Correspondence
Bydgoszcz 85-094, Poland E-mail: [email protected] Conflicts of interest: None. References 1 Lazarczyk M, Wozniak K, Ischi N, et al. Coexistence of psoriasis and pemphigoid – only a coincidence? Int J Mol Med 2006; 18: 619–623. 2 Barnadas MA, Gilaberte M, Pujol R, et al. Bullous pemphigoid in a patient with psoriasis during the course of PUVA therapy: study by ELISA test. Int J Dermatol 2006; 45: 1089–1092. 3 Corey K, Levin NA, Hure M, et al. Eruption of bullae within psoriatic plaques: a rare adverse effect of narrowband ultraviolet B (NB-UVB) phototherapy. Dermatol Online J 2012; 18: 3. 4 Stausbol-Gron B, Deleuran M, Sommer Hansen E, et al. Development of bullous pemphigoid during treatment of psoriasis with adalimumab. Clin Exp Dermatol 2009; 34: e285–e286.
Successful treatment of Schnitzler syndrome with cyclosporine
Editor, Schnitzler syndrome is a rare autoinflammatory disorder defined by urticarial rash and monoclonal gammopathy.1 Further clinical symptoms include episodes of fever, arthralgia or arthritis, lymphadenopathy, and bone and muscle pain.1 We describe a 51-year-old woman with Schnitzler syndrome. A 51-year-old Japanese woman had suffered from a skin rash, myalgia, conjunctivitis, and high fever for six months. Following the initial attack, she had experienced similar episodes approximately every four weeks; each episode lasted one week, and symptoms resolved completely between episodes. Widespread erythematous edematous plaques with severe pruritus were apparent on the subject’s trunk and extremities during the febrile attacks (Fig. 1a). A biopsy taken from an erythematous area on her right arm revealed a moderate perivascular infiltration of lymphocytes, neutrophils, and histiocytes in the dermis (Fig. 1b). No deposit of monoclonal immunoglobulin M (IgM) was seen in the lesional skin. Laboratory tests demonstrated an elevated erythrocyte sedimentation rate (ESR) of 128 mm/h, C-reactive protein at 12.1 mg/dl (normal range: 0–0.6 mg/dl), elevated ferritin at 348.6 ng/ ml (normal range: 6.2–138 ng/ml), elevated IgM at 648 mg/dl (normal range: 35–220 mg/ml), normal IgG at 999 mg/dl (normal range: 920–1800 mg/ml), and normal IgA at 281 mg/dl (normal range: 110–450 mg/ml). Serum protein electrophoresis demonstrated an M-bow, which was shown to be a monoclonal IgM j-type. No abnormal ª 2014 The International Society of Dermatology
5 Chen YJ, Wu CY, Lin MW, et al. Comorbidity profiles among patients with bullous pemphigoid: a nationwide population-based study. Br J Dermatol 2011; 165: 593– 599. 6 Tsai TF, Wang TS, Hung ST, et al. Epidemiology and comorbidities of psoriasis patients in a national database in Taiwan. J Dermatol Sci 2011; 63: 40–46. 7 Pasic A, Ljubojevic S, Lipozencic J, et al. Coexistence of psoriasis vulgaris, bullous pemphigoid and vitiligo: a case report. J Eur Acad Dermatol Venereol 2002; 16: 426–427. 8 Pender MP. CD8+ T-cell deficiency, Epstein-Barr virus infection, vitamin D deficiency, and steps to autoimmunity: a unifying hypothesis. Autoimmune Dis 2012; 2012: 189096. 9 Fierabracci A. Recent insights into the role and molecular mechanisms of the autoimmune regulator (AIRE) gene in autoimmunity. Autoimmun Rev 2011; 10: 137–143. 10 Buschman KE, Seraly M, Thong HY, et al. A predominant IgG4subclass may be responsible for falsenegative direct immunofluorescence in bullous pemphigoid. J Cutan Pathol 2006; 29: 282–286.
findings were revealed in a bone marrow biopsy. Polymerase chain reaction sequencing of the TNFRSF1A gene was performed but did not show any mutations in exons 1–10. The combination of findings led to a diagnosis of Schnitzler syndrome. The patient’s febrile attacks and skin rash were alleviated by the administration of oral prednisolone at 30 mg/d. However, recurrence was observed when prednisolone was decreased to 15 mg/d. Therefore, oral cyclosporine at 3.0 mg/kg/d was started. Strikingly, cyclosporine successfully suppressed the occurrence of the febrile attacks, skin rash, arthralgia, and muscle pain. However, after two years of this therapy, the patient’s blood pressure rose and the oral administration of cyclosporine was discontinued. At this time, oral administration of colchicine at 1 mg/d was started and was effective in resolving all of her symptoms. Initially, tumor necrosis factor receptor-associated periodic syndrome (TRAPS) was suspected. However, the patient had no mutations in exons 1–10 of the TNFRSF1A gene. Schnitzler described cases with nonpruritic urticaria, intermittent fever of unknown origin, arthralgia or bone pain, increased ESR, and IgM paraproteinemia as Schnitzler syndrome.2 It has since been reported that the absence of pruritus is not necessary for the diagnosis of Schnitzler syndrome because the majority of patients report an intensified itching during the course of the disease.3 The histopathological findings in the present patient showed an infiltration of mononuclear cells around the blood vessels in the upper dermis. Generally, neutrophilic urticarial dermatosis is the most common histopathological pattern in Schnitzler syndrome. International Journal of Dermatology 2014, 53, e347–e366
e361
e362
Correspondence
lymphoma has developed in the present patient over the past eight years.
(a)
Kimiko Nakajima, MD, PhD Masato Takahashi, MD Mayuko Yamamoto, MD Aya Takahashi, MD Shigetoshi Sano, MD, PhD Department of Dermatology Kochi Medical School Kochi University Kochi Japan E-mail: [email protected] Hajime Kodama, MD, PhD Skin Clinic Center San-ai Hospital Kochi Japan
(b) Figure 1 (a) Clinical examination shows widespread erythematous edematous plaques on the trunk and extremities in a 51-year-old woman. (b) Histopathological examination of erythema reveals moderate perivascular infiltration of lymphocytes, neutrophils and histiocytes in the dermis. Leukocytoclastic vasculitis and infiltration of eosinophils are not seen. (Hematoxylin and eosin stain; original magnification 9200)
However, a recent report stated that infiltrations of mononuclear cells and/or histiocytes are predominant in some cases of Schnitzler syndrome.1 Recently, a few promising biological therapies, including the interleukin-1 (IL-1) receptor antagonist anakinra, have emerged.1,3–7 By contrast, the efficacy of cyclosporine for treating Schnitzler syndrome is regarded as only partial or temporary. However, in the present patient, symptoms were controlled by cyclosporine. Various patients with Schnitzler syndrome have been treated with cyclosporine or colchicine.4,8,9 The prognosis in Schnitzler syndrome depends on whether the patient develops a lymphoproliferative disorder including lymphoma and Waldenstrom macroglobulinemia. No malignancy such as malignant
International Journal of Dermatology 2014, 53, e347–e366
Kazuhiko Arima, MD, PhD Hiroaki Ida, MD, PhD Department of Immunology and Rheumatology Unit of Translational Medicine Nagasaki University Graduate School of Biomedical Sciences Nagasaki Japan
References 1 Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome. The Mayo Clinic experience (1972–2011). J Am Acad Dermatol 2012; 67: 1289–1295. 2 Schnitzler L. Lesions urticariennes chroniques permanents (erytheme petaloide?). Cas cliniques No. 46B. J Dermatol Angers Abstract 46, 1972. 3 De Koning HD, Bodar EJ, van der Meer JW, et al.; Schnitzler Syndrome Study Group. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum 2007; 37: 137–148. 4 Tinazzi E, Pucccetti A, Patuzzo G, et al. Schnitzler syndrome, an autoimmune–autoinflammatory syndrome: report of two new cases and review of the literature. Autoimmun Rev 2011; 10: 404–409. 5 Schuster C, Kr€ anke B, Aberer E, et al. Schnitzler syndrome: response to anakinra in two cases and a review of the literature. Int J Dermatol 2009; 48: 1190– 1194. 6 de Koning KH, Schalkwijk J, van der Meer JW, et al. Successful canakinumab treatment identifies IL-1b as a
ª 2014 The International Society of Dermatology
Correspondence
pivotal mediator in Schnitzler syndrome. J Allergy Clin Immunol 2011; 128: 1352–1354. 7 Stzurz P, Adam Z, Hajek R, et al. Successful anakinra therapy in two patients with Schnizler syndrome. Onkologie 2011; 34: 265–268. 8 Carbone J, Paravisini A, Sarmento E, et al. Partial response to cyclosporine in a patient with Schnizler
syndrome. Allergol Immunopathol (Madr) 2007; 35: 71–73. ~ez A, S 9 Pascual-L opez M, Hern andez-N un anchez-Perez J, et al. Schnizler syndrome with monoclonal IgG kappa gammopathy: good response to cyclosporine. J Eur Acad Dermatol Venereol 2002; 16: 267–270.
Association between estrogen receptor-a gene polymorphisms and dermatomyositis in Bulgarian patients
the World Medical Association (Declaration of Helsinki) for experiments involving humans. The study was approved by the local ethics committee at the Medical University of Sofia. The PvuII and XbaI polymorphisms were analyzed by polymerase chain reaction restriction fragment length polymorphism (PCR–RFLP) as previously described.6 The ESR1 XbaI x/X and PvuII p/P polymorphisms appeared in linkage dysequilibrium (D′ = 0.84). No statistically significant difference in allele and genotype frequencies was found between patients and controls. Neither did stratification by gender lead to any statistically significant differences. The predominant genotype in both groups was XxPp, followed by xxpp and XXPP; there were no statistically significant differences in their distribution. Thus, in the present study, we observed an association between the X allele and the XX + Xx genotypes (X, P = 0.03, odds ratio [OR] 2.9, 95% confidence interval [CI] 1.1–7.9; XX + Xx, P = 0.025, OR 6.1, 95% CI 1.2–30.1) and photosensitivity in DM patients. The P allele (P = 0.09, OR 2.2, 95% CI 0.8– 6.0) and PP + Pp genotypes (P = 0.057, OR 4.7, 95%
Editor, The etiology of dermatomyositis (DM) is not clearly understood, but the existence of a strong genetic predisposition has been recognized. Environmental and hormonal factors are known to contribute towards the expression of the disease. Since DM affects women three times more often than men, the estrogens have been proposed as obvious candidates to explain the sexual dimorphism.1 Estrogen acts through two nuclear receptors: estrogen receptor-a (ER-a), coded by ESR1, and estrogen receptorb (ER-b), coded by ESR2. The PvuII P (C) and XbaI X (G) variants were found to lead to enhanced ER-a activity2,3 and elevated serum estradiol production.4,5 Until now, there has been no study of the association of ESR1 XbaI x/X and PvuII p/P polymorphisms in DM. We investigated the frequencies of the XbaI x/X and PvuII polymorphisms in 34 patients with DM and 69 healthy control subjects. The work described in this letter was carried out in accordance with the Code of Ethics of
Table 1 Analysis of frequencies of the ESR1 XbaI x/X and PvuII p/P polymorphisms and clinical parameters of
dermatomyositis Genotype Dermatomyositis Clinical parameters Muscle weakness Elevated muscle enzymes EMG findings Muscle biopsy findings Cutaneous disease Photosensitivity Immunological disease (Antisynthetase and anti-myositis specific antibodies)
XbaI xx (n = 11)
Xx (n = 16)
XX (n = 7)
P-value
PvuII pp (n = 10)
Pp (n = 18)
PP (n = 6)
P-value
9 (81.8%)
12 (75.0%)
6 (85.7%)
0.55
9 (90.0%)
13 (72.2%)
5 (83.3%)
0.3
8 (72.7%)
7 (43.8%)
4 (57.1%)
0.16
8 (80.0%)
7 (38.9%)
4 (66.7%)
0.07
6 (54.6%) 0
7 (43.8%) 0
6 (85.7%) 0
0.085 NS
6 (60.0%) 0
9 (50.0%) 0
4 (66.7%) 0
0.33 NS
7 (63.6%)
13 (81.3%)
6 (85.7%)
0.2
7 (70.0%)
14 (77.8%)
5 (83.3%)
0.43
3 (27.3%) 4 (36.4%)
11 (68.8%) 4 (25.0%)
5 (71.4%) 0
0.025 NS
3 (30.0%) 4 (40.0%)
12 (66.7%) 3 (16.7%)
4 (66.7%) 1 (16.7%)
0.056 NS
NS, not significant; EMG, electromyography. ª 2014 The International Society of Dermatology
International Journal of Dermatology 2014, 53, e347–e366
e363
Bydgoszcz 85-094, Poland E-mail: [email protected] Conflicts of interest: None. References 1 Lazarczyk M, Wozniak K, Ischi N, et al. Coexistence of psoriasis and pemphigoid – only a coincidence? Int J Mol Med 2006; 18: 619–623. 2 Barnadas MA, Gilaberte M, Pujol R, et al. Bullous pemphigoid in a patient with psoriasis during the course of PUVA therapy: study by ELISA test. Int J Dermatol 2006; 45: 1089–1092. 3 Corey K, Levin NA, Hure M, et al. Eruption of bullae within psoriatic plaques: a rare adverse effect of narrowband ultraviolet B (NB-UVB) phototherapy. Dermatol Online J 2012; 18: 3. 4 Stausbol-Gron B, Deleuran M, Sommer Hansen E, et al. Development of bullous pemphigoid during treatment of psoriasis with adalimumab. Clin Exp Dermatol 2009; 34: e285–e286.
Successful treatment of Schnitzler syndrome with cyclosporine
Editor, Schnitzler syndrome is a rare autoinflammatory disorder defined by urticarial rash and monoclonal gammopathy.1 Further clinical symptoms include episodes of fever, arthralgia or arthritis, lymphadenopathy, and bone and muscle pain.1 We describe a 51-year-old woman with Schnitzler syndrome. A 51-year-old Japanese woman had suffered from a skin rash, myalgia, conjunctivitis, and high fever for six months. Following the initial attack, she had experienced similar episodes approximately every four weeks; each episode lasted one week, and symptoms resolved completely between episodes. Widespread erythematous edematous plaques with severe pruritus were apparent on the subject’s trunk and extremities during the febrile attacks (Fig. 1a). A biopsy taken from an erythematous area on her right arm revealed a moderate perivascular infiltration of lymphocytes, neutrophils, and histiocytes in the dermis (Fig. 1b). No deposit of monoclonal immunoglobulin M (IgM) was seen in the lesional skin. Laboratory tests demonstrated an elevated erythrocyte sedimentation rate (ESR) of 128 mm/h, C-reactive protein at 12.1 mg/dl (normal range: 0–0.6 mg/dl), elevated ferritin at 348.6 ng/ ml (normal range: 6.2–138 ng/ml), elevated IgM at 648 mg/dl (normal range: 35–220 mg/ml), normal IgG at 999 mg/dl (normal range: 920–1800 mg/ml), and normal IgA at 281 mg/dl (normal range: 110–450 mg/ml). Serum protein electrophoresis demonstrated an M-bow, which was shown to be a monoclonal IgM j-type. No abnormal ª 2014 The International Society of Dermatology
5 Chen YJ, Wu CY, Lin MW, et al. Comorbidity profiles among patients with bullous pemphigoid: a nationwide population-based study. Br J Dermatol 2011; 165: 593– 599. 6 Tsai TF, Wang TS, Hung ST, et al. Epidemiology and comorbidities of psoriasis patients in a national database in Taiwan. J Dermatol Sci 2011; 63: 40–46. 7 Pasic A, Ljubojevic S, Lipozencic J, et al. Coexistence of psoriasis vulgaris, bullous pemphigoid and vitiligo: a case report. J Eur Acad Dermatol Venereol 2002; 16: 426–427. 8 Pender MP. CD8+ T-cell deficiency, Epstein-Barr virus infection, vitamin D deficiency, and steps to autoimmunity: a unifying hypothesis. Autoimmune Dis 2012; 2012: 189096. 9 Fierabracci A. Recent insights into the role and molecular mechanisms of the autoimmune regulator (AIRE) gene in autoimmunity. Autoimmun Rev 2011; 10: 137–143. 10 Buschman KE, Seraly M, Thong HY, et al. A predominant IgG4subclass may be responsible for falsenegative direct immunofluorescence in bullous pemphigoid. J Cutan Pathol 2006; 29: 282–286.
findings were revealed in a bone marrow biopsy. Polymerase chain reaction sequencing of the TNFRSF1A gene was performed but did not show any mutations in exons 1–10. The combination of findings led to a diagnosis of Schnitzler syndrome. The patient’s febrile attacks and skin rash were alleviated by the administration of oral prednisolone at 30 mg/d. However, recurrence was observed when prednisolone was decreased to 15 mg/d. Therefore, oral cyclosporine at 3.0 mg/kg/d was started. Strikingly, cyclosporine successfully suppressed the occurrence of the febrile attacks, skin rash, arthralgia, and muscle pain. However, after two years of this therapy, the patient’s blood pressure rose and the oral administration of cyclosporine was discontinued. At this time, oral administration of colchicine at 1 mg/d was started and was effective in resolving all of her symptoms. Initially, tumor necrosis factor receptor-associated periodic syndrome (TRAPS) was suspected. However, the patient had no mutations in exons 1–10 of the TNFRSF1A gene. Schnitzler described cases with nonpruritic urticaria, intermittent fever of unknown origin, arthralgia or bone pain, increased ESR, and IgM paraproteinemia as Schnitzler syndrome.2 It has since been reported that the absence of pruritus is not necessary for the diagnosis of Schnitzler syndrome because the majority of patients report an intensified itching during the course of the disease.3 The histopathological findings in the present patient showed an infiltration of mononuclear cells around the blood vessels in the upper dermis. Generally, neutrophilic urticarial dermatosis is the most common histopathological pattern in Schnitzler syndrome. International Journal of Dermatology 2014, 53, e347–e366
e361
e362
Correspondence
lymphoma has developed in the present patient over the past eight years.
(a)
Kimiko Nakajima, MD, PhD Masato Takahashi, MD Mayuko Yamamoto, MD Aya Takahashi, MD Shigetoshi Sano, MD, PhD Department of Dermatology Kochi Medical School Kochi University Kochi Japan E-mail: [email protected] Hajime Kodama, MD, PhD Skin Clinic Center San-ai Hospital Kochi Japan
(b) Figure 1 (a) Clinical examination shows widespread erythematous edematous plaques on the trunk and extremities in a 51-year-old woman. (b) Histopathological examination of erythema reveals moderate perivascular infiltration of lymphocytes, neutrophils and histiocytes in the dermis. Leukocytoclastic vasculitis and infiltration of eosinophils are not seen. (Hematoxylin and eosin stain; original magnification 9200)
However, a recent report stated that infiltrations of mononuclear cells and/or histiocytes are predominant in some cases of Schnitzler syndrome.1 Recently, a few promising biological therapies, including the interleukin-1 (IL-1) receptor antagonist anakinra, have emerged.1,3–7 By contrast, the efficacy of cyclosporine for treating Schnitzler syndrome is regarded as only partial or temporary. However, in the present patient, symptoms were controlled by cyclosporine. Various patients with Schnitzler syndrome have been treated with cyclosporine or colchicine.4,8,9 The prognosis in Schnitzler syndrome depends on whether the patient develops a lymphoproliferative disorder including lymphoma and Waldenstrom macroglobulinemia. No malignancy such as malignant
International Journal of Dermatology 2014, 53, e347–e366
Kazuhiko Arima, MD, PhD Hiroaki Ida, MD, PhD Department of Immunology and Rheumatology Unit of Translational Medicine Nagasaki University Graduate School of Biomedical Sciences Nagasaki Japan
References 1 Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome. The Mayo Clinic experience (1972–2011). J Am Acad Dermatol 2012; 67: 1289–1295. 2 Schnitzler L. Lesions urticariennes chroniques permanents (erytheme petaloide?). Cas cliniques No. 46B. J Dermatol Angers Abstract 46, 1972. 3 De Koning HD, Bodar EJ, van der Meer JW, et al.; Schnitzler Syndrome Study Group. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum 2007; 37: 137–148. 4 Tinazzi E, Pucccetti A, Patuzzo G, et al. Schnitzler syndrome, an autoimmune–autoinflammatory syndrome: report of two new cases and review of the literature. Autoimmun Rev 2011; 10: 404–409. 5 Schuster C, Kr€ anke B, Aberer E, et al. Schnitzler syndrome: response to anakinra in two cases and a review of the literature. Int J Dermatol 2009; 48: 1190– 1194. 6 de Koning KH, Schalkwijk J, van der Meer JW, et al. Successful canakinumab treatment identifies IL-1b as a
ª 2014 The International Society of Dermatology
Correspondence
pivotal mediator in Schnitzler syndrome. J Allergy Clin Immunol 2011; 128: 1352–1354. 7 Stzurz P, Adam Z, Hajek R, et al. Successful anakinra therapy in two patients with Schnizler syndrome. Onkologie 2011; 34: 265–268. 8 Carbone J, Paravisini A, Sarmento E, et al. Partial response to cyclosporine in a patient with Schnizler
syndrome. Allergol Immunopathol (Madr) 2007; 35: 71–73. ~ez A, S 9 Pascual-L opez M, Hern andez-N un anchez-Perez J, et al. Schnizler syndrome with monoclonal IgG kappa gammopathy: good response to cyclosporine. J Eur Acad Dermatol Venereol 2002; 16: 267–270.
Association between estrogen receptor-a gene polymorphisms and dermatomyositis in Bulgarian patients
the World Medical Association (Declaration of Helsinki) for experiments involving humans. The study was approved by the local ethics committee at the Medical University of Sofia. The PvuII and XbaI polymorphisms were analyzed by polymerase chain reaction restriction fragment length polymorphism (PCR–RFLP) as previously described.6 The ESR1 XbaI x/X and PvuII p/P polymorphisms appeared in linkage dysequilibrium (D′ = 0.84). No statistically significant difference in allele and genotype frequencies was found between patients and controls. Neither did stratification by gender lead to any statistically significant differences. The predominant genotype in both groups was XxPp, followed by xxpp and XXPP; there were no statistically significant differences in their distribution. Thus, in the present study, we observed an association between the X allele and the XX + Xx genotypes (X, P = 0.03, odds ratio [OR] 2.9, 95% confidence interval [CI] 1.1–7.9; XX + Xx, P = 0.025, OR 6.1, 95% CI 1.2–30.1) and photosensitivity in DM patients. The P allele (P = 0.09, OR 2.2, 95% CI 0.8– 6.0) and PP + Pp genotypes (P = 0.057, OR 4.7, 95%
Editor, The etiology of dermatomyositis (DM) is not clearly understood, but the existence of a strong genetic predisposition has been recognized. Environmental and hormonal factors are known to contribute towards the expression of the disease. Since DM affects women three times more often than men, the estrogens have been proposed as obvious candidates to explain the sexual dimorphism.1 Estrogen acts through two nuclear receptors: estrogen receptor-a (ER-a), coded by ESR1, and estrogen receptorb (ER-b), coded by ESR2. The PvuII P (C) and XbaI X (G) variants were found to lead to enhanced ER-a activity2,3 and elevated serum estradiol production.4,5 Until now, there has been no study of the association of ESR1 XbaI x/X and PvuII p/P polymorphisms in DM. We investigated the frequencies of the XbaI x/X and PvuII polymorphisms in 34 patients with DM and 69 healthy control subjects. The work described in this letter was carried out in accordance with the Code of Ethics of
Table 1 Analysis of frequencies of the ESR1 XbaI x/X and PvuII p/P polymorphisms and clinical parameters of
dermatomyositis Genotype Dermatomyositis Clinical parameters Muscle weakness Elevated muscle enzymes EMG findings Muscle biopsy findings Cutaneous disease Photosensitivity Immunological disease (Antisynthetase and anti-myositis specific antibodies)
XbaI xx (n = 11)
Xx (n = 16)
XX (n = 7)
P-value
PvuII pp (n = 10)
Pp (n = 18)
PP (n = 6)
P-value
9 (81.8%)
12 (75.0%)
6 (85.7%)
0.55
9 (90.0%)
13 (72.2%)
5 (83.3%)
0.3
8 (72.7%)
7 (43.8%)
4 (57.1%)
0.16
8 (80.0%)
7 (38.9%)
4 (66.7%)
0.07
6 (54.6%) 0
7 (43.8%) 0
6 (85.7%) 0
0.085 NS
6 (60.0%) 0
9 (50.0%) 0
4 (66.7%) 0
0.33 NS
7 (63.6%)
13 (81.3%)
6 (85.7%)
0.2
7 (70.0%)
14 (77.8%)
5 (83.3%)
0.43
3 (27.3%) 4 (36.4%)
11 (68.8%) 4 (25.0%)
5 (71.4%) 0
0.025 NS
3 (30.0%) 4 (40.0%)
12 (66.7%) 3 (16.7%)
4 (66.7%) 1 (16.7%)
0.056 NS
NS, not significant; EMG, electromyography. ª 2014 The International Society of Dermatology
International Journal of Dermatology 2014, 53, e347–e366
e363
