Parry-Romberg Syndrome Associated with Hypothyroidism.

Lee, Yeon Hee; Lee, Seong Eun; Kim, Jung Yeul; Kim, Kyoung Nam; Lee, Sung Bok

doi:2017.58.7.857

J Korean Ophthalmol Soc > Volume 58(7); 2017 > Article

Case Report

Journal of the Korean Ophthalmological Society 2017;58(7):857-861.
DOI: https://doi.org/10.3341/jkos.2017.58.7.857 Published online July 15, 2017.

Parry-Romberg Syndrome Associated with Hypothyroidism.

Yeon Hee Lee, Seong Eun Lee, Jung Yeul Kim, Kyoung Nam Kim, Sung Bok Lee

Department of Ophthalmology, Chungnam National University School of Medicine, Daejeon, Korea. sblee@cnu.ac.kr

갑상샘기능저하증이 동반된 패리-롬버그 증후군 1예

이연희⋅이성은⋅김정열⋅김경남⋅이성복

충남대학교 의과대학 안과학교실

Correspondence: Sung Bok Lee,
Email: sblee@cnu.ac.kr

Received: 30 March 2017 • Revised: 8 May 2017 • Accepted: 17 June 2017

Abstract

PURPOSE
We report a case of Parry-Romberg syndrome associated with right enophthalmos, hypotropia, up-gaze limitation, en coup the sabre, morphea, and hypothyroidism. CASE SUMMARY: A 41-year-old woman presented with slowly progressive right enophthalmos that started 5 years previous. With a Hertel exophthalmometer, the right eye measured 10 mm, and the left eye 13 mm. Right enophthalmos and atrophy of subcutaneous tissues of right side orbit were observed. Axial scan of computerized tomography shows atrophy of right side orbital subcutaneous tissues without bony abnormality. At primary gaze, 4 prism diopter hypotropia was observed in the right eye. Up-gaze was limited in both eyes. Visual acuity was 1.0 in both eyes. No specific sign was found in her anterior segment. The patient had experienced right side alopecia 20 years prior and had undergone biopsy, which proved morphea. Linear en coup de sabre morphea was found on the right forehead and scalp. Hypothyroidism due to Hashimoto's thyroiditis was diagnosed based on a blood test, which also showed positive antinuclear antibody. Thus, the patient was diagnosed with Parry-Romberg syndrome associated with hypothyroidism. CONCLUSIONS: Clinicians need to consider the rare disease Parry-Romberg syndrome when a patient with ophthalmologic symptoms such as enopthlamos or eye movement disorders is accompanied by dermatologic symptoms such as linear en coup de sabre morphea or alopecia.

Key Words: Parry-Romberg syndrome;Progressive facial hemiatrophy