Bilateral facial hemi-atropy

A Gangopadhyay

 Department of Dermatology, RKM Seve Pratisthan, Calcutta, India

Correspondence Address:
A Gangopadhyay
7, B.M.Road, Calcutta-26
India

Introduction

Facial hemi-atropy (FH) is an atropic disorder involving superficial facial tissues - although the underlying muscles, cartilage and bones may be involved. Parry was first to describe this disorder in 1825.[1] But this disorder goes with the name of Romberg who described the disease in great detail in 1946.[2] The exact aetiology of this disease is not known. The lesion occurs in one of the divisions of trigeminal nerve, half of the face or very rarely ipsilaleral side of the body. FH is not a common disorder, still more uncommon is the bilateral involvement. Here is a report of very very rare varient of this rare disorder. To the best of my knowledge this is the first report of bilateral FH from India.

Case Report

A 21-year-old woman presented with facial deformity. The episode started some 10 years back, when she first noticed a faint hyper-pigmented patch on her forehead. As the patch was increasing in size, she realised the left side of her face was gradually getting depressed. Similar changes started occurring on the right side of the face which was also getting depressed slowly. The whole process was progressing slowly to involve both sides of the face, on the forehead, the cheeks and the chin. There was no associated symptom and she was keeping good health all these years. There was no significant family history.

On examination the whole face looked depressed on both sides. Interstingly there was a raised fold on the forehead intervening the bilateral depressions. The skin was not adherent to the deeper structure. The eyebrows were normal, there was no deformity in the teeth, tongue, nailsand the jaw bones [Figure:1] X-ray of the skull and face did not reveal any abnormality.

Histology showed unremarkable epidermis with dermal atropy. The subcutis was atropied.

Discussion

The exact aetiology of facial hemiatrophy (FH) is not known. There is no genetic link, although there are few reports of hereditory incidences. Some cases have been reported with syringomyelia, epilepsy or cardiovascular diseases but in 90% cases no such associations warefound. Another interesting postulation is FH, atrophoderma of Pasini and Pierini, lichen sclerosus et atropicus, eosinophilic fascitis and morphea - all these sclerosing disorders have been associated with Borrelia burgdorferi infection, as evidenced by positive serology for borrelia and by isolating the borrelia in histological section.[3] A possible aetiological basis in these five entities - either as the initiating event as in morphea and wosinophilic fascitis of as an end stage phenomenon (atrophoderma, facial hemi-atrophy and lichensclerosus) may relate to the increased production of interleukin-I mediated by Burgdorferi, resulting in enhanced fibroblast production.[4] This view possibly explains the occational co-exsistance of morphea and FH.

FH is usually limited to one half of the face. However it may be rarely bilateral. This particular case is the rare variant of bilateral atropy. There is some resemblence of this case to morphea. But morphea is a more superficial process and the skin is sclerotic, adherent and there is loss of hair unlike our case, where the skin was normal looking, mobile and there was no loss of eyebrow. However these processes may co-exist.[5]

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