Mucocutaneous Presentation of Systemic Lupus Erythematosus
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10-year-old girl with Graves disease presented with a 5day history of malar rash. She was being treated with methimazole and levothyroxine daily. The rash consisted of erythematous patches with a scaly surface on her malar area that did not cross the nasal bridge (Figure 1). Small superficial erythematous painless ulcers with well defined borders were noted on her hard palate (Figure 2). After 3 days of followup, bilateral cervical soft lymphadenopathies and nonerosive hand arthritis were observed. No fever and no other skin lesions were detected. Remaining clinical examination was unremarkable. She had leukopenia, hypocomplementemia, elevated antinuclear antibody, and anti-double-stranded DNA titers. Systemic lupus erythematosus (SLE) was diagnosed based on the clinical and laboratory features of the patient and treatment was started with intravenous prednisone. The skin and mucosal lesions completely resolved and hand arthritis improved within 3 weeks. SLE is a complicated and serious autoimmune disease that involves multiple organs. In general, among children, onset is abrupt with higher rates of organ involvement and more aggressive clinical course than in adults.1 To date, the only clinical features consistently associated with poor prognosis are renal and neurologic involvement.2 The most common manifestations of SLE at onset are arthritis, malar “butterfly rash,” and constitutional symptoms. The cutaneous lesions tend to be transient, follow sun exposure, and resolve without scarring. The oral ulcers on the hard palate are the most frequent mucosal sign of SLE.3 However, the prevalence of oral and cutaneous involve-
Figure 2. Small superficial erythematous ulcers with well defined borders on the hard palate.
ment in patients with SLE is debatable.4 These clinical data prompt us to examine the oral cavity as well as the skin to detect any signs that might serve as a red flag in patients with suspicious systemic lupus. n Azael Freites-Martinez, MD Marta Aguado-Lobo, MD Angelica Calder on-Komaromy, MD Nieves Puente de Pablo, MD Marta Utrera Busquets, MD Jesus Borbujo, MD, PhD Dermatology Service University Hospital of Fuenlabrada Madrid, Spain
References
Figure 1. Erythematous patches with a scaly surface on the malar area that do not cross the nasal bridge.
1. Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am 2012;59:345-64. 2. Bennett M, Hermine I. Brunner biomarkers and updates on pediatrics lupus nephritis. Rheum Dis Clin North Am 2013;39:833-53. 3. Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J Pediatr 2008;152: 550-6. 4. Lourenc¸o SV, de Carvalho FR, Boggio P, Sotto MN, Vilela MA, Rivitti EA, et al. Lupus erythematosus: clinical and histopathologic study of oral manifestations and immunohistochemical profile of the inflammatory infiltrate. J Cutan Pathol 2007;34:558-64.
J Pediatr 2014;165:631. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.05.048
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A
10-year-old girl with Graves disease presented with a 5day history of malar rash. She was being treated with methimazole and levothyroxine daily. The rash consisted of erythematous patches with a scaly surface on her malar area that did not cross the nasal bridge (Figure 1). Small superficial erythematous painless ulcers with well defined borders were noted on her hard palate (Figure 2). After 3 days of followup, bilateral cervical soft lymphadenopathies and nonerosive hand arthritis were observed. No fever and no other skin lesions were detected. Remaining clinical examination was unremarkable. She had leukopenia, hypocomplementemia, elevated antinuclear antibody, and anti-double-stranded DNA titers. Systemic lupus erythematosus (SLE) was diagnosed based on the clinical and laboratory features of the patient and treatment was started with intravenous prednisone. The skin and mucosal lesions completely resolved and hand arthritis improved within 3 weeks. SLE is a complicated and serious autoimmune disease that involves multiple organs. In general, among children, onset is abrupt with higher rates of organ involvement and more aggressive clinical course than in adults.1 To date, the only clinical features consistently associated with poor prognosis are renal and neurologic involvement.2 The most common manifestations of SLE at onset are arthritis, malar “butterfly rash,” and constitutional symptoms. The cutaneous lesions tend to be transient, follow sun exposure, and resolve without scarring. The oral ulcers on the hard palate are the most frequent mucosal sign of SLE.3 However, the prevalence of oral and cutaneous involve-
Figure 2. Small superficial erythematous ulcers with well defined borders on the hard palate.
ment in patients with SLE is debatable.4 These clinical data prompt us to examine the oral cavity as well as the skin to detect any signs that might serve as a red flag in patients with suspicious systemic lupus. n Azael Freites-Martinez, MD Marta Aguado-Lobo, MD Angelica Calder on-Komaromy, MD Nieves Puente de Pablo, MD Marta Utrera Busquets, MD Jesus Borbujo, MD, PhD Dermatology Service University Hospital of Fuenlabrada Madrid, Spain
References
Figure 1. Erythematous patches with a scaly surface on the malar area that do not cross the nasal bridge.
1. Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am 2012;59:345-64. 2. Bennett M, Hermine I. Brunner biomarkers and updates on pediatrics lupus nephritis. Rheum Dis Clin North Am 2013;39:833-53. 3. Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J Pediatr 2008;152: 550-6. 4. Lourenc¸o SV, de Carvalho FR, Boggio P, Sotto MN, Vilela MA, Rivitti EA, et al. Lupus erythematosus: clinical and histopathologic study of oral manifestations and immunohistochemical profile of the inflammatory infiltrate. J Cutan Pathol 2007;34:558-64.
J Pediatr 2014;165:631. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.05.048
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