Amyloidosis is a group of heterogeneous diseases characterized by pathological deposition of proteinaceous substance extracellularly in various tissues. The clinical presentation depends on the site of amyloid deposition, with predominant involvement of mesenchymal elements and cutaneous findings in 30–40% of patients in case of primary systemic amyloidosis. We present a case of idiopathic primary systemic amyloidosis presenting with an unusual finding of nodulo-ulcerative lesion over tongue along with multiple skin-colored nodules, mimicking squamous cell carcinoma of tongue with secondary cutaneous metastasis, as well as lacking the classical presentation of purpura, macroglossia, waxy papules, and plaques.
<p class="abstract"><strong>Background:</strong> Psoriasis, a common skin disorder is now considered as systemic inflammatory disease. Its chronic inflammatory state is thought to predispose patients to metabolic syndrome (MetS), which is a significant predictor of cardiovascular events. The objective of the study is to investigate the prevalence of metabolic syndrome in psoriatic patients with only cutaneous involvement and to evaluate the correlation between presence of MetS and psoriasis severity</p><p class="abstract"><strong>Methods:</strong> We performed hospital based, cross-sectional study on 100 adult patients with psoriasis vulgaris and equi-numbered age and sex matched controls. MetS was diagnosed by revised National Cholesterol Education Program’s Adult Panel III criteria.</p><p class="abstract"><strong>Results:</strong> A higher prevalence of metabolic syndrome was found in psoriasis patients as compared to controls [28 (28%) vs 9 (9%), P value=0.0005].Impaired fasting glucose level, hypertension, hypertriglyceridemia and abdominal obesity were more prevalent in psoriasis group. No statistically significant difference was found between prevalence of low levels of HDL and presence of metabolic syndrome. Presence of metabolic syndrome was not associated with severity and duration of psoriasis. Female patients with psoriasis were more frequently having metabolic syndrome.</p><strong>Conclusions:</strong> Prevalence of metabolic syndrome is higher in patients with psoriasis irrespective of its duration and severity. This stresses on the need of regular evaluation for the presence of MetS or any of its components in psoriasis patients so as to allow early detection and management.
Woolly hair is a hair shaft disorder characterized by fine and tightly curled hair. Woolly hair could be syndromic (associated with systemic disease) or non-syndromic (not associated with any systemic disease). Woolly hair is described in association with many skin, dental, ophthalmic and cardiac anomalies but association of woolly hair with bilateral systematized epidermal nevus described in our case is first of its kind.
Erythromelanosis follicularis faciei et colli (EFFC) is a rare disease characterized by a triad of reddish-brown pigmentation, erythema and follicular papules localized on face and neck and is usually described in males. Erythrosis pigmentosa mediofacialis (also known as Brocq or erythrosis pigmentosa peribuccalis) is a similar disorder of the mediofacial area but with female predominance. We report a case of simultaneous occurrence of erythrosis pigmentosa peribuccalis and EFFC associated with keratosis pilaris in an adolescent female.
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