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Biomedical subjects

W Ch Marsch

Publications and source records attributed to W Ch Marsch.

7 recordsLinked to original sources

[Plaques opalines: a rare form of secondary syphilis of the oral mucous membrane].

A 37 year old woman presented with a 4-month history of "plaques opalines" of the oral mucosa complaining of a rough feeling in her mouth and burning when swallowing. On the basis of clinical, histological and serological details and especially the presence of generalized indolent lymphadenopathy, the diagnosis of secondary syphilis was made. Although plaques opalines are a relatively rare manifestation of secondary syphilis, they can occasionally be the only sign and are important in the differential diagnostic considerations of gray-white mucosal lesions, especially with current continuing rise of syphilis incidence in Central Europe.

Adult↗

[Angioedema in hereditary deficiency of complement factor 1 esterase inhibitor and alpha 1-antitrypsin].

HISTORY AND CLINICAL FINDINGS: A 15-year-old girl had suffered from episodic, sometimes threatening angioedema of the face, nasopharyngeal space and distal extremities beginning at age 13. EXAMINATIONS: A C1-esterase inhibitor (C1-INH) deficiency was revealed protein-chemically and functionally. There was also an alpha (1)-antitrypsin (AAT) deficiency with heterocygotic phenotype PiMZ. The combination of C1-INH and AAT deficiency was also found in the patient's mother and brother. THERAPY AND COURSE: Under 8-month therapy with 200 mg/d danazol per os (reduction of the dosis in the last month to 100 mg/d), there was no further edema, the C1-INH concentration normalized and there was also an increase in C1-INH function. During the observation period, use of the emergency set with C1-INH concentrate was not required. CONCLUSIONS: This is the first reported case of angioedema in combination of two hereditary enzyme defects C1-INH deficiency (autosomal-dominant genetics) and AAT deficiency (autosomal-recessive). In addition to a survey of current literature, the current state of diagnostics and therapy of the hereditary angioedema is presented.

Administration, Oral↗

[Human cowpox/catpox infection. A potentially unrecognized disease].

HISTORY AND ADMISSION FINDINGS: A 36-year-old woman initially noticed a red spot, about pea-sized, with a central pimple over the right eyebrow and a swollen submandibular lymph node. A pressure-sensitive, 4 cm large, node developed out of this small spot, with a central, black, tightly-adhering crust bearing several varioliform vesicles around its edge. In addition to swelling of the right half of the face, the patient had a fever up to 39.5 degrees C, general malaise, nausea and vomiting. Various antibiotics were ineffective. The woman was hospitalized with a diagnosis of facial erysipelas. She owned a cat which had developed a purulent nodule on a forepaw a few days before onset of the patient's disease. LABORATORY TEST: ESR and CRP were moderately elevated, no leukocytosis and blood cultures were sterile. Wound smears showed colonization with Klebsiella pneumoniae and Enterobacter cloacae. DIAGNOSIS, TREATMENT AND COURSE: The patient's general condition improved under initially calculated antibiotic dosages, which was later adapted to the measured resistance. The black-crusted nodes became larger, however, and incision was performed on the 8 th day after hospitalization, under the suspicion of fluctuation. However, no pus was removed, but there was massive inflammatory infiltration of the soft tissue. Examination of samples of skin and part of the crust revealed orthopox virus (cowpox virus). Spontaneous healing followed within 3 weeks, leaving only a small scar. CONCLUSIONS: This was a cowpox virus in the sense of a zoonosis transmitted by the cat. In Germany, now that smallpox has been eradicated, the clinical presentation of infections with the orthopox virus, which are closely related to variola virus, are too little recognized. Atopic and immunocompromised patients are at risk of a cutaneous dissemination with a more severe course of the infectious illness; even a lethal outcome has been reported in Germany.

Adult↗

[Merkel cell carcinoma].

Merkel cell carcinoma (cutaneous neuroendocrine carcinoma) is an uncommon, highly malignant, neuroendocrine skin tumour. Typically, the primary is a fast-growing tough dermal nodule that is characterized histologically by uniform round cells with a small cytoplasmic rim. The tumour cells express the cytokeratins 8, 18, 19, 20, neurofilament, synaptophysin, chromogranin, and neuron-specific enolase. A high frequency of local recurrences (25-77%) and lymph-node metastases (50%) are characteristic features of Merkel cell carcinoma. The 5-year survival rate is 30-74%. Merkel cell carcinomas are highly radiosensitive. Thus, besides surgical methods, radiation should be included into the treatment concept in every stage. We present four cases of Merkel cell carcinoma with different courses for a review-like discussion of this disease giving instructions for rapid diagnosis and effective therapy.

Aged↗

Treatment with cyclosporin A in a patient with Omenn's syndrome.

Unless treated with haematopoetic stem cell transplantation, Omenn's syndrome, a rare variant of severe combined immunodeficiency, is associated with a fatal outcome. We describe a male infant showing all the typical features of Omenn's syndrome, who was successfully treated with cyclosporin A to improve clinical condition prior to haematopoetic stem cell transplantation.

Cyclosporine↗

The hand-foot syndrome--a frequent secondary manifestation in antineoplastic chemotherapy.

The hand-foot syndrome (HFS) (palmoplantar erythrodysesthesia) designates acute, painful erythemas of the palms and soles of the feet caused by antineoplastic chemotherapies. The most frequent trigger substances are 5-fluoruracil and its derivates. At maximum severity, the HFS is bullous to erosive or ulcerous in character. The pathogenesis has not yet been clarified. Histologically, the HFS is characterized by a toxic keratinocyte reaction. Furthermore, there is sub-basal edema with a tendency to bullae, dilated blood and lymph capillaries and usually only mild perivascular lymphocytic infiltration. Early recognition and delineation from other differential diagnoses is prerequisite to targeted management of the disease. Depending on the severity, HFS requires dose reduction, interruption or switch in the antineoplastic chemotherapy.

Antineoplastic Agents↗