Morphea en coup de sabre: An unusual oral presentation

Abstract

Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. She also presented a hypopigmented line on the left side skin of her upper lip, which continued through the vermilion and the lip mucosa, including the gingiva of the affected teeth. Clinical examination, blood tests, computerized axial tomography, echo-Doppler ultrasound and histopathological evaluation confirmed the diagnosis of morphea. Treatment with methotrexate and systemic corticosteroids was conducted. After 24 months, no other lesions appeared. No adverse side effects have been reported so far. Key words:Localized scleroderma, oral morphea, linear scleroderma, oral involvement, intraoral lesions.

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

“…A progressive recession on teeth 11 and 12 was described in 19-year-old patient with ECDS ( 60 ). A progressive recession on gingiva was shown in 13-year-old girl with ECDS as well ( 28 ).…”

“…Verma and colleagues reported a PHA patient with marked left-sided facial atrophy and wasting of the tongue ( 49 ). Mucosa can be involved in PHA ( 54 ) and in ECDS ( 28 ). In some cases mucosal involvement may be accompanied by orthodontic and maxillofacial changes ( 18 , 20 ).…”

“…As for scleroderma, xerostomia, microstomia, idiopathic resorption of tooth and mucosal erosions are diagnosed previously in diffuse scleroderma ( 58 ). Oral involvement in ECDS is rare, and it may be presented by white linear scar-like fibrotic areas, atrophical tongue papillae, gingival recession, and alveolar bone resorption ( 28 ). Hørberg and colleagues presented a case of ECDS in 6-year and 10-month-old Turkish girl.…”

Systemic Sclerosis Mimics

“…It usually starts as a small plaque with the surrounding inflammatory erythematous rim. Parry-Romberg syndrome is a rare variant of linear morphea of the forehead and scalp, with progressive loss of subcutaneous fat, with a smaller share of sclerosis [3,8,9].…”

A case of linear morphea involving the oral cavity

“…It is an inflammatory disease that results in excessive collagen deposition, sclerosis of involved skin and tissues, and progressive atrophy of involved areas. It may present as a hypopigmented or hyperpigmented linear, indurated plaque that progresses to assume an atrophic, depressed, ivory appearance 1, 2, 3, 4. The disease can lead to alopecia of the scalp and eyelids and may progress into deeper tissues and cause muscle atrophy, damage to nerves, and demineralization of bone and teeth 1, 2, 3, 4, 5, 6.…”