Papillon-Lefevre syndrome: case report

Papillon-Lefevre syndrome [PLS] is a rare genodermatosis of autosomal recessive inheritance manifesting as palmoplantar hyperkeratosis with periodontitis. A 4-year old Iranian girl was referred to the Mazandaran University Hospital, Sari complaining of well-demarcated, psoriasiform, yellowish, keratotic plaques over the skin of her palms and soles with transgrediens. For cutaneous lesions, she was treated with oral acitretin 0.5 mg/kg/day and topical keratolytic 5% salicylic acid in combination with 10% urea

Epidemiologic evaluation of patients with blistering lesions in sari: a retrospective study

Background/Objective: Blistering diseases of skin are characterized by presence of lesions containing watery liquids. The aim of our study was to apply the histopathological and demographical evaluation of the patients whose medical records were in the archive of pathology unit of Buali hospital in Sari

Methods and Materials: This was a descriptive retrospective study included medical records of 87 patients with bullous lesions during years 2003-2013 which were archived in the pathology unit of the hospital, were reviewed and evaluated. Biopsies were taken by dermatologist after sterilizing of the bullous lesion with betadine and applying local anesthesia with lidocaine 1%. After appropriate tissue slicing, fixation and H and E staining, the samples were observed under microscope by two pathologists. Histopathological and demographic data [such as age, sex, area of involvement, diagnosis] were recorded. The gathered information were analyzed by SPSS Ver. 16 in terms of central and peripheral statistic distribution

Results: The population of our study was 87 patients and all of them had bullous lesions. the most common diagnoses was Pemphigus Vulgaris with 36 patients [41.4%], then Bullous Pemphigoid with 22 patients [25.3%], Dermatitis Herpetiformis with 8 patients [9.2%] , Pemphigus Foliaceus with 6 patients [6.9%], Epidermolysis Bullosa with 4 patients [4.6%] Pemphigus Erythematosis with 3 patients [3.4%] and Subcorneal Pustular Dermatosis with 1 patient [1.1%] and 7 cases had uncertain diagnoses

Conclusion: Our study demonstrated that the frequency of diagnosis of skin bullous lesions in city of Sari is almost similar to other related studies and the most common diagnoses are pemphigus vulgaris, bullous pemphigoid, herpetiform dermatitis and pemphigus foliaceus. The ratio of female to male was 1.17:1