Neurofibromatosis (NF) is one of the most common genetic disorders. Inherited in an autosomal dominant fashion, this phacomatosis is classified into two genetically distinct subtypes characterized by multiple cutaneous lesions and tumors of the peripheral and central nervous system. Neurofibromatosis type 1 (NF1), also referred to as Recklinghausen's disease, affects about 1 in 3500 individuals and presents with a variety of characteristic abnormalities of the skin and the peripheral nervous system. Neurofibromatosis type 2 (NF2), previously termed central neurofibromatosis, is much more rare occurring in less than 1 in 25 000 individuals. Often first clinical signs of NF2 become apparent in the late teens with a sudden loss of hearing due to the development of bi-or unilateral vestibular schwannomas. In addition NF2 patients may suffer from further nervous tissue tumors such as meningiomas or gliomas. This review summarizes the characteristic features of the two forms of NF and outlines commonalities and distinctions between NF1 and NF2.

Pigmentary abberations of the skin, mucosa and epidermal appendages are common side-effects after systemic treatment with chemotherapeutic agents. These pigment changes appear in different patterns and are partly quite typical for the applied chemotherapeutic drug. The pathogenesis of the different skin pigmentations are not well known. The most often discussed causes are the stimulation of melanocytes, involvement of the tyrosinase enzyme system and thrombophlebitis with postinflammatory hyperpigmentation by the aggressive substances. Nail discolorations are mainly due to direct toxic effects and stimulation of the matrix melanocytes. We report a rare event of supravenous hyperpigmentation, transverse leuconychia and melanonychia after chemotherapy of a patient suffering from Hodgkin's disease.

ver mit tel ten Tran skrip ti on pro in flam ma to rischer Zy to ki ne. Im Ge gen satz zu to pi schen Glu ko kor ti kos te roi den be ein flusst die to pische Be hand lung mit Ta cro li mus nicht die ku ta ne Kol la gen syn the se, so dass kein Ri siko ei ner ku ta nen Atro phie be steht. Als Neben wir kun gen wer den, vor al lem bei ini ti aler An wen dung, Bren nen, Juck reiz oder ein Ery them be ob ach tet. Zahl rei che Stu di en bele gen die gute Wirk sam keit von Ta cro li mus bei der Be hand lung des ato pi schen Ek zems oder an de rer ent zünd li cher Der ma to sen, wie z. B. beim Li chen ru ber mu co sae und Li chen scle ro sus et atro phi cus, bei der steroidin du zier ten Ro sa zea oder bei der se borrhoi schen Der ma ti tis [2].Der vor lie gen de Fall zeigt, dass to pisch ap pli zier tes Ta cro li mus auch eine gute The ra pie op ti on für die Be hand lung der schwe ren, the ra pie re sis ten ten, pe ri ora len Der ma ti tis dar stel len kann. Die In di ka tion soll te al ler dings vor sich tig ge stellt werden, da die lo ka le Im mun sup pres si on eine ver stärk te Pro li fe ra ti on von De mo dexmil ben und so mit auch eine Ver schlech terung des Haut be fun des ver ur sa chen könn-te [1,3]. Aus die sem Grund soll ten vor und wäh rend der The ra pie re gel mä ßi ge Ver laufs kon trol len mit tels Cya no a crylatabriss er fol gen. Kor re spon die ren der Au tor PD Dr. D. Bruch-Ger harzHaut kli nik der Hein rich-Hei ne-Uni ver si tät, Moo ren stra ße 5, 40225 Düs sel dorf E-Mail: bruch-ger harz@med.uni-du es sel dorf.deIn ter es sen kon flikt: Der kor re spon die ren de Au tor ver si chert, dass kei ne Ver bin dun gen mit ei ner Fir ma, de ren Pro dukt in dem Ar ti kel genannt ist, oder ei ner Fir ma, die ein Kon kur renzpro dukt ver treibt, be ste hen. Li te ra tur1. An til le C, Sau rat JH, Lub be J (2004) In duc ti on of rosa cei form der ma ti tis du ring treat ment of fa ci al inflam ma to ry der ma to ses with ta cro li mus oint ment. Arch Der ma tol 140:457-460 2. Ass man T, Ho mey B, Ru zicka T (2001) To pi cal ta croli mus for the treat ment of in flam ma to ry skin di seases. Ex pert Opin Phar ma co ther 2:1167-1175 3. Lub be J, Stucky L, Au rat JH (2003) Ro sa cei form derma ti tis with fol li cu lar de mo dex af ter treat ment of fa ci al ato pic der ma ti tis with 1% pi me cro li mus cream. Der ma to lo gy 207:205-207 4. Plewig G (2005) Pe ri ora le Der ma ti tis. In: Braun-Falco O, Plewig G, Wolff HH et al. (Hrsg) Der ma to lo gie und Ve ne ro lo gie, Sprin ger, Ber lin Hei del berg New York To kyo, S 907-908 5. We ber K (2003) How I tre at pe ri oral der ma ti tis: non-com plian ce with the treat ment gui de li nes. Der ma to lo gy 207:215 6. We ber K, Thur mayr R (2005) Cri ti cal ap prai sal of reports on the treat ment of pe ri oral der ma ti tis. Derma to lo gy 210:300-307 Haut arzt 2005 · 56:968-969 · DOI 10.1007/s00105-005-1026-2 · On li ne pub li ziert: 3. Sep tem ber 2005 © Sprin ger Me di zin Ver lag 2005M. Sonn tag · N. Hod zic-Avda gic · D. Bruch-Ger harz · N. J. Neu mann Hau...

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Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on the scalp and spreads caudally within a few weeks. It often results in a generalized erythroderma with sharply demarcated islands of sparing ("nappes claires"). A 65-year-old patient with severe PRP showed good clinial improvement after 8 months of treatment when treated with acitretin in combination with phototherapy and systemic gluocorticosteroids.