Phacomatosis cesiomarmorata with hypospadias and phacomatosis cesioflammea with Sturge-Weber syndrome, Klippel-Trenaunay syndrome and aplasia of veins -- case reports with rare associations.

Phacomatosis pigmentovascularis (PPV) is a rare genodermatosis characterized by the co-existence of an extensive vascular and a pigmentary nevus with or without extracutaneous manifestations. We report two such rare cases. The first is a 3-year-old boy exhibiting a rare association of cutis marmorata telangiectatica congenita with aberrant dermal melanocytosis along with hypospadias and melanosis oculi (traditionally classified as PPV type Vb or phacomatosis cesiomarmorata - Happle's classification). The other patient is a 5-year-old boy with Sturge-Weber syndrome, Klippel-Trenaunay syndrome, aplasia of iliac, femoral, and popliteal veins and congenital heart disease, associated with aberrant dermal melanocytosis and melanosis oculi (also classified as PPV type IIb or phacomatosis cesioflammea). These sporadic cases display a unique constellation of additional, previously unreported systemic associations, which will further expand the clinical spectrum of phacomatosis pigmentovascularis.

Is Early Onset Androgenic Alopecia a Marker of Metabolic Syndrome and Carotid Artery Atherosclerosis in Young Indian Male Patients?

BACKGROUND: Androgenic alopecia (AGA) is a common cosmetically and psychosocially distressing condition. High androgen level contributes to the development of atherosclerosis, thrombosis leading to hypertension and hypercholesterolemia. OBJECTIVES: To study the clinico-epidemiological profile of AGA and the presence of metabolic syndrome (MetS) and carotid artery atherosclerosis in male patients with early onset AGA as compared to controls. MATERIALS AND METHODS: In this case-control study, 100 male patients of age 18-35 years with AGA and an equal number of age-matched healthy controls attending skin and STD OPD were included. Assessment of the degree of hair loss, evaluation of MetS and carotid artery color Doppler for the atherosclerotic plaque was done in all patients. RESULTS: Statistically significant number of patients with early onset AGA 22/100 (22%) (P < 0.05) fulfilled the criteria for MetS compared to 8/100 (8%) in the control group. There were statistically significant differences in mean values of waist circumference, serum triglycerides, serum cholesterol, systolic blood pressure, diastolic blood pressure, fasting glucose concentration, and very low-density lipoprotein (LDL). However, no significant differences were observed in the mean values of high-density lipoprotein cholesterol and LDL cholesterol. The atherosclerotic plaque was found in two patients of the study group, and no plaque was found in control patients. CONCLUSION: We suggest that all men with AGA should be thoroughly investigated, and lifestyle changes should be started in the early period of life so as to reduce the risk of various problems associated with MetS. AGA can be considered as an early marker for MetS.