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).