Abstract
The association between psoriasis and hypoparathyroidism has been reported by several authors, and it has been suggested that abnormalities in calcium homeostasis may be involved in the development or exacerbation of psoriasis. However, so far there have only been two reports of pseudohypoparathyroidism associated with psoriasis. To describe the familial occurrence of this association for the first time. Two siblings with psoriasis associated with pseudohypoparathyroidism were presented. The first patient was a 24-year-old white male with disseminated erythrodermic pustular psoriasis that began 2 months before admission. He had had a history of mental retardation, recurrent otitis, seizures and arthralgia from the age of 11 years onwards. He presented the characteristic phenotype of Albright osteodystrophy: short stature, obesity, round facies, broad forehead, short neck and brachydactylia. He adopted a position of flexed limbs and showed proximal muscle weakness and a positive Trousseau sign. He had clinical signs of hypocalcemia (0.69 mmol/l ionized calcium and 3.2 mg/dl total calcium), hyperphosphatemia (6.6 mg/dl), hypomagnesemia (1.0 mEq/l), hypoalbuminemia (3.1 g/dl), normal serum intact PTH levels (45.1 pg/ml), primary hypothyroidism (13.2 mU/ml TSH, and 4.7 mg/dl total T(4)), hypergonadotrophic hypogonadism (116.0 ng/ml LH, 13.2 mU/ml FSH and 325.0 ng/dl testosterone), osteoporosis, and diffuse calcifications in soft tissues and in the central nervous system. The second case was a 14-year-old white girl with a history of psoriasis vulgaris from the age of five years onwards, and antecedents of mental retardation. She presented signs of Albright osteodystrophy (short stature, round facies, obesity, short neck, brachydactylia), hypocalcemia (ionized calcium of 1.08 mmol/l and total calcium of 6.7 mg/dl) hyperphosphatemia (9.4 mg/dl), elevated serum PTH levels (223.0 pg/ml), osteoporosis, and hypergonadotrophic hypogonadism (7.0 mU/ml LH, 9.3 mU/ml FSH and undetectable estradiol levels).
Highlights
CASE REPORTS: Two siblings with psoriasis associated with pseudohypoparathyroidism were presented
We have described an unusual association of psoriasis and Albright osteodystrophy, which peculiarly affected 2 siblings
The term pseudohypoparathyroidism describes a group of disturbances characterized by increased PTH levels, hypocalcemia, hyperphosphatemia, and target tissue unresponsiveness to the biological actions of PTH.[11]
Summary
Psoriasis is a skin disease of unknown etiology with prevalence of 1 to 3%, characterized by increased epidermal proliferation and decreased cell turnover.[1]. The association of psoriasis with hypocalcemia has previously been described by several authors.[4,5,6,7,8,9] in most cases the metabolic disturbance was secondary to hypoparathyroidism In these reports, the normalization of calcium levels via administration of calcium and vitamin D resulted in an improvement in the dermatological condition, suggesting that changes in calcemia may be implicated in the development or exacerbation of psoriasis. Physical examination showed: stature of 155 cm; weight of 61.5 kg; body mass index (BMI) of 25.6 kg/m2; slight central subcutaneous distribution of adiposity; round facies; broad forehead; short neck; disseminated erythematous scaly plaques; frontal parietal baldness; bilateral shortening of second and fourth fingers, first left and first and fourth right toes (Figure 2); symmetric bilateral edema in the legs; positive Trousseau and negative Chvosteck signs He had male genitalia of G4 P5 pubertal stage. X-rays of hands and feet showed diffuse osteoporosis, more accentuated in the joints; diffuse micro-calcifications in soft tissue; bilateral shortening of the second and fourth metacarpal bones and the first left and first
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Schedule a callMore From: Sao Paulo medical journal = Revista paulista de medicina
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