Frontal linear scleroderma (en coup de sabre)From the Ronald O. Perelman Department of Dermatology, New York University
Kenneth A Katz MD MSc
Dermatology Online Journal 9(4): 10
En coup de sabre is a type of linear scleroderma that presents on the frontal or frontoparietal scalp. The case of a 54-year-old woman with a 3 and one-half year history of en coup de sabre is presented. The clinical presentation, laboratory findings, epidemiology, etiologic hypotheses, and treatment options are reviewed.
History.—A 54-year-old woman presented with a dermatosis involving the frontal scalp and forehead of 3 and one-half years duration. In April 1998 the patient first noticed an indented white spot on her upper forehead. The lesion was occasionally pruritic and accompanied by a pulling sensation on the left side of her forehead and scalp. She subsequently noted expansion of the lesion inferiorly as well as hair loss in the affected area of the scalp. She does not recall having been bitten by a tick, has no family history of connective-tissue disease, and has no pain in her joints. Color changes in her fingers are consistent with Raynaud phenomenon. Prior to her presentation at the Charles C. Harris Skin and Cancer Pavilion in July 2001, she had been treated with a 6-week course of doxycycline as well as a 1-month course of colchicine. A biopsy specimen was obtained in July 2001 from an erythematous area of a plaque on the frontal scalp. She has subsequently been treated with intralesional glucocorticoid injections of triamcinolone acetonide 2.5 mg/ml and triamcinolone hexacetonide 2.5 mg/ml.
Physical examination.—A hypopigmented, atrophic, linear plaque was present in the center of the forehead. The plaque extended superiorly to the frontal scalp, where it was slightly erythematous. There was alopecia in the frontal scalp in the area of the plaque. The face was normal and symmetric.
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Laboratory data.—An anti-nuclear antibody, rheumatoid factor, eosinophil count, and serum protein electrophoresis were normal. An anti-single-stranded DNA serology was not obtained.
Histopathology.—Hematoxylin and eosin stained sections show epidermal atrophy and thick collagen bundles in the deeper portions of the reticular dermis. There is an associated sparse, superficial and deep, perivascular and periadnexal infiltrate of lymphocytes with a few plasma cells. A Verhoeff-van Gieson stain shows a decrease in elastic fibers in the deep reticular dermis, with the remaining fibers arranged parallel to the epidermal surface.
Diagnosis.—Frontal linear scleroderma (en coup de sabre).
En coup de sabre is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp. Multiple lesions of en coup de sabre may coexist in a single patient, with one report suggesting that the lesions followed Blaschko lines . Unlike skin in localized morphea, skin in linear scleroderma may be fixed to underlying tissue. Calcinosis may rarely occur. Cutaneous changes accompanying the facial hemiatrophy associated with the Parry-Romberg syndrome may be similar to those found in en coup de sabre . Serologic abnormalities may include anti-nuclear antibodies, anti-single-stranded DNA antibodies, and rheumatoid factor. Eosinophilia may be present and may correlate with disease activity. A polyclonal IgG and IgM hypergammaglobulinemia may also be present and is found more often with severe cases and with clinical progression .
On the basis of a retrospective analysis of patients who developed morphea between 1960 and 1993 in Olmsted County Minnesota, the incidence of en coup de sabre is 0.13 cases per 100,000 population . Of the 82 cases of morphea identified in that study, 16 patients had linear scleroderma, including 4 with en coup de sabre and 2 with Parry-Romberg syndrome. None of these patients developed systemic sclerosis, although progression from linear scleroderma to systemic disease has been reported. Skin softening or disease resolution occurred in 8 of 16 patients within 5 years of diagnosis.
As in other types of scleroderma, the etiology of en coup de sabre is unknown. Hypotheses include microchimerism, which leads to a chronic, low-grade graft-versus-host-like disease, or an alteration in antigens caused by ischemic damage . Borrelia burgdorferi DNA has been identified by polymerase chain reaction assays in tissue sections from some,  but not all,  patients with localized scleroderma.
Modalities used in the management of en coup de sabre have included topical, intralesional, or systemic glucocorticoids; vitamin E; vitamin D3; phenytoin; retinoids; penicillin; griseofulvin; interferon-(x), D-penicillamine; antimalarials; ultraviolet A phototherapy with or without psoralens; and surgery .
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