Benign lymphangioendothelioma is a rare locally infiltrative vascular neoplasm, presenting as a slow-growing, asymptomatic, reddish-violaceous plaque. Histopathologically, it is characterized by irregular and thin-walled vascular spaces, lined by a single and discontinuous layer of flat endothelial cells, dissecting dermal collagen bundles. We present the case of a 75-year-old man with a giant benign lymphangioendothelioma, to our knowledge, the largest example described in the literature. The immunohistochemical expression of Wilms tumor 1 gene is useful in vascular lesions to differentiate malformations from proliferative endothelial lesions. In our case, the positivity for WT1 supports the neoplastic nature of this lesion.
Osteoma cutis is a rare lesion that consists of the presence of bone tissue within the dermis and/or hypodermis. It may be classified as primary osteoma cutis, when bone tissue develops in the skin without any pre-existing lesion and secondary osteoma cutis, which is more frequent and occurs when osseous tissue develops on a pre-existing lesion. We present a case of primary plaque-like osteoma cutis involving the scalp, left forehead and left cheek, which appeared in an adult male. Histopathological study showed several islands of mature osseous tissue involving the full thickness of the dermis. In some areas, there was also transepidermal elimination of bone spicules. We review the literature about previously reported similar cases.
Late-onset focal dermal elastosis is a rare disorder of elastic tissue, characterized by a local accumulation of elastic fibers in the mid and deep reticular dermis. This disorder occurs mainly in elderly people and consists of multiple yellow papules that are typically distributed on the sides of the neck and flexural areas, closely resembling pseudoxanthoma elasticum from the clinical point of view. We report 2 sisters who have presented with a 20-year history of multiple yellow papules, mainly located on the dorsum of their hands. They also had few lesions of similar morphology scattered on the anterior aspects of their wrists, thighs, and lower abdomen. Histopathologic study demonstrated a focal increase in thick, interlacing elastic fibers in the mid and deep reticular dermis. The most striking features of our cases were the early age of clinical presentation, the familial involvement, and the predominantly acral distribution of the lesions.
Lipoatrophic panniculitis is the term used to describe those panniculitides in which atrophy of the subcutaneous fat is the main residual lesion. They can be classified as primary or secondary. Primary lipoatrophic panniculitis is idiopathic, whereas secondary lipoatrophic panniculitides include those because of infection, malignancy, pancreatic disorders or connective tissue diseases. Usually, these entities are characterized by the appearance of tender, erythematous nodules or plaques that resolve leaving one or several localized areas with loss of subcutaneous fat. We report a 76-year-old female who developed diffuse and symmetric lipoatrophy of lower limbs after an extensive inflammatory process affecting both extremities from thighs to ankles. Histopathologic assessment revealed a lobular panniculitis with a lymphohistiocytic infiltrate, foamy macrophages and lipophagic granuloma formation. The most striking feature in our patient was the clinical presentation as a symmetrical diffuse inflammatory process resulting in lipoatrophy of the lower limbs.
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