J Korean Ophthalmol Soc.  2017 Jul;58(7):857-861. 10.3341/jkos.2017.58.7.857.

Parry-Romberg Syndrome Associated with Hypothyroidism

• Lee YH
• Lee SE
• Kim JY
• Kim KN
• Lee SB

• KMID: 2387161
• DOI: http://doi.org/10.3341/jkos.2017.58.7.857

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.

Keyword

Parry-Romberg syndrome; Progressive facial hemiatrophy

MeSH Terms

Adult
Alopecia
Antibodies, Antinuclear
Atrophy
Biopsy
Enophthalmos
Facial Hemiatrophy*
Female
Forehead
Hematologic Tests
Humans
Hypothyroidism*
Ocular Motility Disorders
Orbit
Rare Diseases
Scalp
Scleroderma, Localized
Subcutaneous Tissue
Thyroid Gland
Thyroiditis
Visual Acuity
Antibodies, Antinuclear

Figure

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  Figure 1. Clinical photograph and Computed tomography showing right enophthalmos. (A) Right enophthalmos was observed at clinical photograph. (B) Axial scan of computed tomography shows right enophthalmos. There was no specific bony abnormality.

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  Figure 2. At primary gaze, 4 prism diopter hypotropia was observed at right eye. Up-gaze was limited in both eyes.

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  Figure 3. Clinical photographs showing alopecia and linear scleroderma. (A) Linear hair loss was observed on patient’s right scalp (yellow arrows). (B) Clinical photograph shows linear scleroderma (en coup de sabre, red arrows) on patient’s forehead with mild hemifacial atrophy on right side.

Reference

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