Popat Vijay; Vora Deval; Patel Pratik; Maan Pratibha; Brahmbhatt Vinita
Volume 15, Issue 4 , 2012, , Pages 131-133
Abstract
Ehlers-Danlos syndrome (EDS) is a clinically heterogeneous groupof inherited disorders with ten different types, all involving agenetic defect in collagen and connective-tissue synthesis andstructure that affecting the skin, joints, and blood vessels. EDStype VIA, a very rare kyphoscoliotic type, is ...
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Ehlers-Danlos syndrome (EDS) is a clinically heterogeneous groupof inherited disorders with ten different types, all involving agenetic defect in collagen and connective-tissue synthesis andstructure that affecting the skin, joints, and blood vessels. EDStype VIA, a very rare kyphoscoliotic type, is autosomal recessiveand clinically characterized by soft extensible skin, laxity ofjoints and kyphoscoliosis. We present a case of an adult malepatient complaining of recurrent acne over the chin, axilla andpyoderma in the groin region since he was 14 years of age. Pastmedical history was positive for recurrent pyoderma whichhealed with scarring, bleeding tendency, double vision andbackache. On examination, the patient had hyperextensible skin,joint hypermobility, kyphoscoliosis, easy bruisability, and scleralfragility. Urinary analysis revealed a decrease in the hydroxylysylpyidinolineto lysl-pyridinoline ratio indicative of EDS type VIAwith a severely reduced Lysyl Hydroxylase (LH) activity in theskin fibroblast culture..
Vora Deval; Popat Vijay; Bhanvadia Viral; Mehta Dimple A.; Bhetariya Bharat; Kumar Meet
Volume 13, Issue 2 , 2010, , Pages 42-46
Abstract
Background: A clinicopathological study of 75 cases of pemphigus was carried out at Saurashtra region of Gujarat State, India. This study was done to subtype this disease with evaluation of clinical and histopathological presentation. Methods: The study was carried out in a two-year period at skin department ...
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Background: A clinicopathological study of 75 cases of pemphigus was carried out at Saurashtra region of Gujarat State, India. This study was done to subtype this disease with evaluation of clinical and histopathological presentation. Methods: The study was carried out in a two-year period at skin department of Guru Gobind Singh Hospital, Jamnagar. Seventy five patients of pemphigus were examined, admitted and treated. Histopathological examination was done at the department of pathology and direct immunoflouroscence was advised and done at higher centers when it was needed. Result: Out of 75 cases, pemphigus vulgaris constituted the single largest group of cases which made 72 (96%) cases with three (4%) cases of pemphigus foliaceus. The majority of the cases were seen in the age group of 21-60 years, with a slight female predominance. The youngest patient was 18 years while the eldest was 70 years old. Oral mucosal involvement was seen in almost all cases of pemphigus vulgaris while 7 cases of pemphigus vulgaris had mucosal involvement at other sites in addition to oral mucosal involvement. No mucosal involvement was present in cases of pemphigus foliaceus. Flaccid bullae were present in 100% of the cases. Pruritus was present in 15 cases of pemphigus vulgaris. Conclusion: Most of the patients had pemphigus vulgaris followed by pemphigus foliaceus. Pemphigus is more prevalent at certain areas of Saurashtra region showing a geographic distribution.
