- Email: [email protected]
Linear scleroderma in 6 patients
P605
P607
Drug-induced dermatomyositis? Nicole Brey, MD, University of Louisville, Louisville, KY, United States; Jeffrey Callen, MD, University of Louisville, Louisville, KY, United States; Janine Malone, MD, University of Louisville, Louisville, KY, United States
Successful treatment of generalized morphea with infliximab: A case report Mohammad Diab, MD, Ohio State University-Dermatology, Columbus, OH, United States; Melissa Pilewskie, Ohio State University College of Medicine, Columbus, OH, United States; Mark Bechtel, MD, Ohio State UniversityDermatology, Columbus, OH, United States; Gwyn Frambach, Ohio State University College of Medicine, Columbus, OH, United States Generalized morphea (GM) is a subtype of localized scleroderma that manifests as widespread indurated cutaneous plaques that can lead to significant scaring and joint disability. We report, along with clinical and histologic photos, the use of infliximab in a treatment of a case GM with lichen sclerosis atrophicans overlap that failed to respond to traditional therapies. Following 4 monthly injections of infliximab, the patient showed significant reduction in cutaneous sclerosis and dyschromia. GM is a subtype of localized scleroderma that manifests as widespread indurated cutaneous plaques that can lead to significant scaring and joint disability.
Introduction: Classic dermatomyositis (DM) is an idiopathic connective tissue disorder characterized by a symmetric, proximal inflammatory myopathy and a characteristic cutaneous eruption. It has been postulated that DM results from an immune-mediated process triggered by environmental factors in genetically predisposed individuals. We report a case of DM that was precipitated by statins and a fibrate derivative. Observation: A 50-year-old woman was referred for evaluation of a recurrent photosensitive eruption that began in February 2006, 1 month after starting fenofibrate. She initially experienced a similar eruption associated with weakness in August 2001 when she began pravastatin. Atorvastatin (2002) and simvastatin (2004) also produced a similar eruption. Each episode has been accompanied by a positive ANA and a slight elevation of creatine kinase. The resolution of each episode has involved cessation of the lipid-lowering agent and the use of prednisone. Physical examination revealed photodistributed violaceous plaques on the face and chest and a heliotrope eruption. An EMG study failed to demonstrate a myopathy. Laboratory evaluation in April 2006 revealed normal serum chemistries and autoantibodies. Two punch biopsies were consistent with a collagen vascular disease. The patient was placed on a prednisone taper, pimecrolimus, and photoprotection practices. Discussion: The pathogenesis of DM may be related to an underlying genetic predisposition. Loss of self-tolerance induced by UV radiation, viruses, and/or pharmacologic agents has been proposed to induce immune dysregulation producing clinical disease. Nine cases of HMG-CoA reductase inhibitor-induced DM have previously been reported. Of these cases, the majority of patients have been elderly, the eruption appeared within 2 to 3 months after initiating the drug, and subsequently resolved spontaneously or after corticosteroid therapy within months after discontinuing the medication. Serologic testing, EMG evaluation, or muscle biopsy has revealed evidence of muscle involvement in these cases. HMG-CoA reductase inhibitors are well known for inducing a toxic myopathy, however, recent reports have also indicated that the class may also be responsible for triggering autoimmune diseases. Our case is unique because she developed the eruption with two previously reported statins as well as a third agent, atorvastatin. This is the first reported case of DM induced by a fibrate derivative.
Commercial support: None identified.
Commercial support: None identified.
P606 Linear scleroderma in 6 patients Maria Carolina Lopez Santoro, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina; Maria Ines Hernandez, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina; Mariana Alejandra Ferreira, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina; Alejandra Abeldan˜o, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina We describe 6 female patients with linear scleroderma: 3 with progressive facial hemiatrophy (PFH), 1 with localized scleroderma ‘‘en coup de sabre,’’ and 2 with compromised limbs. Physical examinations revealed that those with PFH had unilateral involvement of forehead and cheek hyperpigmentation as well as soft and depressed skin. The patient with linear scleroderma ‘‘en coup de sabre’’ presented unilateral hyperpigmented, depressed, and indurated skin on the forehead, in addition to homolateral cicatrizal alopecia. In the last 2 cases, one displayed hyperpigmented and slight sclerosis in the upper left limb consistent with linear atrophoderma of Moulin, while the other had hyperpigmented skin with severe sclerosis in her left lower limb. Ophthalmic, otic, and neurologic abnormalities were investigated (clinical examination, electroencephalogram, and CT scan) in the 4 patients with cephalic localization. Hypoacusis was found in 1 patient with PFH, who received methotrexate during 2 years until stabilization of progression. The patient with ‘‘coup de sabre’’ was treated with topical calcipotriol with successful results, and the other 2 patients are under control with no progression of the disease. Neurologic and articular compromise was ruled out in the females with affected limbs. The patient with lower limb involvement is under treatment with methotrexate. Linear scleroderma frequently occurs in the limbs, but may also develop in the frontoparietal area. When it occurs on the head, it is referred to as linear scleroderma ‘‘en coup de sabre,’’ given its resemblance of the skin lesions to the stroke of a saber. PFH (ParryeRomberg syndrome) is a related disorder characterized by progressive hemifacial atrophy without cutaneous sclerosis. Debate continues as to whether Parry-Romberg syndrome is a distinct disease entity or a variant of linear scleroderma. Therefore, screening of ophthalmic, otic, and neurologic involvement should be ruled out in both entities considering the multiple reports of their condition. When an affected extremity is present, limb articulation and neuropathy should be studied in order to avoid complications. Commercial support: None identified.
AB66
J AM ACAD DERMATOL
P608 A unique case of generalized and widepread necrobiosis lipoidica Keren Ben-Ari Maoz, MD, Ichilov Sourasky Tel Aviv Medical Center, Tel Aviv, Israel; Itay Klaz, MD, Ichilov Sourasky Tel Aviv Medical Center, Tel Aviv, Israel; Sarah Brenner, MD, Ichilov Sourasky Tel Aviv Medical Center, Tel Aviv, Israel Necrobiosis lipoidica (NL) is a rare degenerative connective tissue disease of unknown etiology, characterized histologically by the presence of palisading granulomas and collagen degeneration leading to cutaneous atrophy. Coexisting with diabetes in about 0.3% of cases. Ulcers, often induced by trauma, occur in 35% of lesions. We present a unique case of a patient with generalized NL. A 59-year-old female developed type II diabetes and a widespread involvement of skin with multiple atrophic lesions compatible histologically with necrobiosis lipoidica. The lesions first appeared 13 years ago on postoperational scar tissue spreading to the shins and demonstrating subsequent ulceration. Lesions were highly resistant to local or systemic treatment. To our knowledge, this is the first case report of such a widespread involvement of the skin total body surface (\60%) by NL. Commercial support: None identified.
FEBRUARY 2007
P607
Drug-induced dermatomyositis? Nicole Brey, MD, University of Louisville, Louisville, KY, United States; Jeffrey Callen, MD, University of Louisville, Louisville, KY, United States; Janine Malone, MD, University of Louisville, Louisville, KY, United States
Successful treatment of generalized morphea with infliximab: A case report Mohammad Diab, MD, Ohio State University-Dermatology, Columbus, OH, United States; Melissa Pilewskie, Ohio State University College of Medicine, Columbus, OH, United States; Mark Bechtel, MD, Ohio State UniversityDermatology, Columbus, OH, United States; Gwyn Frambach, Ohio State University College of Medicine, Columbus, OH, United States Generalized morphea (GM) is a subtype of localized scleroderma that manifests as widespread indurated cutaneous plaques that can lead to significant scaring and joint disability. We report, along with clinical and histologic photos, the use of infliximab in a treatment of a case GM with lichen sclerosis atrophicans overlap that failed to respond to traditional therapies. Following 4 monthly injections of infliximab, the patient showed significant reduction in cutaneous sclerosis and dyschromia. GM is a subtype of localized scleroderma that manifests as widespread indurated cutaneous plaques that can lead to significant scaring and joint disability.
Introduction: Classic dermatomyositis (DM) is an idiopathic connective tissue disorder characterized by a symmetric, proximal inflammatory myopathy and a characteristic cutaneous eruption. It has been postulated that DM results from an immune-mediated process triggered by environmental factors in genetically predisposed individuals. We report a case of DM that was precipitated by statins and a fibrate derivative. Observation: A 50-year-old woman was referred for evaluation of a recurrent photosensitive eruption that began in February 2006, 1 month after starting fenofibrate. She initially experienced a similar eruption associated with weakness in August 2001 when she began pravastatin. Atorvastatin (2002) and simvastatin (2004) also produced a similar eruption. Each episode has been accompanied by a positive ANA and a slight elevation of creatine kinase. The resolution of each episode has involved cessation of the lipid-lowering agent and the use of prednisone. Physical examination revealed photodistributed violaceous plaques on the face and chest and a heliotrope eruption. An EMG study failed to demonstrate a myopathy. Laboratory evaluation in April 2006 revealed normal serum chemistries and autoantibodies. Two punch biopsies were consistent with a collagen vascular disease. The patient was placed on a prednisone taper, pimecrolimus, and photoprotection practices. Discussion: The pathogenesis of DM may be related to an underlying genetic predisposition. Loss of self-tolerance induced by UV radiation, viruses, and/or pharmacologic agents has been proposed to induce immune dysregulation producing clinical disease. Nine cases of HMG-CoA reductase inhibitor-induced DM have previously been reported. Of these cases, the majority of patients have been elderly, the eruption appeared within 2 to 3 months after initiating the drug, and subsequently resolved spontaneously or after corticosteroid therapy within months after discontinuing the medication. Serologic testing, EMG evaluation, or muscle biopsy has revealed evidence of muscle involvement in these cases. HMG-CoA reductase inhibitors are well known for inducing a toxic myopathy, however, recent reports have also indicated that the class may also be responsible for triggering autoimmune diseases. Our case is unique because she developed the eruption with two previously reported statins as well as a third agent, atorvastatin. This is the first reported case of DM induced by a fibrate derivative.
Commercial support: None identified.
Commercial support: None identified.
P606 Linear scleroderma in 6 patients Maria Carolina Lopez Santoro, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina; Maria Ines Hernandez, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina; Mariana Alejandra Ferreira, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina; Alejandra Abeldan˜o, MD, Hospital General de Agudos Doctor Cosme Argerich, Buenos Aires, Argentina We describe 6 female patients with linear scleroderma: 3 with progressive facial hemiatrophy (PFH), 1 with localized scleroderma ‘‘en coup de sabre,’’ and 2 with compromised limbs. Physical examinations revealed that those with PFH had unilateral involvement of forehead and cheek hyperpigmentation as well as soft and depressed skin. The patient with linear scleroderma ‘‘en coup de sabre’’ presented unilateral hyperpigmented, depressed, and indurated skin on the forehead, in addition to homolateral cicatrizal alopecia. In the last 2 cases, one displayed hyperpigmented and slight sclerosis in the upper left limb consistent with linear atrophoderma of Moulin, while the other had hyperpigmented skin with severe sclerosis in her left lower limb. Ophthalmic, otic, and neurologic abnormalities were investigated (clinical examination, electroencephalogram, and CT scan) in the 4 patients with cephalic localization. Hypoacusis was found in 1 patient with PFH, who received methotrexate during 2 years until stabilization of progression. The patient with ‘‘coup de sabre’’ was treated with topical calcipotriol with successful results, and the other 2 patients are under control with no progression of the disease. Neurologic and articular compromise was ruled out in the females with affected limbs. The patient with lower limb involvement is under treatment with methotrexate. Linear scleroderma frequently occurs in the limbs, but may also develop in the frontoparietal area. When it occurs on the head, it is referred to as linear scleroderma ‘‘en coup de sabre,’’ given its resemblance of the skin lesions to the stroke of a saber. PFH (ParryeRomberg syndrome) is a related disorder characterized by progressive hemifacial atrophy without cutaneous sclerosis. Debate continues as to whether Parry-Romberg syndrome is a distinct disease entity or a variant of linear scleroderma. Therefore, screening of ophthalmic, otic, and neurologic involvement should be ruled out in both entities considering the multiple reports of their condition. When an affected extremity is present, limb articulation and neuropathy should be studied in order to avoid complications. Commercial support: None identified.
AB66
J AM ACAD DERMATOL
P608 A unique case of generalized and widepread necrobiosis lipoidica Keren Ben-Ari Maoz, MD, Ichilov Sourasky Tel Aviv Medical Center, Tel Aviv, Israel; Itay Klaz, MD, Ichilov Sourasky Tel Aviv Medical Center, Tel Aviv, Israel; Sarah Brenner, MD, Ichilov Sourasky Tel Aviv Medical Center, Tel Aviv, Israel Necrobiosis lipoidica (NL) is a rare degenerative connective tissue disease of unknown etiology, characterized histologically by the presence of palisading granulomas and collagen degeneration leading to cutaneous atrophy. Coexisting with diabetes in about 0.3% of cases. Ulcers, often induced by trauma, occur in 35% of lesions. We present a unique case of a patient with generalized NL. A 59-year-old female developed type II diabetes and a widespread involvement of skin with multiple atrophic lesions compatible histologically with necrobiosis lipoidica. The lesions first appeared 13 years ago on postoperational scar tissue spreading to the shins and demonstrating subsequent ulceration. Lesions were highly resistant to local or systemic treatment. To our knowledge, this is the first case report of such a widespread involvement of the skin total body surface (\60%) by NL. Commercial support: None identified.
FEBRUARY 2007