BACKGROUND AND OBJECTIVES: Diagnosis-related groups of medical services has recently been introdu... more BACKGROUND AND OBJECTIVES: Diagnosis-related groups of medical services has recently been introduced for reimbursing hospital services. The aim has been to optimize bed capacity, decrease the duration of inpatient stay and provide good follow-up medical care. This confronts hospitals with the need for closely cooperating with the referring general practitioners or specialists. METHOD: A specially structured questionnaire was sent to all those general practitioners and specialists (n=890) who had referred patients to a university department of dermatology. The response rate was 23%. The completed questionnaires were analysed with respect to criteria judged to be important in determining referral for inpatient care and the quality of medical and related service provisions. RESULTS: Especially important to the referring doctors were optimal competence of patient care, the degree of cooperation and prompt, detailed information after the patients have been discharged. CONCLUSION: This type of analysis can serve to optimize the course of diagnosis/treatment and to utilize fully the available hospital resources. Also considered are various ways in which a hospital department can, by networking with general practitioners and specialists, become a centre for providing optimal services.
Journal der Deutschen Dermatologischen Gesellschaft, 2009
Albright hereditary osteodystrophy (AHO), also known as Martin-Albright syndrome (MAS), is a rare... more Albright hereditary osteodystrophy (AHO), also known as Martin-Albright syndrome (MAS), is a rare autosomal dominantly transmitted disease characterized by short stature, obesity, mental retardation, a round facies, and brachymetacarpia and -tarsia, as well as cutaneous calcification. The disease is caused by mutations in the GNAS gene localized on chromosome 20q13.2 encoding for an adenyl-cyclase-stimulating protein (Gsalpha). A 58-year-old patient presented with small stature since childhood, moderate mental retardation, round facies and soft tissue masses on the thighs. A biopsy of the latter showed subcutaneous ossification. Laboratory results showed hypocalcemia, as well as increased plasma levels of PTH and calcitonin. The clinical diagnosis was confirmed by detection of reduced activity of Gsalpha. In patients with cutaneous calcification and disturbed calcium metabolism, AHO is an important differential diagnostic consideration.
Clinical and Experimental Dermatology, Mar 21, 2011
We present the case of a 79-year-old patient with extensive metastatic malignant melanoma (MM) of... more We present the case of a 79-year-old patient with extensive metastatic malignant melanoma (MM) of the scalp. Cutaneous MM of the head and neck often presents a therapeutic challenge. Radical surgical procedures and conventional chemotherapy are often unfeasible and contraindicated because of the difficult anatomy, the extent of the tumour process, and systemic toxicity. In our patient, selective intra-arterial perfusion with pegylated liposomal doxorubicin (PLD) and melphalan was performed after catheterization of both bilateral external carotid arteries with an arterial port system. PLD 4.5 mg/m(2) and melphalan (1.35 mg/m(2), followed by 2.7 mg/m(2) after reaching tolerance) were given as short-term infusions at two-weekly intervals into the right and left external carotid arteries, respectively. After eight applications with tolerable side-effects, no MM cells were detected; however, infiltrates of lymphocytes and melanophages were seen. This case suggests that intra-arterial chemotherapy may be a useful treatment for metastatic melanoma of the scalp.
miR-203 regulates in vivo lung metastasis without triggering differentiation Restoring miR-203 in... more miR-203 regulates in vivo lung metastasis without triggering differentiation Restoring miR-203 in already established metastases elicits regression LASP1, NUAK1, and SPARC are downstream prometastatic ef-fectors of miR-203 ic of over-
Journal of the European Academy of Dermatology and Venereology, 2016
Case report A 50-year-old patient presented with severe scarring alopecia, poikilodermia and skin... more Case report A 50-year-old patient presented with severe scarring alopecia, poikilodermia and skin sclerosis of the head, neck and chest, lower arms and hands (Fig. 1). Small skin blisters, erosions and ulcers on the head and hands became recurrently infected. The patient complained about mouth and hand rigidity, finger pain at low temperatures, hypersensitivity to sunlight in recent years and recently, red urine colouring. The patient was not receiving any medication and had no known family member with similar signs or symptoms. In the last 15 years, he worked as a tiler, turner, window fitter, scrap burner or forklift driver; he had contact with mineral oils, lubricants and organic solvents and was frequently exposed to sunlight. Fearing unemployment, he never visited a physician. In addition, the patient drank up to 1.5 L of beer daily. Paraclinical tests showed mildly increased transaminases, gamma-GT, carbo-deficient-transferrin (CDT 4%; normal range:<2.6) and borrelia-specific IgG-bands on western blot. Values for differential blood count, creatinine, iron and copper were normal. Hepatitis serology was negative except for an inactive hepatitis A infection. Dermatohistology of biopsies from cheeks, head and chest showed severe sclerodermoid changes, plasma cell expansion and focal calcinosis. Additional findings included calcification of cardiac valves, gingival retraction and UVA/B-hypersensitivity. The urine was lightly red-coloured under daylight and showed bright red fluorescence under Wood’s light. Urine analysis demonstrated increased values for total porphyrin (2871 lg/day, range: <150), uroporphyrin-I (1560 lg/day, range: 3–24), hepta-, hexa-, pentacarboxyporphyrin (621 lg/day, range: 0–3; 84 lg/day, range: 0–6; 87 lg/day, range: 0–6 respectively) and coproporphyrin (208 lg/day, range: 14–78), and normal values for protoporphyrin, delta-aminolaevulinic acid, total faecal porphyrin and free protoporphyrin and zinc-protoporphyrin in erythrocytes. Uroporphyrinogen-decarboxylase activity was low (87%, range:>80%). Histopathology of the liver biopsy showed mild periportal fibrosis and mild damage to the parenchyma, but no signs of alcoholic cirrhosis, biliary cirrhosis, Morbus Wilson, alpha-1-antitrypsin deficiency, haemochromatosis or hepatitis B/C. However, uroporphyrin crystals, usually restricted to liver tissue could be detected in the blood (Fig. 2b). Moderately increased CDT levels indicated repeated alcohol consumption; however, taken together with normal abdominal ultrasound and liver histopathology, liver cirrhosis was excluded. PCR from liver and skin tissue for Borrelia burgdorferi was negative. Increased serum levels for antinuclear antibodies (1 : 160) and anti-smooth muscle antibodies (1 : 320), together with the previous findings, indicated mild autoimmune hepatitis Type I, with a score of 13 (normal <11). HFE gene mutations at C282Y and H63D were excluded by gene sequencing. The patient was treated with chloroquine at 2 9 125 mg/week, strict alcohol abstinence and protection from sunlight. Porphyrin values normalized within 9 months (Fig. 2c). Skin lesions healed under additional antiseptic wound management and surgical removal of subcutaneous calcifications. Porphyria cutanea tarda (PCT), the most common porphyria, results from decreased activity of uroporphyrinogen-decarboxylase. In the acquired form, in contrast to autosomal dominant hereditary cases, enzyme activity is decreased only in the liver, but not in erythrocytes. Primary causes of acquired PCT are liver diseases such as alcohol abuse-induced cirrhosis, hepatitis C infection, primary biliary cirrhosis, polychlorinated hydrocarbon intoxication, haemochromatosis or autoimmune hepatitis. Cutaneous manifestations of PCT include increased skin photosensitivity with tense blisters, erosions, milia and scars in (a) (b)
BACKGROUND AND OBJECTIVES: Diagnosis-related groups of medical services has recently been introdu... more BACKGROUND AND OBJECTIVES: Diagnosis-related groups of medical services has recently been introduced for reimbursing hospital services. The aim has been to optimize bed capacity, decrease the duration of inpatient stay and provide good follow-up medical care. This confronts hospitals with the need for closely cooperating with the referring general practitioners or specialists. METHOD: A specially structured questionnaire was sent to all those general practitioners and specialists (n=890) who had referred patients to a university department of dermatology. The response rate was 23%. The completed questionnaires were analysed with respect to criteria judged to be important in determining referral for inpatient care and the quality of medical and related service provisions. RESULTS: Especially important to the referring doctors were optimal competence of patient care, the degree of cooperation and prompt, detailed information after the patients have been discharged. CONCLUSION: This type of analysis can serve to optimize the course of diagnosis/treatment and to utilize fully the available hospital resources. Also considered are various ways in which a hospital department can, by networking with general practitioners and specialists, become a centre for providing optimal services.
Journal der Deutschen Dermatologischen Gesellschaft, 2009
Albright hereditary osteodystrophy (AHO), also known as Martin-Albright syndrome (MAS), is a rare... more Albright hereditary osteodystrophy (AHO), also known as Martin-Albright syndrome (MAS), is a rare autosomal dominantly transmitted disease characterized by short stature, obesity, mental retardation, a round facies, and brachymetacarpia and -tarsia, as well as cutaneous calcification. The disease is caused by mutations in the GNAS gene localized on chromosome 20q13.2 encoding for an adenyl-cyclase-stimulating protein (Gsalpha). A 58-year-old patient presented with small stature since childhood, moderate mental retardation, round facies and soft tissue masses on the thighs. A biopsy of the latter showed subcutaneous ossification. Laboratory results showed hypocalcemia, as well as increased plasma levels of PTH and calcitonin. The clinical diagnosis was confirmed by detection of reduced activity of Gsalpha. In patients with cutaneous calcification and disturbed calcium metabolism, AHO is an important differential diagnostic consideration.
Clinical and Experimental Dermatology, Mar 21, 2011
We present the case of a 79-year-old patient with extensive metastatic malignant melanoma (MM) of... more We present the case of a 79-year-old patient with extensive metastatic malignant melanoma (MM) of the scalp. Cutaneous MM of the head and neck often presents a therapeutic challenge. Radical surgical procedures and conventional chemotherapy are often unfeasible and contraindicated because of the difficult anatomy, the extent of the tumour process, and systemic toxicity. In our patient, selective intra-arterial perfusion with pegylated liposomal doxorubicin (PLD) and melphalan was performed after catheterization of both bilateral external carotid arteries with an arterial port system. PLD 4.5 mg/m(2) and melphalan (1.35 mg/m(2), followed by 2.7 mg/m(2) after reaching tolerance) were given as short-term infusions at two-weekly intervals into the right and left external carotid arteries, respectively. After eight applications with tolerable side-effects, no MM cells were detected; however, infiltrates of lymphocytes and melanophages were seen. This case suggests that intra-arterial chemotherapy may be a useful treatment for metastatic melanoma of the scalp.
miR-203 regulates in vivo lung metastasis without triggering differentiation Restoring miR-203 in... more miR-203 regulates in vivo lung metastasis without triggering differentiation Restoring miR-203 in already established metastases elicits regression LASP1, NUAK1, and SPARC are downstream prometastatic ef-fectors of miR-203 ic of over-
Journal of the European Academy of Dermatology and Venereology, 2016
Case report A 50-year-old patient presented with severe scarring alopecia, poikilodermia and skin... more Case report A 50-year-old patient presented with severe scarring alopecia, poikilodermia and skin sclerosis of the head, neck and chest, lower arms and hands (Fig. 1). Small skin blisters, erosions and ulcers on the head and hands became recurrently infected. The patient complained about mouth and hand rigidity, finger pain at low temperatures, hypersensitivity to sunlight in recent years and recently, red urine colouring. The patient was not receiving any medication and had no known family member with similar signs or symptoms. In the last 15 years, he worked as a tiler, turner, window fitter, scrap burner or forklift driver; he had contact with mineral oils, lubricants and organic solvents and was frequently exposed to sunlight. Fearing unemployment, he never visited a physician. In addition, the patient drank up to 1.5 L of beer daily. Paraclinical tests showed mildly increased transaminases, gamma-GT, carbo-deficient-transferrin (CDT 4%; normal range:<2.6) and borrelia-specific IgG-bands on western blot. Values for differential blood count, creatinine, iron and copper were normal. Hepatitis serology was negative except for an inactive hepatitis A infection. Dermatohistology of biopsies from cheeks, head and chest showed severe sclerodermoid changes, plasma cell expansion and focal calcinosis. Additional findings included calcification of cardiac valves, gingival retraction and UVA/B-hypersensitivity. The urine was lightly red-coloured under daylight and showed bright red fluorescence under Wood’s light. Urine analysis demonstrated increased values for total porphyrin (2871 lg/day, range: <150), uroporphyrin-I (1560 lg/day, range: 3–24), hepta-, hexa-, pentacarboxyporphyrin (621 lg/day, range: 0–3; 84 lg/day, range: 0–6; 87 lg/day, range: 0–6 respectively) and coproporphyrin (208 lg/day, range: 14–78), and normal values for protoporphyrin, delta-aminolaevulinic acid, total faecal porphyrin and free protoporphyrin and zinc-protoporphyrin in erythrocytes. Uroporphyrinogen-decarboxylase activity was low (87%, range:>80%). Histopathology of the liver biopsy showed mild periportal fibrosis and mild damage to the parenchyma, but no signs of alcoholic cirrhosis, biliary cirrhosis, Morbus Wilson, alpha-1-antitrypsin deficiency, haemochromatosis or hepatitis B/C. However, uroporphyrin crystals, usually restricted to liver tissue could be detected in the blood (Fig. 2b). Moderately increased CDT levels indicated repeated alcohol consumption; however, taken together with normal abdominal ultrasound and liver histopathology, liver cirrhosis was excluded. PCR from liver and skin tissue for Borrelia burgdorferi was negative. Increased serum levels for antinuclear antibodies (1 : 160) and anti-smooth muscle antibodies (1 : 320), together with the previous findings, indicated mild autoimmune hepatitis Type I, with a score of 13 (normal <11). HFE gene mutations at C282Y and H63D were excluded by gene sequencing. The patient was treated with chloroquine at 2 9 125 mg/week, strict alcohol abstinence and protection from sunlight. Porphyrin values normalized within 9 months (Fig. 2c). Skin lesions healed under additional antiseptic wound management and surgical removal of subcutaneous calcifications. Porphyria cutanea tarda (PCT), the most common porphyria, results from decreased activity of uroporphyrinogen-decarboxylase. In the acquired form, in contrast to autosomal dominant hereditary cases, enzyme activity is decreased only in the liver, but not in erythrocytes. Primary causes of acquired PCT are liver diseases such as alcohol abuse-induced cirrhosis, hepatitis C infection, primary biliary cirrhosis, polychlorinated hydrocarbon intoxication, haemochromatosis or autoimmune hepatitis. Cutaneous manifestations of PCT include increased skin photosensitivity with tense blisters, erosions, milia and scars in (a) (b)
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