Porphyrias amongst patients with cutaneous photosensitivity in Kashmir: A seven year experience

摘要

Porphyrias are rare, exact prevalence differs, and need to be excluded in patients with typical medical, psychiatric, and surgical history. Cutaneous porphyrias present with skin lesions, photosensitivity, and anemia. Coordination between Deptt. of Dermatology and specialized porphyria testing laboratory in Department of Biochemistry made possible diagnosis, treatment of porphyrias and this pioneering major referral hospital-based 7-year study, from August 2003 to August 2010, to analyze percentage of porphyrias amongst patients with cutaneous photosensitivity from Kashmiri population. No such prior study had been done. Patients with cutaneous photosensitivity, attending OPD and IPD of hospitals associated with Govt. Medical College Srinagar, underwent routine physical examination, relevant biochemical and hematological tests. After confirming normal ANA and ds-DNA levels, 601 symptomatic patients negative for hepatitis and HIV were referred for porphyria testing as part of differential diagnosis comprised study sample. Mathematical and statistical analysis included calculation and comparison of percentage of types of porphyrias diagnosed. As a specialized porphyria testing center of Kashmir, we estimated our data covers 85-95% of all diagnosed cases in Kashmir. Our aim was effective management of cutaneous photosensitivity by improving awareness of porphyrias, seemingly very rare disorder, amongst medical professionals.