[Trigeminal trophic syndrome : Late complication after herpes zoster infection with loss of ala nasi]. / Neurotrophes Trigeminussyndrom : Spätkomplikation nach Herpes-zoster-Infektion mit Verlust des Nasenflügels.

This article presents the case of refractory facial ulcers with loss of ala nasi as a rare differential diagnosis of chronic facial wounds. We diagnosed trigeminal trophic syndrome as a late complication of a herpes zoster infection. Education to avoid self-manipulation and an occlusive local therapy in combination with neuromodulating drug therapy are at the forefront of therapeutic measures. Further surgical plastic procedures can be used with restraint in therapy-resistant cases.

[Anti-p200/anti-laminin γ1 pemphigoid and BP180 NC16A/4575- positive mucous membrane pemphigoid : late diagnosis in a patient with disease-related loss of vision and multiple previous surgical interventions]. / Anti-p200/Laminin-γ1-Pemphigoid in Kombination mit einem BP180 NC16A/4575-positiven Schleimhautpemphigoid : Späte Diagnosestellung bei krankheitsbedingter Amaurosis und mehrfachen Voroperationen.

A 87-year-old woman presented with a three-year history of partially erosive, partially bullous skin and mucosal lesions, symblepharon of both eyelids as well as dysphagia. To date, multiple excisions of the skin lesions, which had been described as "skin tumors" by surgeons, had been performed. The synopsis of histology, direct and indirect immunofluorescence established the diagnosis of anti-p200/anti-laminin γ1 pemphigoid and BP180 NC16A/4575- positive mucous membrane pemphigoid with an unusual "epitope-spreading" phenomenon. Due to the late initiation of therapy, the disease-related loss of vision unfortunately was irreversible.

[Successful therapy of metastatic basal cell carcinoma with vismodegib]. / Erfolgreiche Therapie eines metastasierten Basalzellkarzinoms mit Vismodegib.

A 71-year-old man presented with giant basal cell carcinoma on the abdomen which had metastasized. He was treated with oral vismodegib. Both the primary ulcerated tumor on the abdomen and the metastases responded. Vismodegib was well tolerated without significant side effects. The tumor recurred promptly after vismodegib was discontinued, and then was resistant to therapy when vismodegib was re-administered.

[Giant squamous cell carcinoma of the scalp: successful excision with use of bovine dermal substitute]. / Gigantisches Plattenepithelkarzinom des Kopfes: Erfolgreiche operative Therapie und Einsatz eines bovinen Dermisersatzes.

A 68-year-old woman presented with an enormous exophytic tumor on her forehead extending to the scalp. After complete resection of the tumor, the resulting defect was closed successfully using a bovine dermal substitute followed by a split-thickness skin graft.

Lacking CD56 expression in a relapsing cutaneous blastic plasmacytoid dendritic cell neoplasm after allogeneic bone marrow transplantation: FISH analysis revealed loss of 11q.

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare entity characterized by a CD4+/CD56+/CD123+ immunophenotype and a fatal clinical course. The average survival of 12-14 months may be prolonged by allogeneic bone marrow transplantation (BMT). OBJECTIVES: We report about a male patient who suffered from BPDCN with a typical histology and co-expression of CD4/CD123 and a CD56 expression by 80% of the tumour cells. The cutaneous tumour relapse after chemotherapy and allogeneic BMT was completely negative for CD56. METHODS: We performed interphase fluorescence in situ hybridization (FISH) analysis of tumour tissue, asserved before and after BMT, using specific probes for chromosome 11, which encompass the CD56 gene region. RESULTS: The tumour cells revealed a partial loss of 11q as well as a monosomy of chromosome 11. CONCLUSION: This case demonstrates for the first time that loss of CD56 expression can also occur as a secondary event after chemotherapy and BMT. In our case, DNA loss of 11q23 could be responsible for the negativity of 20% of tumour cells as observed before chemotherapy. However, the complete loss of CD56 expression in the relapsed tumour cannot be explained by the loss of 11q23 alone. Additional factors such as chemotherapy-induced mutations might also have contributed.

Papular exanthem discloses acute myeloid leukaemia: interphase fluorescence in situ hybridization revealed deletion of p53 and gain at 8q22/8q24/Tel8q without trisomy 8.

We describe a 79-year-old patient who presented with fatigue, weight loss, pancytopenia and a papular exanthem. Previous attempts to taking bone-marrow biopsies had resulted in a 'dry tap', with no material collected, suggesting idiopathic myelofibrosis. Histological examination of skin biopsies showed dermal infiltration of monocytoid cells, resulting in a diagnosis of acute myeloid leukaemia (French-American-British M5 morphology) with leukaemia cutis (LC). Numerous abnormalities of chromosome 8 (trisomy or tetrasomy) have been identified in association with LC. We performed fluorescent in situ analysis on cutaneous tissue using directly labelled probes for various gene loci often involved in patients with AML; these tests showed deletion of p53 and excluded trisomy 8. However, application of probes for AML/ETO, MYC and telomere 8q revealed a gain at 8q22/8q24/8q telomere in a significant number of infiltrating cells. We hypothesize that a partial gain at 8q rather than trisomy of the whole chromosome 8 exhibits an association with LC in AML.

Large speckled lentiginous naevus superimposed with Spitz naevi: sequential digital dermoscopy may lead to unnecessary excisions triggered by dynamic changes.

We report a 14-year-old girl with a large speckled lentiginous naevus (SLN) on her left arm and shoulder. As the occurrence of melanoma within SLN has been described previously, long-term follow-up of atypical lesions by digital dermoscopy was started at the age of 4 years. To date, nine Spitz naevi and four dysplastic compound naevi have been excised due to dynamic changes over time. No melanoma has so far been detected. We critically discuss the possibility of an 'overtreatment' because of a high rate of physiological changes within SLN of children. In conclusion, we would like to encourage a close follow-up of large SLN whenever complete excision is not an option. In order to avoid unnecessary excisions triggered by subtle dynamic changes, a standard approach with overview images, conventional dermoscopy and early excision of lesions that are rated as suspicious for melanoma by established algorithms may be recommended.

Molecular cytogenetic analysis of chromosomal breakpoints in the IGH, MYC, BCL6, and MALT1 gene loci in primary cutaneous B-cell lymphomas.

Chromosomal translocations affecting the IGH locus and various oncogene loci are recurrent in many types of systemic B-cell lymphomas. Hardly any data exist, however, on such translocations in primary cutaneous B-cell lymphomas (PCBCL). Here, a series of 29 PCBCL was investigated by interphase fluorescence in situ hybridization with probes for the IGH, MYC, BCL6, and MLT1 loci. None of the six follicle center cell lymphomas and nine marginal zone lymphomas showed evidence for any translocation affecting these loci. In contrast, 11 of 14 large B-cell lymphomas of the leg harbored breakpoints in at least one of the loci. Translocations involving the MYC locus were detected in six cases, five of them derived from a MYC/IGH juxtaposition and one from a translocation involving a non-IG gene partner. Rearrangements of the BCL6 locus were detected in five B-cell lymphomas of the leg, and involved IGH (two cases), IGL (one case), and non-IG genes (two cases). This study shows that large B-cell lymphomas of the leg display a pattern of chromosomal translocations similar to their systemic counterparts whereas primary cutaneous follicle center cell lymphomas and marginal zone lymphomas lack these typical chromosomal translocations.