Case report
A 20 years old female presented to Department of Dermatology and Venerology, BP Koirala Institute of Health sciences with appearance of depigmented macule on right half of the forehead and upper eyelid in the distribution of ophthalmic (V1) nerve with leukotrichia including right eyebrow, eyelashes and frontal scalp hair and a hyperpigmented, atrophied and indurated lesion on the the right half of the chin along mandibular (V3) distribution. The patient first noticed depigmentation of skin at the age of 7, followed few months later by hyperpigmentation on right half of chin which gradually progressed over a period of 4 years to form atrophic and indurated plaque with deviation of mouth and nose towards the affected side. There was no history of trauma or injury or vaccination to the site prior to onset of lesion, family history of similar lesions, diminished vision headache, seizures or difficulty in opening mouth.
On examination, a well-defined depigmented macule of size 4cm × 3 cm was present on right half of forehead and right upper eyelid with leukotrichia including frontal scalp hair, right eyebrow and eyelashes (Figure 1 a and b). Similarly, a hyperpigmented indurated atrophic plaque was present on right half of chin with visible asymmetry towards the right half (Figure 1 a and b).
The general physical examination other than cutaneous examination was unremarkable. Laboratory studies including complete blood cell count, erythrocyte sedimentation rate (ESR), thyroid function tests, anti-nuclear antibody and urine analysis were all negative or within normal ranges. Radiological features of the skull showed no bony involvement. Skin biopsy from atrophic plaque revealed epidermal atrophy with homogenistion of dermis, markedly reduced adnexal structures and pulled up appearance of subcutis which was consistent with Morphea (Figure 2 a and b).
She was managed with topical Tacrolimus 0.1%, cream Fluticasone 0.05% and oral Chloroquine 125 mg three times a day for a duration of 3 months for progressive hemifacial atrophy and topical Tacrolimus 0.1%, cream Fluticasone 0.05%, topical PUVA and betamethasone oral mini pulse for vitiligo for 3 months after which the disease progression stopped. The disease has remained static since last 9 years and she is planned for surgical correction of the progressive hemifacial atrophy (figure c).