Progressive Facial Hemiatrophy

Abstract

fact is the more remarkable since progressive facial hemiatrophy hardly presents diagnostic or therapeutic problems of magnitude. It is rather the great variety of clinical features and the pathogenesis of this puzzling disease that have attracted such widespread attention. From this standpoint the following clinical observations are noteworthy. REPORT OF CASESCase 1 (National Hospital Queen Square, London, service of the late S. A. K. Wilson).\p=m-\Thepatient was a 20 year old girl. Her complaints were: wasting of the right side of the face since the age of 7 years; fits since the age of 15 ; general headache and knifelike pains through the right side of the head for four or five years, and recent weakness of the whole left side.When she was 4 years old, a "white spot" was noticed on the right side of her forehead, near the midline. This spot gradually spread upward to the scalp, which became completely bald in that area. When she was 7 years old, it was noticed that the right side of the head sank in; for the four years prior to her admission to the hospital this depression had remained stationary.At the age of 7 she had for the first time a sudden attack of a tight feeling spreading up from the left thumb to the shoulder, the face and down the leg, the left foot being affected last. There was no loss of consciousness. This was followed by pain down the whole left side of the body and by muscular twitchings ; sometimes the drawing up started in the toes of the left foot and worked into the left arm and the left side of the face. These attacks occurred once in three or four weeks; each of them left her with weakness of the left side, especially of the left arm, which would abate in one or two days. For hours after an attackTo the memory of Samuel Alexander Kinnier Wilson, who suggested this publication.

Progressive Hemifacial Atrophy with Linear Scleroderma

“…In our patient, a unilateral, bandlike, indurated skin lesion on the right submandibular area, facial hemiatrophy of the right mandibular area, hemiatrophy in the right part of the tongue, and histopathologically, cutaneous inflammation were observed. These findings were consistent with a diagnosis of linear scleroderma with hemifacial atrophy, which is similar to the findings of some other authors (14–16,20,21).…”

Progressive Hemifacial Atrophy with Linear Scleroderma

“…In our patient, a unilateral, bandlike, indurated skin lesion on the right submandibular area, facial hemiatrophy of the right mandibular area, hemiatrophy in the right part of the tongue, and histopathologically, cutaneous inflammation were observed. These findings were consistent with a diagnosis of linear scleroderma with hemifacial atrophy, which is similar to the findings of some other authors (14–16,20,21).…”

Scleroderma ‘en coup de sabre’ and progressive facial hemiatrophy. Is it possible to differentiate them?

“…In SCS, predominant unilateral involvement of the parietal and frontal area has often been referred to in the literature, and in PHF involvement of the whole hemiface is often referred to; 1,5,6 this was confirmed in our series because total hemifacial involvement was present in six of nine patients with PFH and in only five of 13 with SCS.…”

“…As has been referred in the literature, 1–8 in both SCS and PFH, females were predominant in our series. In 10 of 13 cases of SCS and in all with PFH, the initial lesions appeared during the first decade of life.…”

Parry-Romberg syndrome with band-like alopecia

“…1 The eyelashes, eyebrows and scalp hair on the involved side may be affected and ipsilateral alopecia may occur. 2 Although alopecia is commonly seen, the distinct morphology of the longitudinal band like form as evident in the present case has not been described with this condition.…”

Progressive Hemifacial Atrophy with Linear Scleroderma

“…In our patient, a unilateral, bandlike, indurated skin lesion on the right submandibular area, facial hemiatrophy of the right mandibular area, hemiatrophy in the right part of the tongue, and histopathologically, cutaneous inflammation were observed. These findings were consistent with a diagnosis of linear scleroderma with hemifacial atrophy, which is similar to the findings of some other authors (14–16,20,21).…”

Scleroderma ‘en coup de sabre’ and progressive facial hemiatrophy. Is it possible to differentiate them?

“…In SCS, predominant unilateral involvement of the parietal and frontal area has often been referred to in the literature, and in PHF involvement of the whole hemiface is often referred to; 1,5,6 this was confirmed in our series because total hemifacial involvement was present in six of nine patients with PFH and in only five of 13 with SCS.…”

“…As has been referred in the literature, 1–8 in both SCS and PFH, females were predominant in our series. In 10 of 13 cases of SCS and in all with PFH, the initial lesions appeared during the first decade of life.…”

Parry-Romberg syndrome with band-like alopecia

“…1 The eyelashes, eyebrows and scalp hair on the involved side may be affected and ipsilateral alopecia may occur. 2 Although alopecia is commonly seen, the distinct morphology of the longitudinal band like form as evident in the present case has not been described with this condition.…”

Progressive Hemifacial Atrophy with Linear Scleroderma

“…In our patient, a unilateral, bandlike, indurated skin lesion on the right submandibular area, facial hemiatrophy of the right mandibular area, hemiatrophy in the right part of the tongue, and histopathologically, cutaneous inflammation were observed. These findings were consistent with a diagnosis of linear scleroderma with hemifacial atrophy, which is similar to the findings of some other authors (14–16,20,21).…”

Scleroderma ‘en coup de sabre’ and progressive facial hemiatrophy. Is it possible to differentiate them?

“…In SCS, predominant unilateral involvement of the parietal and frontal area has often been referred to in the literature, and in PHF involvement of the whole hemiface is often referred to; 1,5,6 this was confirmed in our series because total hemifacial involvement was present in six of nine patients with PFH and in only five of 13 with SCS.…”

“…As has been referred in the literature, 1–8 in both SCS and PFH, females were predominant in our series. In 10 of 13 cases of SCS and in all with PFH, the initial lesions appeared during the first decade of life.…”

Parry-Romberg syndrome with band-like alopecia

“…1 The eyelashes, eyebrows and scalp hair on the involved side may be affected and ipsilateral alopecia may occur. 2 Although alopecia is commonly seen, the distinct morphology of the longitudinal band like form as evident in the present case has not been described with this condition.…”