[Basal cell carcinomas in radiotherapy-treated port wine stains. An ideal indication for Mohs surgery]. / Basalzellkarzinome auf mit Radiotherapie vorbehandelten vaskulären Malformationen. Eine ideale Indikation für mikroskopisch kontrollierte Chirurgie.

BACKGROUND: Early treatment of port wine stains with ionizing radiation can lead to the development of often multifocal basal cell carcinomas (BCC) after decades. In most cases it is clinically impossible to distinguish between the tumor and the underlying vascular malformation and to decide where to set surgical margins. PATIENTS AND METHODS: We report on a series of three patients with BCC overlying a port wine stain that had previously been treated with radiation therapy in early childhood. In all patients Mohs surgery was performed to insure complete excision of the BCC. RESULTS: In our patients, development of BCC occurred about 20 to 40 years after radiation therapy. Clinically - without the help of Mohs surgery - the borders of the BCC could not have been detected due to the underlying nevi flammei in all cases. CONCLUSIONS: BCC overlying a port wine stain previously treated with radiation therapy is a rare but ideal indication for Mohs surgery. In addition we recommend regular clinical follow-ups to detect recurrent or additional BCC as early as possible.

Mobile encapsulated bodies comprising fat necrosis and fibrous tissue in the abdominal cavity of cows.

The microscopical features of 18 samples of fat necrosis and/or fibrous tissue removed from the abdominal cavity during laparotomy from 15 cows were studied. The nodular, ivory-coloured mobile structures were free-floating in the abdominal cavity, were not attached to any abdominal tissues or organs, and were completely surrounded by a fibrous capsule. Abdominal fat necrosis (bovine lipomatosis) was not observed in any animal. The structures comprised either necrotic fat, fibrous tissue or varying proportions of both. Focal calcification and mild inflammatory cell infiltration and accumulations of haemosiderin were also present. Microscopically, the lesions resembled encapsulated fat necrosis occurring in human subcutaneous tissue. The mechanisms of development of these mobile encapsulated bodies in cows is unknown and it is not clear how, in the absence of a blood supply, there can be inflammatory cell infiltration, calcification and proliferation of fibroblasts.

[Hereditary photodermatoses]. / Hereditäre Photodermatosen.

Hereditary photodermatoses are characterized by an increased photosensitivity caused by an inherited single gene defect. With few exceptions, they manifest in early childhood, reveal heterogeneous clinical symptoms, and are difficult to treat. Although these diseases are rare, it is very important to make an accurate diagnosis on the basis of clinical symptoms, specific diagnostic tests, and direct DNA analysis. We review the spectrum of inherited photodermatoses, including porphyria cutanea tarda, erythropoietic protoporphyria, actinic prurigo, Kindler syndrome, and disorders associated with a defect in DNA repair, including xeroderma pigmentosum, trichothiodystrophy, Cockayne syndrome, and Bloom syndrome. Early diagnosis may prevent complications associated with prolonged unprotected exposure to sunlight and makes it possible to offer genetic counseling and, when indicated, prenatal diagnosis to families at risk for these rare heritable disorders.

Induction of a chemoattractive proinflammatory cytokine response after stimulation of keratinocytes with Propionibacterium acnes and coproporphyrin III.

BACKGROUND: The inflammation in acne vulgaris is widely thought to be induced by an immunological reaction, but the role of Propionibacterium acnes is unclear. OBJECTIVES: To examine the local host response mechanism of a keratinocyte cell line 3 h and 6 h after stimulation with viable and heat-killed P. acnes. METHODS: The quantitative expression of cytokines was measured at the mRNA level by real-time reverse transcription-polymerase chain reaction. RESULTS: The coincubation of a keratinocyte cell line with viable, but not heat-killed, P. acnes modulated an adequate cytokine response for interleukin (IL)-1beta, granulocyte/macrophage colony-stimulating factor and IL-8. High-performance liquid chromatographic analysis of the in vivo porphyrin pattern secreted by P. acnes revealed a predominance of coproporphyrin III in acne lesions. This same porphyrin fraction also modestly induced IL-8 expression by keratinocytes. CONCLUSIONS: This cytokine pattern may favour a chemotactic response and implicates P. acnes and coproporphyrin III in the recruitment of inflammatory cells to the site of infection and in the development of acne lesions.

Concordance between telepathologic diagnosis and conventional histopathologic diagnosis: a multiobserver store-and-forward study on 20 skin specimens.

OBJECTIVE: To study the validity and feasibility of transferring images of cutaneous biopsy specimens via e-mail to remote physicians active in dermatopathology for teleconsultation. DESIGN: Twenty skin specimens previously diagnosed at the Department of Dermatology, University of Graz, Austria, were subsequently sent for teleconsultation using the store-and-forward method. For each case, 3 or 4 images at different magnifications were sent by e-mail to 16 colleagues (11 dermatopathologists and 5 pathologists) in 15 centers in 6 different countries. Six weeks later each observer received the hematoxylin-eosin-stained specimens to render a conventional diagnosis. SETTING: Dermatopathology and pathology units within institutional and private settings. MATERIAL: Twenty small skin biopsy specimens of cutaneous diseases were selected randomly from a study set of 80. MAIN OUTCOME MEASURE: Concordance between telepathologic diagnoses and conventional histopathologic diagnoses of 20 skin specimens. RESULTS: On average, 78% of the telediagnoses were correct (range, 60%-95%), whereas 85% of the conventional diagnoses were correct (range, 60%-95%). A perfect diagnostic concordance was obtained in 7 (35%) of 20 cases, and a significant difference was identified in only 1 case. CONCLUSIONS: Results suggest that telepathology performed by physicians active in dermatopathology may serve as a reliable technique for the diagnosis of cutaneous diseases when experts in dermatopathology are not available locally. Furthermore, teledermatopathology is attractive because it provides an opportunity to obtain timely consultation on difficult cases.

Macrolactam immunomodulators for topical treatment of inflammatory skin diseases.

The immunomodulatory macrolactams provide an alternative to glucocorticosteroids for the topical treatment of atopic dermatitis and other inflammatory dermatoses. Tacrolimus (FK506), as well as the newer ascomycin derivative ASM 981 (pimecrolimus), penetrate the inflamed epidermis and are suitable for topical therapy. Both substances inhibit the transcription of proinflammatory cytokine genes such as interleukin 2, which are dependent on the nuclear factor NF-AT. They block the catalytic function of calcineurin, which leads to the inhibition of the transport of the cytoplasmic component of NF-AT to the cell nucleus. Multicenter, randomized, double-blind clinical trials with topical formulations have shown the efficacy of both substances in moderate to severe atopic dermatitis. A review is presented of the biochemical and cell biologic properties, mode of action, pharmacokinetic data, side effects, results of the clinical trials, and further indications for tacrolimus and ASM 981, along with an overview of the related substances cyclosporine and sirolimus (rapamycin).

Cowden syndrome-diagnostic skin signs.

Cowden syndrome is a rare autosomal dominant familial cancer syndrome with a high risk of breast cancer. The most important clinical features include carcinomas of the breast and thyroid, and hamartomatous polyps of the gastrointestinal tract. There are characteristic mucocutaneous features which allow early recognition of the disease and are generally present before internal malignancies develop. We report on a woman in whom the diagnosis of Cowden syndrome was first made after she had been treated for both breast cancer and melanoma.

[Actinic prurigo. An assessment of current status]. / Aktinische Prurigo. Eine Standortbestimmung.

BACKGROUND AND OBJECTIVE: Actinic prurigo (AP) is a hereditary photodermatoses with typical symptoms and is quite common in North- and South-America. The key genetic factor appears to be a Native American background. In Europeans this type of AP is extremely rare; some dispute if this disease exists in Caucasians. Some newer publications postulate that these patients share HLA markers with the Native Americans. The most important differential diagnosis for AP is polymorphic light eruption which can be excluded relatively accurately by the clinical picture, typical histology and HLA pattern. PATIENTS/METHODS: The case of a female patient of Mayan ancestry living in Germany is presented. CONCLUSIONS: Since in literature sometimes cases from Europe are diagnosed as AP this is a problem of naming the disease. It would be helpful to integrate the terms hereditary or hereditaria into the name of the disease in indians.

Muir-Torre syndrome - treatment with isotretinoin and interferon alpha-2a can prevent tumour development.

Muir-Torre syndrome is a genodermatosis in which multiple internal malignancies are associated with cutaneous sebaceous tumours and kerato-acanthomas. A 57-year-old man presented with multiple sebaceous tumours, kerato-acanthomas, verrucous carcinoma of the nose, renal cell and transitional cell carcinomas of the left kidney, adenoma of the colon and a positive family history of colon carcinoma. He was treated with interferon (IFN-alpha2a) s.c. 3 x 10(6) U three times a week along with 50 mg isotretinoin daily as well as topical isotretinoin gel. During a follow-up of 29 months, only 1 sebaceous skin tumour developed and was removed, whereas more than 30 such skin tumours had been surgically removed during the last 3 years. No evidence of internal tumour development or recurrence was found. The combination of IFN with retinoids seems to be of promise to prevent tumour development in Muir-Torre syndrome.

Myxoid liposarcoma in a 12-year-old girl.

A 12-year-old girl developed a myxoid liposarcoma on the inner side of her right thigh. Liposarcomas are extremely rare at this age compared to benign lipoblastomas, which are the most frequent tumors of fatty tissue in childhood. The prognosis of myxoid liposarcoma is good, especially when, as in this patient, the tumor is located in the subcutis where a large and deep excision is possible and often curative.