|THROUGH THE LENS
|Year : 2017 | Volume
| Issue : 5 | Page : 382-383
Old age comes early
Harsha Siddappa, Leena Raveendra
Department of Dermatology, Rajarajeswari Medical College and Hospital, Bengaluru, Karnataka, India
|Date of Web Publication||15-Sep-2017|
Department of Dermatology, Rajarajeswari Medical College and Hospital, Bengaluru - 560 074, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Siddappa H, Raveendra L. Old age comes early. Indian Dermatol Online J 2017;8:382-3
A 40-year-old female patient born to a second-degree consanguineous marriage presented to us with complaints of premature graying of hair and diffuse loss of hair over the scalp, eyelashes, and eyebrows since 3 years. There was history of developing multiple depigmented and hyperpigmented macules over both the forearms, legs, and upper chest at the same time. She also gave a history of weight loss since 2 years, which was associated with thinning and wrinkling of the skin over both the upper limbs and lower limbs. Hoarseness of voice was present since the past 2 years. She was operated for bilateral cataract at the age of 12 years.
She gave history of 3 still birth deliveries. She attained menopause at the age of 30 years. The patient had 4 other siblings. One of her sister also had similar complaints.
On examination, the patient was poorly built and nourished. Her weight was 25 kg. Wrinkling of the skin and generalized loss of subcutaneous fat and muscles was present all over the body. Depigmented and hyperpigmented macules were present all over the body, predominantly over the extensor aspect of both upper and lower limbs and V-area of the chest. Graying of hair and diffuse loss of hair over the scalp, eyebrows, and eyelashes was seen [Figure 1].
|Figure 1: Graying of hair over the scalp, eyebrows, and eyelashes. Wrinkling of the skin and loss of subcutaneous fat seen over the face. Depigmented macules present at median angle of the eye|
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X-ray of the limbs showed reduced bone density and calcification of tendoachillies. Ultrasound of the abdomen and pelvis was normal except for atrophic uterus, and ovaries were not visualized. Histopathological examination of the skin showed increased deposition of collagen in the dermis suggestive of sclerodermoid changes.
The patient presented to us with classical clinical features of Werner's syndrome (late-onset premature graying of hair, loss of subcutaneous fat and muscles, hoarseness of voice, bilateral cataractomy, sclerodermoid changes of skin, osteoporosis, and calcification of tendons).
Werner's syndrome (Pangeria/Progeria adultorum) is a hereditary premature aging syndrome affecting connective tissues throughout the body. Mutations have been identified in the DNA helicase gene (WRN, RECQL2). It is known to affect multiple systems of the body and may be associated with internal malignancy. Werner's syndrome patients must be kept under regular follow-up to look for any signs of malignancy and other systemic involvement.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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