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Calcinosis cutis associated with amyopathic dermatomyositis: Response to intravenous immunoglobulin
1076 Letters
J AM ACAD DERMATOL JUNE 2009
are several case reports in the Japanese literature describing patients with lupus erythematosus panniculitis successfully treated with dapsone, only isolated cases have been reported in the English literature.6 Our case is unusual because of the extensive unilateral involvement of the breast with a carcinoma erysipelatoideseappearing morphology and the presence of telangiectasia on the surface of the skin. In addition, the response to antimalarial drugs was not assessed because she could not have a full course because of an adverse drug reaction. The diagnosis was also difficult because there were no manifestations of lupus elsewhere and her serologic markers were negative. Lupus mastitis may be mistaken for breast carcinoma; therefore, the necessity of a correct diagnosis to avoid useless and harmful surgery is evident. Rosa Ferna´ndez-Torres, MD,a Felipe Sacrista´n, MD,b Jusu´s del Pozo, MD,a Walter Martı´nez, MD,a Luis Albaina, MD,c Marta Mazaira, MD,a and Eduardo Fonseca, MDa Departments of Dermatology,a Pathology,b and General Surgery,c Hospital Juan Canalejo, La Corun ˜ a, Spain Funding sources: None. Conflicts of interest: None declared. Correspondence to: Rosa Ferna´ndez-Torres, MD, Department of Dermatology, Hospital Juan Canalejo, Xubias de Arriba 84, 15006 A Corun ˜ a, Spain E-mail: [email protected] REFERENCES 1. Winkelmann RK. Panniculitis in connective tissue disease. Arch Dermatol 1983;119:336-44. 2. Carducci M, Mussi A, Lisi S, Muscardin L, Solivetti FM. Lupus mastitis: a 2-year history of a single localization of lupus erythematosus mimicking breast carcinoma. J Eur Acad Dermatol Venereol 2005;19:260-2. 3. Whitaker-Worth DL, Carlone V, Susser WS, Phelan N, Grant-Kels JM. Dermatologic diseases of the breast and nipple. J Am Acad Dermatol 2000;43(5 Pt 1):733-51. 4. Cernea SS, Kihara SM, Sotto MN, Vilela MA. Lupus mastitis. J Am Acad Dermatol 1993;29(2 Pt 2):343-6. 5. Chung HS, Hann SK. Lupus panniculitis treated by a combination therapy of hydroxychloroquine and quinacrine. J Dermatol 1997;24:569-72. 6. Ujiie H, Shimizu T, Ito M, Arita K, Shimizu H. Lupus erythematosus successfully treated with dapsone: review of the literature. Arch Dermatol 2006;142:398-9. doi:10.1016/j.jaad.2008.09.047
Calcinosis cutis associated with amyopathic dermatomyositis: Response to intravenous immunoglobulin To the Editor: We present a 55-year-old female with a history of amyopathic dermatomyositis and progressive calcinosis cutis that improved after intravenous immunoglobulin (IVIG) treatment. The patient was initially seen 13 years earlier, at which time physical examination revealed a heliotrope rash and Gottron papules, along with periungual erythema and telangiectasia. The limbs and trunk had confluent erythematous desquamating plaques. She had never experienced muscle weakness and a neurologic examination was normal. Laboratory and autoimmunity studies were repeatedly normal, as were additional tests (electromyogram, chest radiograph, computed tomography scan, abdominal and pelvic ultrasound, and digestive endoscopy). Histopathologic findings of skin lesions were consistent with dermatomyositis. The patient was treated with prednisone (60 mg/d), leading to side effects that included hypertension and osteoporosis. In an effort to allow the dose of corticosteroids to be lowered, various immunosuppressants were added: hydroxychloroquine (400 mg/d), chloroquine (250 mg/d), methotrexate (7.5 mg/wk), azathioprine (100 mg/d), cyclosporine (200 mg/d), and mofetil mycophenolate (1500 mg/d). Nevertheless, it was not possible to reduce the prednisone dose to less than 30 mg/day, because worsening of the lesions was always observed. Furthermore, in the 2 years after the initial consultation the patient developed progressive dystrophic calcifications on the limbs, leading to ulceration of the skin, joint discomfort, and pain. Diltiazem (120 mg/d) for 3 months was ineffective. Given the lack of response, a decision was made to begin treatment with IVIG (2 g/kg/mo) at a dose of 0.4 g/day for 5 consecutive days, in combination with a reduced dose of prednisone. After five courses of IVIG, the skin lesions of dermatomyositis and the ulcers resolved, while the dermal calcifications became asymptomatic. The reduction in the calcifications was apparent both clinically and radiologically (Fig 1). After 5 years of follow-up, during which time the patient has received maintenance treatment with a yearly course of IVIG without prednisone, the skin condition continues to be well controlled. The only side effect has been the occurrence of headaches after infusions. Calcinosis cutis is a common complication of dermatomyositis, but its cause is unknown. Although various treatments have been described, the results have not been satisfactory; these
J AM ACAD DERMATOL
Letters 1077
VOLUME 60, NUMBER 6
Correspondence to: Yeray Pen ˜ ate, MD, Hospital Universitario Insular de Gran Canaria, Department of Dermatology, Avda. Marı´tima del Sur s/n. 35016, Las Palmas de Gran Canaria, Spain E-mail: [email protected]; [email protected]
Fig 1. Posteroanterior radiograph of the legs showing calcifications around the joints. A, Before treatment with intravenous immunoglobulin. B, After 5 courses of intravenous immunoglobulin.
REFERENCES 1. Boulman N, Slobodin G, Rozenbaum M, Rosner I. Calcinosis in rheumatic diseases. Semin Arthritis Rheum 2005;34:805-12. 2. Chan AY, Li E. Electric shock wave lithotripsy (ESWL) as a pain control measure in dermatomyositis with calcinosis cutis—old method, new discovery. Clin Rheumatol 2005;24:172-3. 3. Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993;329:1993-2000. 4. Schanz S, Ulmer A, Fierlbeck G. Response of dystrophic calcification to intravenous immunoglobulin. Arch Dermatol 2008;144:585-7. doi:10.1016/j.jaad.2008.09.051
treatments include surgical excision, aluminium hydroxide, calcium antagonists, alendronate, colchicine, probenecid, warfarin, and electric shock wave lithotripsy.1,2 IVIG has proven useful in the management of dermatomyositis, as shown by the results of a controlled trial in 15 patients.3 The beneficial effects in terms of resolution of dystrophic calcifications have only been reported previously in a case of CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome.4 The mechanism underlying the effect on dystrophic calcifications is unknown. Calcifications may resolve spontaneously during the course of the disease. However, our patient had received various treatments without response and the disappearance of the calcifications coincided with improvement and stabilization of symptoms following five courses of IVIG. This suggests that in our patient, the use of IVIG was useful for the treatment of dermatomyositis and associated calcifications that were resistant to conventional therapy. Although we report an isolated case, this therapeutic option should be considered and confirmation of its efficacy sought through clinical trials. Yeray Pen ˜ ate, MD, Noemi Guillermo, MD, Priti Melwani, MD, Rosa Martel, MD, Buenaventura Herna´ndez-Machı´n, MD, and Leopoldo Borrego, MD Department of Dermatology, Hospital Universitario Insular de Gran Canaria, Las Palmas de Gran Canaria, Spain Funding sources: None. Conflicts of interest: None declared.
Infliximab treatment for severe psoriasis in a patient with active hepatitis B virus infection To the Editor: Infliximab is a tumor necrosis factore alfa (TNFa)eblocking agent that has been shown to be effective in moderate to severe psoriasis. As a general rule, the presence of chronic hepatitis B virus (HBV) infection is recognized as a major contraindication to the usage of anti-TNF agents. To our knowledge, we present the first documented use of infliximab in a patient with severe psoriasis and active chronic HBV infection. A 36-year-old male was referred to our department with a long-standing psoriasis. He suffered from chronic active HBV infection, with a high viral load ([110,000 DNA copies) and mild liver fibrosis. Over the previous months, he had been administered cyclosporine to treat his psoriasis, and renal impairment occurred. The cyclosporine was discontinued and acitretin treatment was initiated. Although moderate improvement occurred, there were also complications with an increase in liver enzymes. At the time of consultation, he showed symptoms of diffuse psoriasis, with widespread scaling and erythema (Fig 1), which also affected his scalp and nails. The Psoriasis Area and Severity Index (PASI) score was 16.8. The creatinine level was 2.07 mg/dl (normal, 0.1-1.35 mg/dl), reflecting renal impairment. The liver enzyme values were alanine aminotransferase (ALT) 349 U/l (normal, 5-40), aspartate aminotransferase (AST) 161 U/l (normal, 5-40), and gamma-glutamyl transferase 208 U/l (normal, 1-55). Mantoux and booster tests were performed, with negative results; serology for HIV was also negative.
J AM ACAD DERMATOL JUNE 2009
are several case reports in the Japanese literature describing patients with lupus erythematosus panniculitis successfully treated with dapsone, only isolated cases have been reported in the English literature.6 Our case is unusual because of the extensive unilateral involvement of the breast with a carcinoma erysipelatoideseappearing morphology and the presence of telangiectasia on the surface of the skin. In addition, the response to antimalarial drugs was not assessed because she could not have a full course because of an adverse drug reaction. The diagnosis was also difficult because there were no manifestations of lupus elsewhere and her serologic markers were negative. Lupus mastitis may be mistaken for breast carcinoma; therefore, the necessity of a correct diagnosis to avoid useless and harmful surgery is evident. Rosa Ferna´ndez-Torres, MD,a Felipe Sacrista´n, MD,b Jusu´s del Pozo, MD,a Walter Martı´nez, MD,a Luis Albaina, MD,c Marta Mazaira, MD,a and Eduardo Fonseca, MDa Departments of Dermatology,a Pathology,b and General Surgery,c Hospital Juan Canalejo, La Corun ˜ a, Spain Funding sources: None. Conflicts of interest: None declared. Correspondence to: Rosa Ferna´ndez-Torres, MD, Department of Dermatology, Hospital Juan Canalejo, Xubias de Arriba 84, 15006 A Corun ˜ a, Spain E-mail: [email protected] REFERENCES 1. Winkelmann RK. Panniculitis in connective tissue disease. Arch Dermatol 1983;119:336-44. 2. Carducci M, Mussi A, Lisi S, Muscardin L, Solivetti FM. Lupus mastitis: a 2-year history of a single localization of lupus erythematosus mimicking breast carcinoma. J Eur Acad Dermatol Venereol 2005;19:260-2. 3. Whitaker-Worth DL, Carlone V, Susser WS, Phelan N, Grant-Kels JM. Dermatologic diseases of the breast and nipple. J Am Acad Dermatol 2000;43(5 Pt 1):733-51. 4. Cernea SS, Kihara SM, Sotto MN, Vilela MA. Lupus mastitis. J Am Acad Dermatol 1993;29(2 Pt 2):343-6. 5. Chung HS, Hann SK. Lupus panniculitis treated by a combination therapy of hydroxychloroquine and quinacrine. J Dermatol 1997;24:569-72. 6. Ujiie H, Shimizu T, Ito M, Arita K, Shimizu H. Lupus erythematosus successfully treated with dapsone: review of the literature. Arch Dermatol 2006;142:398-9. doi:10.1016/j.jaad.2008.09.047
Calcinosis cutis associated with amyopathic dermatomyositis: Response to intravenous immunoglobulin To the Editor: We present a 55-year-old female with a history of amyopathic dermatomyositis and progressive calcinosis cutis that improved after intravenous immunoglobulin (IVIG) treatment. The patient was initially seen 13 years earlier, at which time physical examination revealed a heliotrope rash and Gottron papules, along with periungual erythema and telangiectasia. The limbs and trunk had confluent erythematous desquamating plaques. She had never experienced muscle weakness and a neurologic examination was normal. Laboratory and autoimmunity studies were repeatedly normal, as were additional tests (electromyogram, chest radiograph, computed tomography scan, abdominal and pelvic ultrasound, and digestive endoscopy). Histopathologic findings of skin lesions were consistent with dermatomyositis. The patient was treated with prednisone (60 mg/d), leading to side effects that included hypertension and osteoporosis. In an effort to allow the dose of corticosteroids to be lowered, various immunosuppressants were added: hydroxychloroquine (400 mg/d), chloroquine (250 mg/d), methotrexate (7.5 mg/wk), azathioprine (100 mg/d), cyclosporine (200 mg/d), and mofetil mycophenolate (1500 mg/d). Nevertheless, it was not possible to reduce the prednisone dose to less than 30 mg/day, because worsening of the lesions was always observed. Furthermore, in the 2 years after the initial consultation the patient developed progressive dystrophic calcifications on the limbs, leading to ulceration of the skin, joint discomfort, and pain. Diltiazem (120 mg/d) for 3 months was ineffective. Given the lack of response, a decision was made to begin treatment with IVIG (2 g/kg/mo) at a dose of 0.4 g/day for 5 consecutive days, in combination with a reduced dose of prednisone. After five courses of IVIG, the skin lesions of dermatomyositis and the ulcers resolved, while the dermal calcifications became asymptomatic. The reduction in the calcifications was apparent both clinically and radiologically (Fig 1). After 5 years of follow-up, during which time the patient has received maintenance treatment with a yearly course of IVIG without prednisone, the skin condition continues to be well controlled. The only side effect has been the occurrence of headaches after infusions. Calcinosis cutis is a common complication of dermatomyositis, but its cause is unknown. Although various treatments have been described, the results have not been satisfactory; these
J AM ACAD DERMATOL
Letters 1077
VOLUME 60, NUMBER 6
Correspondence to: Yeray Pen ˜ ate, MD, Hospital Universitario Insular de Gran Canaria, Department of Dermatology, Avda. Marı´tima del Sur s/n. 35016, Las Palmas de Gran Canaria, Spain E-mail: [email protected]; [email protected]
Fig 1. Posteroanterior radiograph of the legs showing calcifications around the joints. A, Before treatment with intravenous immunoglobulin. B, After 5 courses of intravenous immunoglobulin.
REFERENCES 1. Boulman N, Slobodin G, Rozenbaum M, Rosner I. Calcinosis in rheumatic diseases. Semin Arthritis Rheum 2005;34:805-12. 2. Chan AY, Li E. Electric shock wave lithotripsy (ESWL) as a pain control measure in dermatomyositis with calcinosis cutis—old method, new discovery. Clin Rheumatol 2005;24:172-3. 3. Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993;329:1993-2000. 4. Schanz S, Ulmer A, Fierlbeck G. Response of dystrophic calcification to intravenous immunoglobulin. Arch Dermatol 2008;144:585-7. doi:10.1016/j.jaad.2008.09.051
treatments include surgical excision, aluminium hydroxide, calcium antagonists, alendronate, colchicine, probenecid, warfarin, and electric shock wave lithotripsy.1,2 IVIG has proven useful in the management of dermatomyositis, as shown by the results of a controlled trial in 15 patients.3 The beneficial effects in terms of resolution of dystrophic calcifications have only been reported previously in a case of CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome.4 The mechanism underlying the effect on dystrophic calcifications is unknown. Calcifications may resolve spontaneously during the course of the disease. However, our patient had received various treatments without response and the disappearance of the calcifications coincided with improvement and stabilization of symptoms following five courses of IVIG. This suggests that in our patient, the use of IVIG was useful for the treatment of dermatomyositis and associated calcifications that were resistant to conventional therapy. Although we report an isolated case, this therapeutic option should be considered and confirmation of its efficacy sought through clinical trials. Yeray Pen ˜ ate, MD, Noemi Guillermo, MD, Priti Melwani, MD, Rosa Martel, MD, Buenaventura Herna´ndez-Machı´n, MD, and Leopoldo Borrego, MD Department of Dermatology, Hospital Universitario Insular de Gran Canaria, Las Palmas de Gran Canaria, Spain Funding sources: None. Conflicts of interest: None declared.
Infliximab treatment for severe psoriasis in a patient with active hepatitis B virus infection To the Editor: Infliximab is a tumor necrosis factore alfa (TNFa)eblocking agent that has been shown to be effective in moderate to severe psoriasis. As a general rule, the presence of chronic hepatitis B virus (HBV) infection is recognized as a major contraindication to the usage of anti-TNF agents. To our knowledge, we present the first documented use of infliximab in a patient with severe psoriasis and active chronic HBV infection. A 36-year-old male was referred to our department with a long-standing psoriasis. He suffered from chronic active HBV infection, with a high viral load ([110,000 DNA copies) and mild liver fibrosis. Over the previous months, he had been administered cyclosporine to treat his psoriasis, and renal impairment occurred. The cyclosporine was discontinued and acitretin treatment was initiated. Although moderate improvement occurred, there were also complications with an increase in liver enzymes. At the time of consultation, he showed symptoms of diffuse psoriasis, with widespread scaling and erythema (Fig 1), which also affected his scalp and nails. The Psoriasis Area and Severity Index (PASI) score was 16.8. The creatinine level was 2.07 mg/dl (normal, 0.1-1.35 mg/dl), reflecting renal impairment. The liver enzyme values were alanine aminotransferase (ALT) 349 U/l (normal, 5-40), aspartate aminotransferase (AST) 161 U/l (normal, 5-40), and gamma-glutamyl transferase 208 U/l (normal, 1-55). Mantoux and booster tests were performed, with negative results; serology for HIV was also negative.