Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept.

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis of unknown etiology. The familial subtype is rare and usually presents as type V PRP. It is generally inherited in an autosomal dominant fashion with variable expression. Other forms of inheritance, such as autosomal recessive and X-linked, have also been reported. The use of etanercept in treating resistant forms of PRP is promising given reports of its success in a few cases. Herein, the authors report two cases of PRP arising in a mother and son and review the rare familial subtype of this disease. In addition, a successful therapeutic trial of etanercept was initiated in the mother based on case reports of its efficacy in other patients with PRP.

Postoperative calcium requirements in 6,000 patients undergoing outpatient parathyroidectomy: easily avoiding symptomatic hypocalcemia.

BACKGROUND: To determine the amount and duration of supplemental oral calcium for patients with varying clinical presentations discharged immediately after surgery for primary hyperparathyroidism. STUDY DESIGN: A 4-year, prospective, single-institution study of 6,000 patients undergoing parathyroidectomy for primary hyperparathyroidism and discharged within 2.5 hours. Based on our previous studies, patients are started on a sliding scale of oral calcium determined by a number of preoperative measures (ie, serum calcium, body weight, osteoporosis) beginning 3 hours postoperation and decreasing to a maintenance dose by week 3. Patients reported all hypocalcemia symptoms daily for 2 weeks. RESULTS: Seven parameters were found to have a substantial impact on the amount of calcium required to prevent symptomatic hypocalcemia: preoperative serum calcium >12 mg/dL, >13 mg/dL, and >13.5 mg/dL, bone density T score less than -3, morbid obesity, removal of >1 parathyroid, and manipulation/biopsy of all remaining glands (all p < 0.05). Each independent variable increased the daily calcium required by 315 mg/day. Using our scaled protocol, <8% of patients showed symptoms of hypocalcemia, nearly all of whom were successfully self-treated with additional oral calcium. Only 6 patients (0.1%) required a visit to the emergency room for IV calcium, all occurring on postoperative day 3 or later. CONCLUSION: After outpatient parathyroidectomy, a specific calcium protocol has been verified that eliminates development of symptomatic hypocalcemia in >92% of patients, identifies patients at high risk for hypocalcemia, and allows self-medication with confidence in a predictable fashion for those patients in whom symptoms develop.