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Biomedical subjects

J Schönlebe

Publications and source records attributed to J Schönlebe.

13 recordsLinked to original sources

Cutaneous Langerhans cell histiocytosis with subsequent development of haematological malignancies. Report of two cases.

Adult cutaneous Langerhans cell histiocytosis (LCH) is a rare disease. We report two cases illustrating the variability of the clinical presentation and the response to treatment. In both cases a remission was achieved: in one case a partial remission with psoralen plus UVA irradiation (PUVA) and methotrexate plus topical corticosteroid ointment; in the other case by treatment with thalidomide. Despite a therapeutic response, both patients later developed haematological malignancies: a chronic myelo-monocytic leukaemia and an acute lymphatic leukaemia. In conclusion, patients with adult cutaneous LCH should be monitored carefully so that a secondary malignancy is not overlooked.

Adult↗

[Acute liver failure caused by phenprocoumon -- three case reports].

We report on three women with phenprocoumon-induced liver failure. In all three, jaundice was the main symptom. Liver failure was manifest 6 months after initial exposure to phenprocoumon in two women and 18 months in the third. None received corticosteroids. The course was prolonged in each patient, but all three finally recovered. Liver failure associated with oral anticoagulation is rare. Most published reports describe only individual cases. Instances of fatal outcome and instances requiring liver transplantation for survival have been seen. Coumarin treatment should be considered as a possible etiology of acute liver failure, even if the drug has been administered for a longer period without any previous problems. While cross-reactions with other coumarin substances have been reported, if coumarin anticoagulation is absolutely required, use of a congener under close monitoring seems justifiable.

Aged↗

Lipoid proteinosis.

The case of a 37-year-old woman with lipoid proteinosis is presented. The first symptoms of hoarseness of voice occurred in puberty. Additional symptoms included beaded eyelid papules, macroglossia with yellowish papules, yellowish deposits of soft palate, popular eruption in the axillary area and tuberous nodules on the elbows.

Journal Article↗

Liposomal daunorubicin in tumor stage cutaneous T-cell lymphoma: report of three cases.

PURPOSE: Advanced cutaneous T-cell lymphoma (CTCL) is a hard-to-treat condition. Complete response is rare even with polychemotherapy. The use of liposomal formulation anti-cancer drugs can improve the efficacy and the risk-benefit ratio. Liposomal doxorubicin was shown to be effective as a second-line treatment in CTCL. There is no data available on another classical anthracycline, daunorubicin, when given in liposomal formulation as a monotherapy. METHODS: Monotherapy with liposomal-encapsulated daunorubicin (DNX) was given as a monotherapy once a month at 20 mg/m(2) three times to achieve a clinical response. In the case of limited response the drug was given once every 3 weeks and a dose increase was performed. Three patients were treated. RESULTS: A complete response was achieved in one patient (dosage 20 mg/m(2 )once per month). Two other patients achieved a partial response. The final outcome was disease-free survival of more than 10 months in the patient with a complete response and survival of >8 months and 6 months in those with a partial response. Adverse effects were grade 4 anemia in one patient, lymphopenia grade 2 with grade 1 anemia, and grade 1 lymphopenia in the other patients. CONCLUSION: This is the first report on DNX monotherapy in CTCL. In a small group of three patients a response rate of 100% was achieved with one complete response. DNX seems to be another option in advanced cases of CTCL.

Adult↗

Interstitial granulomatous dermatitis with plaques and arthritis.

Interstitial granulomatous dermatitis and arthritis (IGDA) is a rare disease entity with female predominance. The case of a 53-year-old woman with erythemas, plaques and nodules associated with polyarthritis is presented. She was treated with cyclosporin A, with improvement of the joint affliction and complete clearance of skin lesions. The differential diagnosis of IGDA is discussed briefly.

Arthritis↗

Mycological and histological investigations in humans with middle ear infections.

The aim of our investigations was to characterize fungal colonization of the ear in immunocompetent patients. From 1993 to 2000, 128 patients supposed to suffer from otomycosis were included. Mycological examination conducted by direct microscopy and fungal cultures was performed on 139 specimens. Among these, 115 patients suffered from chronic otitis media with persisting tympanum perforation and otorrhea. A further 13 patients had clinical signs of an otitis externa only. Out of 139 samples, fungi were identified in the auditory canal (n = 54), on the tympanic membrane (n = 5), and in the middle ear (n = 5). Two-thirds were as moulds and one-third yeasts. The dominating species were Aspergillus niger and Candida parapsilosis. Samples from 15 patients supposed to have mastoiditis or cholesteatoma were examined histologically. Fungal hyphae were observed in the middle ear cavity and/or between horny lamellae of cholesteatoma in four patients. In the middle ear of immunocompetent patients chronic-hyperplastic (polypoid) inflammation was detected with increased production of mucus, which probably promotes colonization by pathogenic fungi in the middle ear as well as in the auditory canal. Invasive fungal growth into the subepithelial connective tissue was not observed.

Adult↗

Merkel cell carcinoma--a retrospective analysis of 17 cases.

OBJECTIVE: To report clinical experience with the rare neuroendocrine Merkel cell carcinoma of the skin. SUBJECTS AND SETTING: Seventeen patients with Merkel cell carcinoma of the skin treated at the Departments of Dermatology and ENT, Krankenhaus Dresden-Friedrichstadt, Dresden, Germany, during the years 1984-2000 were evaluated. METHODS: A retrospective analysis was performed. Age and sex distribution, clinical data and therapy were collected. Outcome measures including overall survival, tumour-free survival and relapse-free survival were determined. RESULTS: Six male and 11 female patients with an age range of 68-90 years (mean age 73.3 years) were identified. The primary tumour localization was head and neck region (n = 8), upper limbs (8), lower limbs (1). Twelve patients presented in tumour stage I, three in stage II and one in stage III. First line therapy was complete surgical excision with wide margins in 16 patients followed by loco-regional radiation in 12 of 16 cases. In 16 patients follow up data were available. After primary treatment complete response was achieved in 14 of 16 patients (87.5%), two patients had a partial response. The median of relapse-free survival was 44 weeks [mean +/- standard deviation: (44 +/- 118) weeks]. The median of overall survival was 102 weeks [mean +/- standard deviation: (137 +/- 94) weeks]. Three patients with a PR after primary treatment had a median overall survival of only 48 weeks [mean +/- standard deviation: (51 +/- 20) weeks]. CONCLUSIONS: Primary surgical treatment with wide excision combined with radiotherapy seems to be a reasonable first-line treatment but prospective controlled multicentre trials are necessary for validation.

Aged↗

Polydactylous Bowen's disease.

Malignant nail tumours are rare. We report on a 52-year-old patient with chronic verrucous lesions of the nail bed of fingers and toes. Nail biopsy revealed polydactylous Bowen's disease. This is the first case reported with hand and foot lesions.

Bowen's Disease↗

Mineral oil granuloma of the penis.

We report a 30-year-old-patient with penile sclerosing lipogranuloma following injections of Vaseline. He was successfully treated by complete surgical removal of the subcutaneous material and excision of atrophic skin, followed by penoplasty. Temporary impairment of wound healing was overcome by application of topical carbon dioxide gas. A brief review on international literature is given.

Adult↗

[Histologic studies on otomycosis].

Fungal infections of the ear are mostly described as mycoses of the auditory canal. The aim of our investigations was to find out how fungi colonize the ear in immunocompetent patients. In the years from 1993 to 2000, 128 patients suspected of having otomycosis were examined. Of these 115 patients suffered from chronic otitis media with persisting tympanum perforation and otorrhea. A further 13 patients had clinical signs of otitis externa only. In 54 out of 139 samples, fungi were found in the auditory canal, in five on the tympanic membrane, and in five in the middle ear. Two-thirds were isolated as moulds and one-third as yeasts. Dominating species were Aspergillus niger and Candida parapsilosis. Samples of 15 patients suspected of having mastoiditis or cholesteatoma were examined histologically. Fungal hyphae were observed in the middle ear cavity and/or between horny lamellae of cholesteatoma in 4 patients. In the middle ear of immunocompetent patients chronic-hyperplastic (polypous) inflammation was detected with increased production of mucus, which probably promotes the colonization with pathogenic fungi as in the middle ear just like in the auditory canal.

Aspergillosis↗

Gastric wall abscess, a rare endosonographic differential diagnosis of intramural tumors: successful endoscopic treatment.

Abscesses in the gastric wall are extremely rare. As the mucosa remains intact in most cases, clear differential diagnosis is required in order to distinguish the condition from the more frequent intramural mesenchymal tumors. Endoscopic ultrasonography provides a valuable tool for imaging intramural tumors, but this approach does not allow a definitive assessment of malignancy. We report about two patients with intramural abscesses in the gastric wall. Endosonography showing eccentric tumors from the gastric wall in the two patients. The endosonographic image was inhomogenous, the wall layer structure of the gastric wall was not preserved. A laparotomy was carried out on the first patient. In the second case, the diagnosis was easy, as pus was emptying from a small opening in the mucosa, which had already been detected at gastroscopy. Endoscopic intervention was carried out based on the endosonographic findings. In one patient, mucosa and submucosa were opened by a needle knife. These cases show that gastric wall abscesses do not have a typical endoscopic ultrasound appearance. However, endosonography is an essential method prior to endoscopic interventional therapy.

Abscess↗

[Microcystic adenoma of the pancreas--a case report].

A 65-year-old female patient with microcystic adenoma of the pancreatic head is reported. Under suspicion of a malignant pancreatic tumour a kephalic duodenopancreatectomy (Kausch-Whipple) was performed. Diagnosis, therapy, prognosis and pathological anatomical aspects of benign microcystic adenoma are discussed.

Adenoma↗