Ann Dermatol Vener
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Porphyria cutanea tarda (PCT) is associated with cutaneous accumulation of porphyrins. This accumulation results from a deficiency of uroporphyrinogen decarboxylase occurring only in the liver. The classical presentation is blistering on sun-exposed areas. ⋯ Sclerodermatous lesions are an unusual presentation of PCT and cause delays in diagnosis. The accumulation of uroporphyrins in the dermis stimulates fibroblasts, which then synthesize collagen, resulting in cutaneous sclerosis.
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Paget's disease of the breast is rare, even more so when it occurs in a supernumerary breast since diagnosis is delayed due to lack of exploration and ignorance of the pathology of ectopic breast. Based on an original clinical case, we provide a clinical update concerning ectopic breast and ectopic carcinoma, including Paget's disease. ⋯ An ectopic breast, present in 6 % of the population, is often ignored, despite being subject to the same diseases as anatomical breasts: it is at the origin of 0.6 % of breast cancers. Paget's disease is a rare in situ adenocarcinoma comprising fewer than 3 % of cases of breast cancer. It is extremely rare in ectopic breast. Ours is only the fourth reported case in the literature. This case is original because of the lack of objective clinical signs. Subjective symptoms such as pain, tingling, burning sensation and pruritus related to the supernumerary breast must be taken into consideration in order to enable early diagnosis of Paget's disease. Exploration by ultrasound and mammography generally appears flawed. Breast MRI may be useful but is not performed routinely. A promising future approach may consist of confocal microscopy screening of all ectopic breasts with areola or nipple patches, since this method enables the visualization of Paget cells and their organization.
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Pseudoaneurysm of the superficial temporal artery causes tumefaction in the temporal region. Herein, we report two cases. ⋯ Pseudoaneurysm of the superficial temporal artery must be considered for all temporal cutaneous formations, particularly when there is a history of trauma. The clinical diagnosis may be confirmed by Doppler ultrasound. Surgery is the treatment of reference.
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Von Willebrand disease (VWD) and hemophilia A and B are the most common types of hereditary coagulation-factor deficiencies. The frequency and type of complications of skin surgery in these patients are unknown. The increasing incidence of skin cancer prompted us to reflect upon this issue. While the incidence of skin cancer is increasing, the complications of skin surgery or ablative laser treatment remain unknown in this population. ⋯ The rate of hemorrhagic complications was higher than in the general population. However, these complications can be considered non-serious and the risk-benefit ratio remains favorable. Multidisciplinary management and coordination with the reference hemophilia center are mandatory in this population to establish a coagulation-factor (CF) substitution protocol suited to the disease characteristics and the surgical procedure.
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[Late-onset systemic sclerosis: A retrospective study of 27 patients diagnosed after the age of 70].
The aim of this study was to describe special features of patients with systemic sclerosis (SSc) diagnosed after the age of 70. ⋯ Cases of SSc diagnosed after 70 years are mostly limited cutaneous forms. Suspicion of PAH is frequent, and PAH may be the main initial sign of the disease for patients at this age. There may be association with synchronous cancer. Survival is poor.