Leprosy: two case reports from Dresden, Germany.
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Adult↗
Biomedical subjects
Erich Köstler
Publications and source records attributed to Erich Köstler.
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.
Aged↗
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.
Adult↗
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.
Chloroquine↗
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.
Adult↗
Gabapentin-induced generalized cutaneous small-vessel leukocytoclastic vasculitis.
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Letter↗
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.
Adult↗
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.
Journal Article↗
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.
Adult↗
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.
Calcinosis↗
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.
Acne Vulgaris↗