ALBRIGHT HEREDITARY OSTEODYSTROPHY

By Gary M. White, MD


Albright's hereditary osteodystrophy (AHO) is a syndrome which combines pseudohypoparathyroidism (secondary to end-organ resistance to PTH), decreased calcium, increased PO4, short stature, obesity, round face, and brachydactyly. The main skin finding is bone formation (osteoma cutis--OC). In contrast to actual hypoparathyroidism, the PTH level is normal or even increased.

Clinical

The lesions of osteoma cutis may occur anywhere, but have a predilection for sites around large joints and areas of trauma. The lesions initially may be pinpoint in size and then enlarge, e.g., becoming 5-8 cm in size. Shortening of the metacarpal bones may occur in patients with AHO.

Treatment

With regard to treatment of the osteoma cutis, see osteoma cutis.

References

BMJ. 2008 Jun 7; 336(7656): 1298–1302.

A 68-year-old woman with a history of mild acne vulgaris as a teenager presented complaining of multiple facial lesions. These asymptomatic lesions first appeared at the age of 16 years. Her past medical history was notable for hysterectomy because of uterine calcification in her 50s. Her face was typical shape but hadmany scattered one to three millimeter skin-colored papules apparent on the face, cheeks, and forehead. Notably, the fifth digits of both the right and left hands were significantly shortened Dermatology Online Journal 23(3)

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.