ABSTRACT
BACKGROUND: Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. METHODS: We reviewed six localized scleroderma cases that were initially treated with medication and then received follow-up surgery between April 2003 and February 2015. Six patients had facial lesions. These cases presented with linear dermal sclerosis on the forehead, oval subcutaneous and dermal depression in the cheek. RESULTS: En coup de sabre (n=4), and oval-shaped lesion of the face (n=2) were successfully treated. Surgical methods included resection with or without Z-plasty (n=3), fat graft (n=1), dermofat graft (n=1), and adipofascial free flap (n=1). Deformities of the affected parts were surgically corrected without reoccurrence. CONCLUSION: We retrospectively reviewed six cases of localized scleroderma that were successfully treated with surgery. And we propose an algorithm for selecting the best surgical approach for individual localized scleroderma cases. Although our cases were limited in number and long-term follow-up will be necessary, we suggest that surgical management should be considered as an option for treating scleroderma patients.
Subject(s)
Humans , Cheek , Collagen , Congenital Abnormalities , Contracture , Depression , Extremities , Follow-Up Studies , Forehead , Free Tissue Flaps , Joints , Quality of Life , Retrospective Studies , Scleroderma, Localized , Sclerosis , Skin , TransplantsABSTRACT
Parry and Romberg described progressive facial hemiatrophy, characterized by progressive unilateral wasting of the skin, fat, muscle and bone in 1825 and 1846, respectively. Involvement of the central nervous system, with impairment of neurologic function, occurs infrequently. Linear scleroderma on frontal and parietal scalp, named 'en coup de sabre', is considered an overlapping condition. An 11 year-old girl was presented to our office with left facial hemiatrophy and transverse linear band-like alopecia on her posterior parietal scalp. Histopathologically, there was a sclerotic change of the collagen fibers and dermal lymphocytic infiltrations around the skin appendages. Her skin lesion has improved by intralesional corticosteroid injection. Linear scleroderma frequently coexists with Parry-Romberg syndrome. But in this case, she had a unique pattern of scleroderma, which is distinct from the previously reported cases.
Subject(s)
Alopecia , Central Nervous System , Collagen , Facial Hemiatrophy , Muscles , Scalp , Scleroderma, Localized , SkinABSTRACT
Linear scleroderma is a kind of disease that can cause complication of the central nervous system. Sometimes, ipsilateral intracerebral or white matter lesions in the brain magnetic resonance imaging are noted. Nystagmus is important for the differential diagnosis of dizziness. Positional nystagmus was classified into regular direction nystagmus, direction changing positional nystagmus (DCPN) and irregular nystagmus by their character. DCPN is defined as a nystagmus that changes its direction with different head and body positions, and it can be the sign of lesion in the central vestibular system. Recently, we experienced a 17-year-old woman who had a scalp linear scleroderma, treated for about 10 years and showed DCPN induced by positional nystagmus test. We report a case of linear scleroderma accompanied by cerebellar lesion with a brief review of literature.
Subject(s)
Adolescent , Female , Humans , Brain , Central Nervous System , Cerebellum , Diagnosis, Differential , Dizziness , Head , Magnetic Resonance Imaging , Nystagmus, Physiologic , Scalp , Scleroderma, Localized , VertigoABSTRACT
Lichen sclerosus et atrophicus (LSA) is an inflammatory disease that primarily causes anogenital lesion in middle aged women. We present here a case of facial LSA with an asymptomatic, well-demarcated, whitish to bluish, atrophic patch in a linear pattern on the forehead of a 48-year-old woman. This case showed an atypical clinical presentation and it mimicked en coup de sabre, but the histopathologic results confirmed the diagnosis of LSA.
Subject(s)
Female , Humans , Middle Aged , Forehead , Lichen Sclerosus et Atrophicus , Lichens , Scleroderma, LocalizedABSTRACT
Morphea is a sclerotic connective tissue disorder and it is thought to be caused by the decreased activity of collagenase. Various therapeutic modalitiessuch as topical steroid and systemic steroid, antimalarial agents, cyclosporine and phototherapy have been shown to be effective to treat this illness. Recently, PUVA and UVA1 phototherapy for localized scleroderma have been shown to have excellent efficacy. But PUVA and UVA1 phototherapy also have some disadvantages. So another phototherapy modality is warranted. Herein we report on a case of a 7 year female patient with linear scleroderma and she obtained a successful result with NBUVB phototherapy.
Subject(s)
Female , Humans , Antimalarials , Collagenases , Connective Tissue , Cyclosporine , Phototherapy , Polyenes , Scleroderma, LocalizedABSTRACT
BACKGROUND: Localized scleroderma, a rare autoimmune disease, can be classified into three subtypes.linear scleroderma, morphea and generalized morphea. Localized scleroderma is usually limited to the skin and subcutaneous tissue but may result in permanent functional disability. Because of its rarity, there are few large scale clinical studies of pediatric patients with localized scleroderma in Korea. OBJECTIVE: In this study we have examined 11 Korean pediatric patients with localized scleroderma registered at Hanyang university hospital. A review of the clinical presentation, laboratory data, therapy and complcations was carried out in detail. METHODS: The medical records of 11 pediatric patients with localized scleroderma seen at the department at Hanyang University Hospital, between 1990 and 1996, were reviewed. The diagnoses were classified into one of the above three localized scleroderma subtypes based on the results of the physical examination, and all patients were examined periodically. The clinical features of the disease, laboratory results, treatment modalities, and complications were summerized from the review of the records. RESULTS: Among the 11 patients, 8 were female and 3 were male; 7 had morphea and 4 had linear scleroderma. The average age at onset was 8.6 years. Antinuclear antibodies above the titer of 1:40 were present in 36%. One of 3 patients with linear scleroderma which involved the upper extremities had moderate degrees of joint contracture. Ten of 11 patients were treated with intralesional injection of corticosteroid and oral medications of hydroxychloroquine, prednisolone and/or nosteraidal anti-inflammatory drugs. CONCLUSION: Localized scleroderma has no recognized internal organ involvements and is not thought to progress to systemic sclerosis. Since localized scleroderma may cause complications from local atrophy to joint contractures, patients will need to take adequate treatment. and follow-up examinations.