[Multiple lentigines (LEOPARD) syndrome. Case reports and review of the literature]. / Multiple-Lentigines (LEOPARD)-Syndrom. Kasuistiken und Literaturübersicht.

The rarely occurring multiple lentigines (LEOPARD) syndrome represents a complex of skin, heart, skeleton and other malformations and is described in a 36-year-old male and his 9-year-old daughter. With the occurrence of multiple lentigines, the diagnostic search for further malformations should always be undertaken. Its differential diagnosis and its pathogenetic and clinical aspects are discussed in this paper.

Juvenile papular-purpuric gloves and socks syndrome.

Papular-purpuric gloves and socks syndrome (PPGSS) occurs mainly in young adults, but it should also be considered in the differential diagnosis of childhood exanthems. The case presented here is the youngest patient hitherto reported with PPGSS and supports the concept of a viral genesis.

[Rare and newly described histological variants of cutaneous squamous epithelial carcinoma. Classification by histopathology, cytomorphology and malignant potential]. / Seltene und neu beschriebene histologische Varianten des kutanen Plattenepithelkarzinoms. Klassifikation nach Histopathologie, Zytomorphologie und Malignität.

The histological spectrum of squamous cell carcinoma (SCC) of the skin is presented, including recently mentioned and rarely appearing tumor subtypes. Beside the most frequently occurring common SCC with variable degree of differentiation, the following tumor forms can be identified microscopically: Bowen-SCC, acantholytic SCC, clear cell SCC, spindle cell SCC, keratoacanthoma-like SCC, desmoplastic SCC, neurotrophic SCC, verrucous SCC, adenosquamous carcinoma and basosquamous cell carcinoma. The classification of lymphoepithelioma-like carcinoma as a cutaneous SCC variant is disputed, because epidermal connections are regularly absent and evidence of glandular and follicular differentiation suggest an adnexal origin. Beside the description of distinctive histological and cytomorphological features of each SCC subtype, important differences in malignant behavior and clinical course are emphasized.

Histologic subtyping and malignancy assessment of cutaneous squamous cell carcinoma.

BACKGROUND: Squamous cell carcinomas (SCCs) of the skin have a wide range of histologic subtypes and there are indications of differences in prognosis. OBJECTIVE: The morphologic variety of SCCs with respect to its biological behavior and the further course of disease is analyzed, with emphasis on histopathologic criteria, briefly quoting the main clinical and pathogenetic aspects. METHODS: Referring to the international tumor classification of the World Health Organization, histologically different carcinoma variants are presented and discussed, based on a review of the literature regarding each subtype, and also including the desmoplastic SCC type. RESULTS: Histologically, common invasive SCCs are most frequently found, while metastases mainly occur in tumors of high thickness and poor differentiation. The immature spindle cell carcinoma type resembles sarcoma and may grow rapidly with an aggressive clinical course. Lymphoepithelioma-like carcinoma of the skin is extremely rare and its histogenesis remains to be elucidated. Thus far, one case with metastasis and lethal outcome has been reported. As details determining the progression ability have so far only been scanty and partially contradictory, more investigations are necessary, especially for acantholytic SCCs and invasive SCCs developing from Bowen's disease, whereas verrucous carcinomas can be categorized as low malignancy neoplasms. Desmoplastic SCCs, especially with large tumor thickness, should be separated from other SCC subtypes due to their high risk of local recurrence and metastatic spread. CONCLUSION: The future outcome of SCCs of the skin is significantly influenced by their histologic grade and tumor thickness. In addition, subtyping represents another valuable histopathologic tool for improving the assessment of malignancy.

[Histological and clinical prognostic factors in squamous cell carcinoma of the skin. A contribution to the multicenter carcinoma study of the association of surgical and oncological dermatology]. / Pathohistologische und klinische Prognosefaktoren beim Plattenepithelkarzinom der Haut. Ein Beitrag zur multizentrischen Karzinomstudie der Vereinigung für Operative und Onkologische Dermatologie.

The guidelines of the International Union against Cancer are generally used to assess malignancy of squamous cell carcinoma of the skin. Beside TNM-staging, histological grading is performed. This system has been extended by a selection of histological parameters which were studied with respect to prognosis, based on a review of 184 patients with squamous cell carcinoma. An increased malignancy was found in carcinomas with the following features: clinical diameter greater than 2 cm, low degree of keratinization, high degree of cellular polymorphism, high mitotic index and high tumor thickness index (metastases only from tumor thickness 2.4 mm and subcutaneous infiltration), desmoplasia and ulceration. Consideration of histological tumor parameters improved prognosis assessment. Multivariate analyses with large case series are necessary to determine ranks of the prognostic factors. Therefore, the data analysis of all centers that contribute to the carcinoma registry will make a precise definition of high- and low-risk carcinomas possible and can thus influence therapy and follow-up procedures.

[Stevens-Johnson syndrome with transition to toxic epidermal necrolysis after carbamazepine administration, heroin and alcohol abuse]. / Stevens-Johnson-Syndrom mit Ubergang in eine toxisch epidermale Nekrolyse nach Carbamazepin-Einnahme, Heroin- und Alkoholabusus.

A 28 year old patient developed a severe bullous exanthem and enanthem combined with hepatitis, fever and blood count abnormalities after taking carbamazepine and consumption of heroin and alcohol. After discontinuing carbamazepine, prednisolone was given over a five day period accompanied by intravenous fluid and electrolyte substitution and local therapy which lead to improvement. Severe bullous skin reactions nowadays are classified into erythema exsudativum multiforme majus (EEMM), Stevens-Johnson syndrome (SJS), overlap Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS/TEN), TEN with maculae and TEN on large erythema, and they are most often caused by antibiotics and anticonvulsant drugs. Heroin and alcohol abuse alters host immunity which subsequently may increase susceptibility to allergic reactions. There is a high (40%) mortality rate for TEN, and patients with organ involvement are at increased risk.

Squamous cell carcinoma of the skin--histopathological features and their significance for the clinical outcome.

BACKGROUND: Owing to the rising incidence of tumours, the question of reliable risk classification is becoming increasingly significant. OBJECTIVE: Participation in the multicentre carcinoma registry maintained by the Association of Operative and Oncological Dermatology of the German Dermatological Society requires, in addition to the parameters of clinical staging and grading already established by the International Union against Cancer, description of other histopathological criteria related to prognosis, with special attention to microstaging. METHODS: One hundred and eighty-four patients with squamous cell carcinoma of the skin were examined. The histological parameters, carcinoma type, Breslow index, invasion level, growth form, grading and mitotic index were recorded and classified, and clinical staging was performed. RESULTS: It was found that the clinical criterion of tumour diameter (T-category) determines the further course of the disease. The other parameters taken into account, namely pathohistological microstaging and grading, can increase the accuracy of prognosis evaluation, and in particular enable carcinomas of the T1-category to be classified as either high malignant or low malignant tumours. The desmoplastic squamous cell carcinoma subtype is a potential risk tumour. Endophytic tumours are more malignant in their development than exophytically-growing carcinomas, and the probability of recurrence and metastatic tumour spread further increases when ulceration can be detected by microscope. In order to distinguish metastatic and recurring carcinomas, in addition to determining the invasion levels after Clark, measuring the Breslow index proved to be an important criterion. Quoting the mitotic index augmented the grading system, which further improved the reliability of malignancy assessment. CONCLUSIONS: The TNM categories currently applied to squamous cell carcinomas of the skin must be supplemented by additional histological parameters which, according to our findings, permit more accurate classification of high and low malignant tumours.