AI Article Synopsis
- Scleredema adultorum of Buschke is a condition characterized by thickening of the skin, particularly starting at the neck and spreading, which can limit mobility due to increased dermal stiffness.
- Diagnosis relies on histological evidence of mucin deposits and is differentiated from other sclerosing diseases, with three subtypes linked to infections, paraproteins, or diabetes.
- Treatment includes physiotherapy and therapies like ultraviolet irradiation, along with addressing underlying conditions, particularly focusing on optimizing diabetes management to reduce insulin use.
Article Abstract
Scleredema adultorum of Buschke is an important differential diagnosis in sclerosing diseases. Diagnosis is based on the typical histology with mucin deposits and grossly increased dermal width and a clinical presentation of diffuse non-pitting induration of the skin starting at the nape of the neck and interscapular region extending to shoulders and upper thorax, causing dysmobility due to dermal stiffness. Even though the pathogenesis remains unclear, three subtypes can be distinguished: association with infections, paraproteins, or most frequently with diabetes mellitus. Management of the disease includes physiotherapy, physical therapies such as ultraviolet (UV) or ionizing irradiation, intravenous immunoglobulins and interdisciplinary treatment directed at associated diseases. Optimizing diabetes therapy and thereby decreasing insulin use may confer significant improvement.
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| http://dx.doi.org/10.1007/s00105-024-05305-y | DOI Listing |
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