Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete
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Ichthyoses comprise a heterogeneous group of Mendelian disorders of cornification (MEDOC) affecting the entire skin and characterized by hyperkeratosis and/or scaling. The genetic basis of almost all ichthyosis forms has been elucidated. In 2009, the worldwide first Ichthyosis Consensus Classification was approved. ⋯ Ichthyoses due to keratin mutations are referred to as KPI/keratinopathic ichthyosis and include epidermolytic ichthyosis (EI) and superficial epidermolytic ichthyosis (SEI). In Germany the Network for Ichthyoses and Related Keratinization Disorders (NIRK) and the patient organization Selbsthilfe Ichthyose e. V. provide contact points for diagnostic and therapeutic questions.
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Pyoderma gangrenosum is a rare neutrophilic inflammatory skin disease, mostly observed in middle-aged adults. Etiology and pathogenesis remain unclear. Autoimmune mechanisms including immune complex-mediated neutrophilic vascular reactions have been suggested. ⋯ The aims of therapy are the complete suppression of inflammatory disease activity, promotion of wound healing and control of pain. Frequently, successful treatment of associated diseases leads to an improvement or complete remission of pyoderma gangrenosum. Surgical interventions, including aggressive ulcer excision, recipient site preparation and autologous skin grafting have to be avoided during the active phase of the disease because the likely occurrence of pathergy inducing new lesions at surgical sites and causing a worsening the original lesions.
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A 22-year-old woman presented with a superficial spreading melanoma on her right thigh (tumor thickness 1.0 mm, Clark-Level III). She also had decorative tattoos on her right ankle, right groin and coccyx. The staging results gave no indication for metastases. ⋯ Clinical differentiation between tattoo pigments and metastatic disease within lymph nodes is not possible. Histological confirmation of an enlarged pigmented lymph node is therefore essential before radical surgery is performed. Hence, accumulation of tattoo pigment within enlarged and pigmented lymph nodes needs to be included into the differential diagnosis and the documentation of decorative tattoos is important during skin cancer screening as well as during the follow-up of melanoma patients.
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Case Reports
[Solarium-induced pseudoporphyria and variegate porphyria as rare differential diagnoses of porphyria cutanea tarda].
Three patients presented with typical porphyria cutanea tarda-like vesicles, erosions and scars as well as increased fragility, primarily on the back of the hands. In two of the three, porphyrin workup was normal. Skin biopsy was compatible with porphyria cutanea tarda (PCT) or pseudoporphyria. ⋯ Further investigations revealed a metastasized carcinoma of the colon, which probably triggered the acute attack. Our cases show rare differential diagnoses in patients presenting with typical PCT-like skin lesions. The discrimination between porphyria cutanea tarda and its differential diagnoses is very important since it has an important impact not only on the treatment modality but also on the course and the prognosis of the disease.