Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone.

BACKGROUND: The group of autoinflammatory syndromes associated with Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis are poorly defined and difficult to control with currently available treatment modalities. OBJECTIVES: We describe a patient with PASH syndrome and report about the successful multimodal treatment with infliximab, cyclosporine, and dapsone. METHODS: A review of the available literature to date about this group of autoinflammatory diseases was performed. We performed genetic analysis for PSTPIP1 mutations associated with PAPA syndrome. RESULTS: A 22-year-old woman presented to our department with pyoderma gangrenosum, concomitant acne, and suppurative hidradenitis. She had previously been treated unsuccessfully with etanercept, adalimumab, fumaric acid and the IL-1 receptor antagonist (IL-1RA) anakinra without prolonged remission. Treatment with intravenous infliximab in combination with cyclosporine and dapsone lead to sudden and prolonged improvement of the clinical symptoms that we classified as PASH syndrome. We review the literature about this group of diseases and report the third case of PASH syndrome to date. CONCLUSION: PASH syndrome and associated diseases should be considered whenever hidradenitis suppurativa is found in association with pyoderma gangrenosum. We provide a systematic overview about PASH syndrome and suggest a novel multimodal therapeutic regimen beyond isolated inhibition of TNF or IL-1.

[Surgical reduction in benign symmetrical lipomatosis]. / Reduktionsplastik bei benigner symmetrischer Lipomatose.

We report on a 50-year-old female patient suffering from benign symmetric lipomatosis of her upper arms, shoulders, neck, back, abdomen, and thighs. Concomitantly, we found hypertension, hyperuricemia, and hypertriglyceridemia. Since the patient complained of occasional pain in her upper arms and shoulders induced by work or pressure and suffered psychically from her deformity, we decided to reduce the fatty tissue of the upper arms surgically--with good cosmetic and functional results.

[Quantitative studies of the interaction of polyvinylpyrrolidone iodine and spermatozoa]. / Quantitative Untersuchungen zur Interaktion von PVP-Jod und Spermatozoen.

27 ejaculates were incubated together with polyvidone iodine diluted with physiological saline solution in various proportions. 10 minutes, 1 hour, 4 hours, and 20 hours later, we determined the percentages of mobile and viable spermatozoa in both the native and the incubated seminal fluid. 1% polyvidone iodine solution was totally spermicidal already after 10 minutes of incubation. 0.1% solution only slightly reduced the sperm motility and viability. 0.01% polyvidone iodine solution even resulted in a temporary increase of motility.

[Combined topical treatment of acne with erythromycin and tretinoin]. / Die topische Kombinationsbehandlung der Akne mit Erythromycin und Tretinoin.

Vitamin A acid is the most effective comedolytic agent in the therapy of acne vulgaris. Antibiotics are suitable for the treatment of inflammatory lesions (papulo-pustules). Even topically applied, some antibiotics show a sufficient anti-inflammatory effect. Above all, erythromycin is reliable in the topical treatment of acne. Combined therapy with both topical tretinoin and erythromycin is more effective than either alone. During the first weeks of treatment, tretinoin leads to temporary deterioration of the disease, which can mostly be avoided by the anti-inflammatory effect of erythromycin simultaneously applied.

[Co-occurrence of plane and verrucous leukoplakia evolving into an invasive squamous cell carcinoma]. / Koinzidenz von planer und verruköser Leukoplakie mit Ubergang in ein invasives Plattenepithelkarzinom.

A 40-year old female patient showed extended areas of homogeneous and verrucous leukoplakias on the floor of her mouth. In addition, we found leukoplakia of the speckled type on her right lower alveolar ridge. Histological examination of specimens taken from this region revealed squamous cell carcinoma with invasion into the mandibular bone. Heavy smoking as well as chronic irritation caused by a poorly fitted denture were considered predisposing factors. We carried out radical excision of the carcinoma and the verrucous leukoplakias including partial bone resection. During a follow-up period of two years, we did not observe any recurrences of the carcinoma.

[Cutaneous Kaposi sarcoma in chronic lymphatic leukemia]. / Kutanes Kaposi-Sarkom bei chronischer lymphatischer Leukämie.

A classical Kaposi sarcoma, a tumor appearing sporadically mainly in elderly males, occurred in its typical cutaneous location in a male aged 67 years. Routine laboratory investigations revealed a marked leucocytosis which, with the aid of a bone-marrow biopsy and immunocytological methods, could be attributed to a hitherto undetected chronic lymphatic B-cell leukaemia.

Histopathologic types and clinical behaviour of occult papillary carcinoma of the thyroid.

On studying 182 papillary neoplasms of the thyroid, 32 nonencapsulated lesions were found to be occult papillary carcinomas (maximum diameter 1.5 cm); out of these 32 patients 18 cases of cervical lymph node metastases were observed. Histologically, 4 circumscribed microcarcinomas, 3 partially encapsulated carcinomas, and 25 occult sclerosing carcinomas were distinguished. The mean diameter of the 4 circumscribed tumours as compared to that of the remaining 28 lesions proved to be statistically significantly smaller, but no significant differences could be observed regarding the rate of lymph node metastasis. One occult sclerosing carcinoma showed a morphologic pattern different from the remaining cases--namely that it had an exclusively solid appearance in the primary lesion and also in its lymph nodes metastases. The follicle cell origin of this lesion was established immunohistochemically by patchy thyroglobulin synthesis. The excellent prognosis of the occult category of papillary thyroid carcinoma was confirmed by a follow-up (mean 7.7 years, range 1.1 to 16.3 years), in which only 2 instances of eradicable recurrent lymph node metastases within the group of occult sclerosing carcinomas were recorded.

The encapsulated follicular carcinoma of the thyroid. A clinicopathologic study of 35 cases.

In a retrospective study of 86 follicular carcinomas of the thyroid gland, 35 lesions were classified as encapsulated carcinomas (40.7%). In two of these, lymph node metastases were detected initially. Another patient presented with distant metastases. The biological behaviour of these 35 tumours was studied over a long-term follow-up period (0.4-19.1 years, mean 10.3 years) which featured three cases of death from thyroid carcinoma 0.4-5.0 years after thyroidectomy. Another patient suffered from local recurrence of a follicular carcinoma 13.9 years later. The morphological and clinical findings of those five patients who initially presented with metastases and/or whose follow-up registered the local recurrence of thyroid cancer or death as a result of it, were compared with the remaining 30 cases which were of a benign clinical course. Statistical analysis showed that the prognosis of encapsulated follicular carcinoma is more serious when tumours occur in patients older than 65 years of age and when the tumour diameter is 5.0 cm or more. There was a tendency towards poorer prognosis in those tumours exclusively composed of oxyphilic epithelium.