[Muir-Torre syndrome. Diagnostic criteria and review of the literature]. / Muir-Torre-Syndrom. Diagnosekriterien und Literaturübersicht.

We report on a 63-year-old female patient with Muir-Torre syndrome (MTS). In the course of this disease two carcinomas of the colon, a kerato-acanthoma and multiple sebaceous gland tumours, including four sebaceous carcinomas, appeared. This case is thought to be a hereditary form as one of daughters was also found to have a sebaceous epithelioma. MTS is a mostly autosomal-dominant disease with the association of sebaceous gland tumours and internal carcinomas. As the malignant tumours only show slight aggressiveness the prognosis is quite favourable. Oral isotretinoin therapy was successfully used for the inhibition of sebaceous gland proliferation. A narrower definition is presented and an updated survey of the published cases is given. Furthermore, the histopathologic peculiarities of sebaceous gland tumours, especially of sebaceous gland carcinomas, are discussed and compared to sebaceous gland tumours not connected with MTS. A total number of 100 of the 135 published cases of MTS were included and analysed regarding sebaceous gland tumours and other skin tumours. The data on internal carcinomas were taken from the work of Cohen et al. (1991) and 11 current cases were added.

[Bazex-Dupré-Christol syndrome. Follicular atrophoderma, multiple basal cell carcinomas and hypotrichosis]. / Das Bazex-Dupré-Christol-Syndrom. Follikuläre Atrophodermie, multiple Basaliome und Hypotrichose.

The Bazex-Dupré-Christol syndrome (BDC syndrome) was first described in 1964. Only 14 affected families and 47 patients with this very rare dominant genodermatosis have been reported so far. The three main features of BDC syndrome are (1) follicular atrophoderma, especially on the dorsum of hands and feet, (2) multiple basal cell carcinomas of the face, occurring mainly during the second decade of life, reflecting the clinical relevance of BDC syndrome and (3) congenital generalized hypotrichosis, sometimes with pili torti and trichorrhexis nodosa. The expressivity of the main features varies. Common associated symptoms are milia, calcifying epithelial tumours and hypohidrosis. We present three members of one family with BDC syndrome, a mother and her two sons. In the mother and one son the clinical picture is very typical with all major features, whereas in the other son only follicular atrophoderma and hypotrichosis are present.

[Cutaneous condylomata acuminata of an unusual site and extent. Successful CO2 laser therapy]. / Kutane Condylomata acuminata in ungewöhnlicher Lokalisation und Ausbreitung. Erfolgreiche CO2-Laser-Therapie.

We report on a 33-year-old patient with unusually extensive and predominantly cutaneous condylomata acuminata spreading from the penis over the mons pubis to the entire lower abdominal wall but almost completely sparing the genital mucous membranes. Single lesions were found on the left arm and on the buttocks. Southern blot hybridization detected HPV-6a DNA sequences within the fibroepithelial tumours. Carbon dioxide laser treatment resulted is cosmetically acceptable healing without recurrence.

[Multiple woolly hair nevi with linear epidermal nevus and persistent pupillary membrane]. / Multiple Wollhaarnävi mit linearem epidermalen Nävus und persistierender Pupillarmembran.

The woolly hair nevus is a very rare disease that only occurs sporadically. So far, approximately 48 patients with woolly hair nevus have been described in the world literature. Both sexes are equally affected. Mostly, the dermatosis manifests itself from birth or within the first 2 years of life. Up to 5 circumscribed areas with clearly lighter curly hairs can be observed. About 50% of the patients present a linear epidermal nevus at the same time. Microscopically, the hair shaft diameter of the woolly hair is significantly reduced. Next to oval structures the cross-sections of the hair often show triangular and irregular forms. Furthermore, pili canaliculi and pili torti as well as circumscribed cuticula defects can be observed. We present a 5-year-old boy, who manifested 4 woolly hair nevi on the capillitium in the 9th month of life. From the 2nd year of life onwards he developed a linear epidermal nevus. The ophthalmological examination showed a persistent pupillary membrane. Based on this case and a bibliographical overview, the light and electron microscopical peculiarities are presented.

[Primary cutaneous leiomyosarcoma]. / Primäres kutanes Leiomyosarkom.

Primary leiomyosarcomas of the superficial soft tissues are very rare neoplasms. Because of the different biological behaviour, they should be divided into cutaneous and subcutaneous tumours. Differential diagnosis includes leiomyomas, skin metastases from extracutaneous leiomyosarcomas, and other spindle-cell tumours of the skin. Ultrastructural and immunohistochemical investigations are useful when light microscopic features do not allow an unequivocal diagnosis. Although local recurrences develop in almost half the patients with cutaneous leiomyosarcomas, the prognosis is predominantly good. In contrast, metastatic spread is more common in patients with subcutaneous leiomyosarcomas. Treatment for superficial leiomyosarcomas consists in wide excision. We report on a 76-year-old women with a local recurrence of a primary cutaneous leiomyosarcoma.

[Squamous cell cancers in Hallopeau-Siemens recessive dystrophic epidermolysis bullosa]. / Spinozelluläre Karzinome bei Epidermolysis bullosa dystrophica recessiva Hallopeau-Siemens.

Recessive dystrophic epidermolysis bullosa is one of the most severe hereditary mechano-bullous diseases, characterized by scarring blister formation, nail dystrophy and onycholysis, cutaneous contractures, synechiae, mutilations of the hands and feet and oesophageal stenosis. With increasing age the patients may develop multiple, fast-growing and early-metastasizing squamous cell carcinomas. When epidermolysis is present, precise determination of which of the various forms is concerned is necessary soon after birth, to make it possible to advise parents about the prognosis of the disease and the likelihood of its occurrence in further children. In pregnancies at risk of severe epidermolysis bullosa a prenatal diagnosis should be performed. We present two siblings with recessive dystrophic epidermolysis bullosa, each of whom developed two squamous cell carcinomas.

[Paroxysmal finger hematoma (Achenbach syndrome)]. / Paroxysmales Fingerhämatom (Achenbach-Syndrom).

Paroxysmal haematoma of the fingers (Achenbach's syndrome) is a rarely reported entity. It often occurs spontaneously or subsequent to minor injuries. Because of the sudden onset of intense burning pain and the subsequent development of haematoma, the patients are frequently alarmed. The etiology is still unknown. We report on 3 cases of paroxysmal haematoma of the fingers. The harmless nature of the condition is emphasized.

Allergic contact dermatitis to naftifine. Report of two cases.

We report on two cases of allergic contact dermatitis due to naftifine, an antimycotic substance. Epicutaneous tests were positive with the reagent itself but not with other compounds of the ointment. Additionally, one of the patients reacted to an ointment containing clotrimazole. Until now, only few cases of contact allergy to naftifine have been described, but an increasing number is expected.

[Condylomata acuminata and HIV infection--an epidemiologic study]. / Condylomata acuminata und HIV-Infektion--eine epidemiologische Studie.

Within a period of 15 months, 134 patients suffering from condylomata acuminata were investigated with regard to simultaneous infection with HIV. 3 of these patients--2 showing warts in the anal region, 1 on the glans penis--were found positive for anti-HIV antibodies.

[Folliculitis nuchae scleroticans--successful treatment with 13-cis-retinoic acid (isotretinoin)]. / Folliculitis nuchae scleroticans--erfolgreiche Behandlung mit 13-cis-Retinsäure (Isotretinoin).

Acne keloidalis nuchae is characterized by keloidal papules and plaques. The lesions are located on the occipital scalp and posterior neck. This rare therapy-resistant condition is nearly always seen in men, particularly in negroes. Advanced stages of the disease require surgical excision with split-thickness skin grafts or treatment with a CO2 laser. Oral therapy with 13-cis-retinoic acid (isotretinoin) in a 23-year-old white man resulted in remarkable improvement within a few weeks.

[Localized Sweet syndrome in urethro-prostatitis caused by Ureaplasma]. / Lokalisiertes Sweet-Syndrom bei Urethro-Prostatitis durch Ureaplasmen.

A 36-year-old patient with localized Sweet's syndrome and concomitant Ureaplasma urealyticum infection is presented. In addition to infectious diseases, malignant neoplasms are also associated with this dermatosis.

[Neck appendages--branchiogenic surplus malformations (choristoma)]. / Halsanhänge--branchiogene Uberschussmissbildungen (Choristome).

Congenital cartilaginous rests of the neck are branchiogenic surplus malformations that are very rare. They arise from epithelial growth in a false place and frequently contain elastic cartilage. Congenital cartilaginous rests of the neck are only rarely associated with other congenital malformations.

[Basal cell nevus syndrome with squamous cell carcinoma of the larynx]. / Basalzellnävus-Syndrom mit Plattenepithelkarzinom des Larynx.

We report on a 54-year-old patient suffering from basal cell nevus syndrome. At the age of 51, squamous cell carcinoma of the larynx had been diagnosed. The frequent occurrence of various kinds of benign and malignant neoplasms in patients with basal cell nevus syndrome illustrates the close relationship between this syndrome and phacomatoses.

[Acanthosis nigricans maligna and Leser-Trélat sign in double malignancy of the breast and stomach]. / Acanthosis nigricans maligna und Leser-Trélat-Zeichen bei Doppelmalignom von Mamma und Magen.

A female patient with primary cancer of the breast and the stomach developed acanthosis nigricans maligna and Leser-Trélat's sign. Acanthosis nigricans maligna is a well-known cutaneous marker of internal malignancy. It may be intensified by radiotherapy. A variant is called florid cutaneous papillomatosis. Leser-Trélat's sign is a rare cutaneous marker of internal neoplasms. According to the literature, we present 46 patients showing Leser-Trélat's sign. Neither Leser nor Trélat described this sign.

[Multiple basaliomas in Curschmann-Steinert myotonia atrophica]. / Multiple Basaliome bei Myotonia atrophica Curschmann-Steinert.

The occurrence of multiple basal-cell epitheliomas in connection with myotonic dystrophy has not previously been reported. The possibility of myotonic dystrophy being a predisposing factor for different kinds of neoplasms is discussed.