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Pigmentary Changes
Published in Giuseppe Micali, Pompeo Donofrio, Maria Rita Nasca, Stefano Veraldi, Vulval Dermatologic Diagnosis, 2015
Francesco Lacarrubba, Aurora Tedeschi, Giuseppe Micali
Definition: Hyperpigmented spots due to benign epidermal melanocytic hyperplasia of less than 4 mm in diameter (lentigo simplex) or larger (vulvar melanosis). Multiple lesions (lentiginosis) may sometimes occur in the setting of a congenital and/or inherited disorder (e.g., Peutz–Jeghers and LEOPARD syndromes or somatic mosaicism).
Importance of cardiovascular examination in patients with multiple lentigines: two cases of LEOPARD syndrome with hypertrophic cardiomyopathy
Published in Acta Clinica Belgica, 2019
Tomas Jurko, Alexander Jurko, Jana Krsiakova, Alexander Jurko, Milan Minarik, Michal Mestanik
Although LEOPARD syndrome is a rare condition, it is supposedly underdiagnosed as manifestations can be mild [4]. Diagnosis of LEOPARD syndrome during the first months/years of life is very difficult and it can be clinically suspected in patients who have three main features: characteristic facial features (100%), hypertrophic cardiomyopathy (87%) and café au lait spots (75%) [6]. Later, it seems that ventricular hypertrophy usually precedes the occurrence of multiple lentigines and progresses [4,5]. Previously, pulmonary stenosis has been considered to be a typical cardiovascular structural defect associated with LEOPARD syndrome. However, recent reviews suggest that the incidence of pulmonary stenosis is approximately 10–20%, which is much lower compared to the initially documented 40% [5–7]. It has been confirmed, that hypertrophic cardiomyopathy of the left ventricle is more common [5]. The combination of lentiginosis and cardiac abnormalities led to the introduction of the term cardiocutaneous syndrome or later, the designation lentigino-cardiomyopathic syndrome, which appears to be more appropriate since it expresses the characteristic changes on the skin (lentigines) and the heart (cardiomyopathy) [7–10]. Additional but less frequent cardiac abnormalities include mitral valve prolapse and septal defects.
Emerging drugs for the treatment of vitiligo
Published in Expert Opinion on Emerging Drugs, 2020
Priyanka Karagaiah, Yan Valle, Julia Sigova, Nicola Zerbinati, Petar Vojvodic, Davinder Parsad, Robert A. Schwartz, Stephan Grabbe, Mohamad Goldust, Torello Lotti
Clinically the lesions can be categorized into segmental (SV) and non-segmental vitiligo (NSV). SV presents as a focal, dermatomal patch with rapid progression and often associated with leukotrichia. Neural mechanisms were proposed as the probable cause, as SV was believed to follow a dermatomal distribution and several studies have also reported increased neuropeptide release (e.g. neuropeptide Y) in the lesional skin. However, SV need not necessarily follow a dermatomal distribution and more often overlap with the pattern of segmental lentiginosis. Currently, somatic mosaicism and subsequent inflammatory reaction leading to melanocyte destruction is the most plausible theory [12].