A Case of Lamellar Ichthyosis with Rickets and Carcinoma of the Hypopharynx

Lamellar ichthyosis (LI) is an autosomal recessive disorder rarely associated with systemic organ involvement and development of carcinoma. Rickets has occasionally been described with LI owing to impaired vitamin D synthesis following altered keratinization. There has also been a high association of cutaneous cancers in patients of LI. We as Dermatologists should therefore be very meticulous while doing a full work up of these patients. We report here a case of LI associated with rickets and carcinoma of the hypopharynx.     

Prevalence of Metabolic Syndrome In South Indian Patients with Psoriasis Vulgaris and the Relation Between Disease Severity and Metabolic Syndrome: A Hospital-Based Case-Control Study

Background:

Psoriasis is a chronic inflammatory disease of the skin and joints with an increased cardiovascular risk. Previous studies have shown a higher prevalence of metabolic syndrome (MS) in psoriatic patients.

Objective:

To investigate the prevalence of MS in patients with psoriasis and healthy controls, and to determine the relation between disease severity and the presence of MS.

Materials and Methods:

We performed a hospital-based case-control study on 118 adult patients with psoriasis vulgaris and 120 controls matched for age, sex and body mass index. MS was diagnosed by the presence of three or more of the South Asian Modified National Cholesterol Education Program''s Adult Panel III criteria.

Results:

MS was significantly more common in psoriatic patients than in controls (44.1% vs. 30%, P value = 0.025). Psoriatic patients also had a higher prevalence of triglyceridemia (33.9% vs. 20.8%, P value = 0.011), abdominal obesity (34.7% vs. 32.5%, P value = 0.035) and elevated blood sugar. There was no difference in the high density lipoprotein (HDL) levels and presence of hypertension among patients with psoriasis and normal controls. There was no correlation between the severity and duration of psoriasis with MS.

Conclusion:

MS is frequent in patients with psoriasis. We have found no relationship between disease severity and presence of MS. Hence, we suggest that all patients must be evaluated for the MS, irrespective of the disease severity.     

The increased prevalence of non-alcoholic fatty liver disease in psoriatic patients: A study from South India

Background: Psoriasis is an inflammatory disease of the skin and joints. Previous studies have shown a higher prevalence of metabolic syndrome (MS) in psoriatic patients. Recent studies show that non‐alcoholic fatty liver disease (NAFLD) is also frequent in psoriasis patients. Objectives: To investigate the occurrence and severity of NAFLD in South Indian psoriatic patients and healthy controls. Methods: In phase 1 we performed a case control study on 333 adult psoriasis patients and 330 controls matched by age, sex and body mass index. NAFLD was diagnosed by ultrasonography and liver enzymes after excluding other liver diseases. In phase 2 we compared the NAFLD subgroups in psoriasis patients and controls by determining their fibrosis, steatosis and non‐alcoholic steatohepatitis (NASH) scores. Results: The occurrence of NAFLD was higher in psoriasis patients than in controls (17.4 vs 7.9%; P = 0.002). NAFLD patients in the psoriasis group (n = 58) were more likely to have MS (P = 0.03) and diabetes (P = 0.02) than those with psoriasis alone (n = 254). The former group had a longer duration of psoriasis and arthritis (P = 0.003 and 0.005). Psoriasis patients with NAFLD had more severe disease as per the psoriasis area and severity index scores (P = 0.02). Psoriasis patients had more severe NAFLD than controls as reflected by the steatosis, NASH and fibrosis scores (P = 0.001, 0.003, 0.03 respectively). Conclusion: NAFLD is the commonest liver disease in Indian psoriatic patients when compared to controls. As NAFLD is more severe in psoriasis patients we suggest routine screening for NAFLD in this group especially when systemic therapy is considered.     

Sitosterol xanthomatosis

BACKGROUND: Sitosterolaemia is a lipid disorder in which plasma plant sterol levels are extremely elevated. Sitosterolaemia is clinically characterized by tuberous and tendon xanthomas, premature vascular disease and arthritis. OBJECTIVE: To report a case of sitosterolaemia diagnosed by cutaneous manifestations and to review this rare disease. METHODS: We report the case of a 60-year-old woman who presented with cutaneous xanthomas, arterial hypertension and polyarthralgias. The patient had had hypercholesterolaemia for many years without reduction of serum cholesterol, despite treatment with fenofibrate. RESULTS: Ezetimibe therapy was started, decreasing sitosterol plasmatic levels and tuberous xanthomas after 3 months of treatment. CONCLUSION: It is important to detect levels of sitosterol in plasma in patients with premature vascular disease, presence of xanthomas, and uncontrolled hypercholesterolaemia. Ezetimibe therapy is effective.