both the relapses and the residual sweating, this modified relapse rate per axilla was 12.8% for Method A and 14.5% for Method B. Patients who underwent Method B had significantly less pain, no atrophic or hypertrophic scars, and no complications such as wound infections, bleeding (with the need of a second operation), or delayed healing. Using Method A, the stay in hospital was on average 5.8 days per patient or 3.2 days per axilla. Mean time to return to work was 8.8+/-3.5 days. For Method B, the procedure was performed in an outpatient setting. The mean time to return to professional work was 1.3+/-0.8 days. The total satisfaction rate was 97% for Method A and 89.2% for Method B, respectively. CONCLUSIONS: As shown by this study, minor skin resection with subcutaneous curettage of axillary sweat glands (Method A) is somewhat more effective in permanent reduction of hyperhidrosis than suction curettage. The minimal invasiveness of suction curettage and the minimal scarring, however, are significant advantages over excisional surgery. Downtime after surgery is significantly shorter for suction curettage. Therefore, suction curettage might be the surgical treatment of choice for axillary hyperhidrosis.

Tumor necrosis factor-alpha inhibitor-induced psoriasis or psoriasiform exanthemata: first 120 cases from the literature including a series of six new patients.

Tumor necrosis factor-alpha (TNFalpha) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients from the literature including six new patients (three women and three men) who developed pustular lesions during treatment with TNFalpha inhibitors. We identified 72 women and 36 men (several papers did not specify the gender of patients) with an age range of 13-78 years (mean 42.3 years). The primary diagnoses were rheumatoid arthritis (n = 61), ankylosing spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFalpha treatment (n = 73), followed by palmoplantar pustular psoriasis (n = 37) and psoriasis of the nail (n = 6), sometimes combined in the same patient. Palmoplantar pustulosis and psoriasiform exanthema was the diagnosis in ten patients each. A positive personal history of psoriasis was recorded in 25 patients. A positive family history was noted in eight patients. No data about personal (n = 7) or family history (n = 46) were available in a number of patients. Newly induced psoriasis was diagnosed in 74 patients whereas an exacerbation or aggravation of a pre-existing psoriasis was noted in another 25 patients. All three TNFalpha inhibitors available on the market were involved: infliximab (63 patients), etanercept (37 patients), and adalimumab (26 patients). Several patients were treated with more than a single TFNalpha inhibitor. The timing of cutaneous adverse effects (psoriasis and psoriasiform rash) varied considerably among patients, ranging from after a single application to a delayed response of up to 63 months after initiation of treatment. The mean time to appearance of the cutaneous adverse effect for all TNFalpha inhibitors was 9.5 months. Cessation of the responsible TNFalpha inhibitor was carried out in 47 patients either alone or in association with adjuvant anti-psoriatic therapy (mostly topical). This resulted in complete remission in 21 patients, partial remission in 20 patients, and stable disease in another three patients; in the other three patients, the outcome was not reported. TNFalpha inhibition was continued in 47 patients but anti-psoriatic adjuvant therapy was introduced. The outcome in this group was complete remission in 22 patients, partial remission in 25 patients, and stable disease in 2 patients. The response rate (complete remission plus partial remission) was 93.2% and 95.9%, respectively, in each group. In six patients, switching from one TNFalpha inhibitor to another one immediately after cutaneous adverse effects occurred resulted in an improvement in five patients. In nine patients, a second TNFalpha inhibitor was initiated after a break in TNFalpha inhibition. The response to a second or third drug in these patients was mixed. The underlying pathomechanisms of induction of psoriasis or psoriasiform exanthemata by TNFalpha inhibitors remain elusive but there is reason to assume that induction of such adverse events has more than one pathophysiology.

Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma.

Disabling pansclerotic morphea of childhood (DPMC) is a rare and severe variant of scleroderma. This report presents 3 cases that presented to the authors and studies 25 patients from the literature (English language only) for the presence of chronic nonhealing ulcers of skin and skin cancer. The authors identified a total of 30 patients (9 male and 21 female) aged between 1 and 37 years at time of presentation. All cases were less than 14 years old when the disease started. The majority of patients had an aggressive course with deep sclerotic lesions leading to joint contractures and immobility. Five patients suffered from chronic nonhealing leg ulcers (17%), but ulcers were present on other parts of the body (upper limbs, trunk, head) as well (n = 6). Four patients died because of complications of the disease such as sepsis or gangrene. Two patients developed a squamous cell carcinoma at the age of 16 years and 19 years, respectively (6.7%). The available treatment of DMPC-associated ulcers is unsatisfying. Only temporary improvements have been seen in a minority of patients. We report on marked improvement of chronic leg ulcers by a combination of sildenafil 3 x 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix.

Painful ANA-positive scleroderma-like disease with acral ulcerations: a case of chronic gangrenous ergotism.

Chronic ergotism is a rare cause of limb ischemia. In this case report, the authors present a 62-year-old woman with history of long-term use of ergotamine alkaloids for the treatment of menstrual pain, who developed a severe painful disease initially misdiagnosed as systemic sclerosis (scleroderma) for 3 decades. She presented with a combination of acral gangrene, foot ulcer, renal obstruction, mild pulmonary fibrosis, and reduced esophageal motility. Right-sided renal obstruction was evident. The condition was extremely painful and had led to muscular contractions and immobility, drug abuse, and anemia. After establishing the diagnosis of chronic gangrenous ergotism, changing drug therapy, mobilization, and treatment of chronic wounds, she showed a remarkable recovery. Eventually the foot ulcer was closed successfully using a mesh graft transplantation, and the patient was able to walk alone. Chronic ergotism is rare but has to be taken into account when presented with painful chronic digital and foot ulcers.

Tattoos: surgical removal.

Surgical treatment of tattoos remains a useful tool for complete removal despite the availability of laser and other nonsurgical techniques. The procedures are in accordance with standard dermatosurgery used in aesthetic and oncologic therapies. Healing by primary intention or healing by secondary intention has its own indications. Skin defects can be closed by flaps, grafts, tissue extension, or tissue expansion.

Glabellar pomade crust mimicking dyskeratosis follicularis.

A 79-year-old female patient presented with a slowly developing crusting, itching verrucous lesion of the forehead of 3 months' duration. She had no personal or family history of skin disease. On examination she presented with a hyperpigmented plaque of the glabellar region that resembled dyskeratosis follicularis Darier. A detailed medical history was taken and a skin biopsy was performed. Epidermal acanthosis and enlargement of follicle ostia with foreign material were found. The patient reported use of an ointment twice daily. She had a single cosmetic treatment where powder of unspecified composition had been used. The diagnosis of pomade crust was confirmed. Follicular material was enucleated mechanically and the area was treated with a metronidazole cream. The lesions completely disappeared.

Cutaneous manifestations of metabolic diseases: uncommon presentations.

Metabolic diseases are common diseases in the Western world. Many of these diseases, including diabetes mellitus, hyperlipoproteinemia, gout, calcinosis, and hemochromatosis, are associated with skin diseases or often present with specific cutaneous signs. A knowledge of cutaneous manifestations helps to identify patients at risk, establish the internal diagnosis, and monitor the adverse effects of therapy.

Case reports: acne fulminans in Marfan syndrome.

We report on a 12-year-old boy suffering from acne fulminans in combination with Marfan syndrome. The trigger for acne induction seemed to be a testosterone therapy. The particular therapeutic problems in the present case are described. No acne keloids were observed but atrophic scars that may be due to Marfan syndrome.

Leg ulcers are a diagnostic and therapeutic challenge.

Leg ulcers are common. They cause a substantial burden to the patient and society. However, there is no need for therapeutic nihilism. The target of leg ulcer therapy is the individual patient. To be treated in a rational and successful way, exact diagnosis of the underlying cause(s) and associated diseases is necessary. This can be done in the most effective way with an interdisciplinary approach. The collection of cases demonstrates the need for careful clinical investigation substantiated and supported by vascular, histopathologic, and microbiologic techniques whenever needed. It is difficult to heal every ulcer completely, but improvement of the medical situation as well as the quality of life of the patient is possible in most cases.

Therapy of porphyria cutanea tarda.

Porphyria cutanea tarda (PCT) is the most common type of porphyria. There is an association of PCT with haemochromatosis, diabetes mellitus and hepatitis C infection. The basis of treatment of PCT consists of three elements: avoidance of triggering factors, iron depletion and porphyrin elimination. Alcohol and certain systemic medical drugs, such as oestrogens (or tuberculostatics), should be considered as triggering factors, and as far as possible, avoided. Other triggering factors, such as chronic haemodialysis in renal insufficiency, need a different approach. The hallmark in iron depletion is phlebotomy. Porphyrin elimination is achieved using low-dose chloroquin therapy. The treatment is safe and effective but has its limits in cases with haemochromatosis (HFE) gene mutations. Here iron depletion needs additional phlebotomy. In patients with chronic haemodialysis-associated PCT, chloroquine is ineffective. Erythropoietin, desferroxamine and small-volume phlebotomy have been employed to control the disease. Childhood PCT is very rare. No controlled studies are available, but published experience suggests that body weight-adapted chloroquine therapy or small volume phlebotomy might be useful.

Oral capecitabine plus subcutaneous interferon alpha in advanced squamous cell carcinoma of the skin.

PURPOSE: Squamous cell carcinoma of the skin (SSC) is a UV-damage-related skin tumor. The first-line treatment is surgery, which has a high cure rate. In advanced cases, however, the established treatment is often not curative and shows a high rate of side effects. Improved treatment modalities are necessary. METHODS: Oral capecitabine plus subcutaneous interferon alpha were used in a prospective case series in advanced SSC of the skin at an academic teaching hospital for dermatology. Four patients with advanced SCC were included. Capecitabine 950 mgm(-2) body surface on days 1 to 14 was combined with interferon alpha 3x3 mioU s.c. three times a week. The chemotherapy was repeated on day 22. Clinical response, histology, monitoring of side effects and health performance status were assessed. RESULTS: Four patients (two females and two males) with advanced SCC were included (age range: 19 to 75 years). Complete remission (CR) was obtained in two and partial response (PR) in two. The final outcome was CR in two and progressive disease in one. One patient died of an unknown cause. Side effects were mild. Adjuvant treatment was unnecessary. Health performance status was not affected by the treatment. CONCLUSIONS: The treatment protocol with a combination of capecitabine and interferon alpha seems to be effective and well tolerated in patients with advanced SCC. Controlled trials are necessary to prove the benefit we observed in this case series.

Hemochromatosis (HFE) gene mutations and response to chloroquine in porphyria cutanea tarda.

OBJECTIVE: To examine the role of hemochromatosis (HFE) gene mutations, which are associated with porphyria cutanea tarda (PCT), in the therapeutic response to chloroquine. DESIGN: We retrospectively analyzed a database (Excel version 2001 [Microsoft Excel, Redmond, Wash]; date range of search, 1985-1999) of chloroquine-treated patients with PCT on whether HFE mutations (C282Y and H63D) might have influenced the clinical response, urinary porphyrin excretion, liver enzyme activities, and serum iron markers. Serum samples and corresponding complete sets of data before and after therapy were available in 62 of 207 patients with PCT who were treated exclusively with chloroquine. SETTINGS: Academic teaching hospital. INTERVENTION: For treatment, low-dose chloroquine diphosphate, 125 to 250 mg twice weekly, was used during a median time of 16 months (range, 12-26 months). RESULTS: Of the 62 German patients with PCT, 37 (60%) carries HFE mutations. Chloroquine therapy was accompanied by clinical remission and reduced urinary porphyrin excretion (P<.001) in the 24 patients (39%) with HFE wild type as well as in 35 HFE heterozygous patients with PCT (56%). Decreases of serum iron markers following chloroquine therapy were limited to patients with PCT and HFE wild type. All patients homozygous for the C282Y mutation (3 [5%] of 62) had high serum iron, ferritin, and transferrin saturation and failed to respond to chloroquine treatment. CONCLUSIONS: The therapeutic response to chloroquine was not compromised by C282Y heterozygosity and compound heterozygosity of HFE mutations. Because HFE C282Y homozygotes (+/+) did not respond to chloroquine and a decrease in serum iron concentration was limited to patients with PCT and HFE wild type, phlebotomy should be first-line therapy in patients with PCT and HFE mutations.

Ulcerated necrobiosis lipoidica: a combined treatment approach with dermatosurgery and PUVA.

Ulcerated necrobiosis lipoidica is one of the differential diagnoses in leg ulcers. The diagnosis is confirmed by histopathology. The authors report on a 68-year-old female patient with a history of chronic venous insufficiency who developed a chronic leg ulcer that did not respond to good ulcer care and compression bandaging. Skin biopsies revealed necrobiosis lipoidica. The patient was recently discovered to have diabetes mellitus that was treated orally by ascarose. Phlebosurgery and mesh grafting were performed, and the patient was given pentoxyfiline, dapsone, and clofazimine, but none of these treatments was successful. Therefore, topical PUVA therapy was introduced that resulted in partial remission and stabilizing of the residual pathology. Based on these experiences, the use of surgical methods alone is questionable.

Autoimmunity and HCV infection in porphyria cutanea tarda: a controlled study.

Autoimmunity and high rates of autoantibodies have been implicated in the pathogenesis of porphyria cutanea tarda. These abnormalities could be in part virus-induced, since porphyria cutanea tarda in most geographical regions is highly associated with hepatitis C virus infection. We analyzed the link of autoantibodies, autoimmune hepatitis and systemic lupus erythematosus in 111 patients with porphyria cutanea tarda and sex- and age-matched controls (mean age 58+/-13 years) in Germany, a region with a low prevalence of hepatitis C virus infection. Patients with porphyria cutanea tarda displayed lower rates of anti-nuclear antibodies (16/111, 14% vs 28/111, 25%, p<0,05) and of antibodies against smooth muscle (25/111, 23% vs 48/111, 43%, p<0,01), than controls. The percentage of patients with porphyria cutanea tarda with positive anti-HCV was low but significantly higher than in our controls (9/111, 8% vs 0/111, 0%, respectively), (p<0,05). Two patients with porphyria cutanea tarda (2/111, 2%) fulfilled the criteria for systemic lupus erythematosus and not one of 65 patients was found to have clinical autoimmune hepatitis. In the first controlled study of a large cohort of patients with porphyria cutanea tarda no increased prevalence of selected autoantibodies and autoimmune hepatitis was found. However, a higher prevalence of HCV infection and systemic lupus erythematosus in patients with porphyria cutanea tarda was confirmed.