Systemic amyloidosis presenting as mucocutaneous bullous lesions

A 65-year-old male presented with hemorrhagic bullous skin lesions with purpura and ecchymoses. There was increased skin fragility with a strongly positive Nikolsky sign. Histopathology of the skin revealed large amounts of amyloid deposits in the dermis with a positive Congo Red staining around the dermal vessels. Examination and tests in this patient also revealed anemia, hepatomegaly, infiltrative cardiomyopathy, polyneuropathy and immunoglobulin λ deposition, favoring a diagnosis of primary amyloidosis (AL type). The present case is reported in view of the rarity of the bullous variant of primary systemic amyloidosis as well as presence of mucosal lesions and a positive Nikolsky sign

Hirsutism in Kashmir: An etiological study

<b>Background: </b> Hirsutism refers to the presence of terminal hairs at the body sites under androgenic control. Various factors, including genetic makeup and hormonal status, influence the rate and pattern of hair growth at these sites.<b> Purpose: </b> To study the pattern of hirsutism in Kashmir<b> . Materials and Methods: </b> Thirty five consecutive patients of hirsutism were included in the study. After detailed history taking, physical examination and relevant investigations, scoring of hirsutism was done using the Ferriman Gallwey (FG) scoring system.<b> Findings: </b> The FG score ranged from 10-34. Twenty patients had associated menstrual abnormalities. Polycystic ovarian syndrome (PCOS) was diagnosed in four patients, hypothyroidism in two and congenital adrenal hyperplasia (CAH) in one. The rest of the patients had idiopathic hirsutism.<b> Conclusion: </b> Idiopathic hirsutism was the most common category, whilst PCOS, hypothyroidism and CAH were also seen

Epidermolysis bullosa: A series of 12 patients in Kashmir valley

<b>Background:</b> Epidermolysis Bullosa (EB) is a genetically determined mechano-bullous disorder of the skin encompassing a group of conditions that share skin fragility as a common feature. <b>Materials and Methods:</b> Twele patients with Epidermolysis Bullosa from Kashmir valley are reported. <b>Results:</b> Our series included 12 patients, 5 males and 7 females. Features were consistent with EB simplex in 8 patients, EB pruriginosa in 2 patients, generalized atrophic benign EB in one patient and EB acquista in one patient. <b>Conclusion:</b> EB is a rare, genetically determined, blistering disorder affecting both males and females with predominant involvement of hands and feet. In the absence of specific therapy, treatment mainly involves avoidance of provoking factors, prevention and treatment of complications