Cutaneous leiomyosarcoma with myxoid alteration arising in a setting of multiple cutaneous smooth muscle neoplasms.

BACKGROUND: Cutaneous leiomyomas and leiomyosarcomas are rare tumors that originate from the arrector muscle of hair follicles or the smooth muscle of blood vessels. CASE REPORT: A 74-year-old male presented with a single, erythematous nodule on the left upper arm. This lesion developed within the excision scar of a piloleiomyoma that had been excised 3 years ago. Additionally, physical examination revealed a tender nodule on the right ear. RESULTS: Histological examination showed a cutaneous leiomyosarcoma with myxoid alteration on the upper arm and an angioleiomyoma on the right ear. CONCLUSION: Myxoid leiomyosarcomas have exclusively been reported in the uterus and gastrointestinal tract, but not in the skin. Here, we describe a case of cutaneous leiomyosarcoma with myxoid alteration that developed in the excision scar of a piloleiomyoma and was accompanied by a cutaneous angioleiomyoma in a different location.

[Angioma serpiginosum following the lines of Blaschko--an effective treatment with the IPL technology]. / Angioma serpiginosum in den Blaschkolinien--eine effektive Behandlung mit der IPL Technik.

Angioma serpiginosum, first described by Hutchinson in 1889, is a rare benign vascular nevus with dilatation and proliferation of the capillaries in the upper dermis. A 15-year-old boy presented with an angioma serpiginosum on the right side of the body following the lines of Blaschko. Both the clinical pattern and the appearance in a male are unusual. Treatment with IPL technology (intense pulsed light), which emits polychromatic light from a high-intensity flashlamp, proved to be an effective approach.

Functional characterization of the epidermal cholinergic system in vitro.

The aim of this study was to analyze the influence of cholinergic and anticholinergic drugs on epidermal physiology using organotypic cocultures (OTCs). Blocking of all acetylcholine receptors (AChRs) by combined treatment with mecamylamine and atropine or treatment with strychnine (blocking alpha9nAChR) for 7-14 days resulted in a complete inhibition of epidermal differentiation and proliferation. Blockage of nicotinic (n)AChR with mecamylamine led to a less pronounced delay in epidermal differentiation and proliferation than blockage of muscarinic (m)AChR with atropine, evidenced by reduced epithelial thickness and expression of terminal differentiation markers like cytokeratin 2e or filaggrin. In OTCs treated with atropine, mecamylamine, or strychnine, we could demonstrate intracellular lipid accumulation in the lower epidermal layers, indicating a severely disturbed epidermal barrier. In addition, we observed prominent acantholysis in the basal and lower suprabasal layers in mecamylamine-, atropine-, and strychnine-treated cultures, accompanied by a decreased expression of cell adhesion proteins. This globally reduced cell adhesion led to cell death via intrinsic activation of apoptosis. In contrast, stimulation of nAChR and mAChR with cholinergic drugs resulted in a significantly thickened epithelium, accompanied by an improved epithelial maturation. In summary, we show that epidermal AChR are crucially involved in the regulation of epidermal homeostasis.

Additional Her 2/neu gene copies in patients with Sézary syndrome.

BACKGROUND: Her 2/neu gene amplification has been reported in several types of cancer. Monoclonal antibodies against the Her 2/neu receptor are used as a treatment in e.g. metastatic breast cancer. AIM: The aim of this study was to determine the frequency of additional Her 2/neu gene copies in relations to the number of chromosome 17 centromeres of patients with Sézary syndrome. METHODS: Fluorescence in situ hybridization (FISH) with probes specific for the Her 2/neu gene locus and the centromere of chromosome 17 was performed on nuclei from peripheral blood cells of 9 patients with Sézary syndrome. For analysis of Her 2/neu protein expression immunostaining was performed. In addition, FISH was used to analyze distribution of typical lymphocytes on cryo-cut sections of affected skin of two patients. RESULTS: 7/9 cases showed additional Her 2/neu gene copies in relation to the number of chromosome 17 centromeres. 4/5 cases with additional Her 2/neu gene copies in which immunostaining was performed expressed Her 2/neu protein. On cryo-cut sections atypical lymphocytes with additional Her 2/neu gene copies were detected in the dermis as well as in the epidermis of affected skin. DISCUSSION: These data suggest that Her 2/neu might be involved in the pathogenesis of Sézary syndrome and that Her 2/neu might be a promising target for antitumor therapy in a subgroup of patients.

[Ulceration of the scalp: a unique manifestation of pyoderma gangrenosum]. / Ulzera der Kopfhaut: eine spezielle Manifestation des Pyoderma gangraenosum.

Pyoderma gangrenosum is a chronic, noninfectious, ulcerating cutaneous disease of unknown etiology usually presenting with multiple lesions on the legs and trunk. Solitary lesions in other locations are rare and therefore hard to diagnose. A 78-year-old and a 81-year-old woman with extensive scalp necrosis were both diagnosed as pyoderma gangrenosum after exclusion of other differential diagnostic possibilities. The separate diagnosis of malignant pyoderma for this disease state no longer seems justified. Scalp necrosis is infrequently seen in dermatological patients; most previous reports describe it in giant cell arteritis. Other differential diagnoses include viral, bacterial and mycotic infections as well as erosive pustular dermatosis of the scalp. In conclusion, we again draw attention to unilesional pyoderma gangrenosum of the scalp as a rare disease entity. Immunosuppressive drugs are an effective treatment modality.