1326
DE BOYSSON ET AL
of the central nervous system: experience of a Victorian tertiaryreferral hospital. Intern Med J 2013;43:685–92. 29. Volcy M, Toro ME, Uribe CS, Toro G. Primary angiitis of the central nervous system: report of five biopsy-confirmed cases from Colombia. J Neurol Sci 2004;227:85–9. 30. Yin Z, Li X, Fang Y, Luo B, Zhang A. Primary angiitis of the central nervous system: report of eight cases from southern China. Eur J Neurol 2009;16:63–9. 31. El M’Kaddem B, Clapuyt P, de Bruycker JJ, Lauwerys B, Raftopoulos C, Godfraind C, et al. Primary cerebral arteritis in a young girl: an unusual cause of acute recurrent neurological deterioration. Eur J Paediatr Neurol 2012;16:196–9.
32. Hutchinson C, Elbers J, Halliday W, Branson H, Laughlin S, Armstrong D, et al. Treatment of small vessel primary CNS vasculitis in children: an open-label cohort study. Lancet Neurol 2010;9:1078–84. 33. Concato J, Peduzzi P, Holford TR, Feinstein AR. Importance of events per independent variable in proportional hazards analysis. I. Background, goals, and general strategy. J Clin Epidemiol 1995;48:1495–501. 34. Peduzzi P, Concato J, Feinstein AR, Holford TR. Importance of events per independent variable in proportional hazards regression analysis. II. Accuracy and precision of regression estimates. J Clin Epidemiol 1995;48:1503–10.
DOI 10.1002/art.38350
Clinical Images: Diffuse papules and skin tightening
The patient, a 41-year-old woman, presented (current presentation) with a history of progressive skin thickening on the entire body, and chronic fatigue. She had initially presented 4 years previously, with waxy papules on her arms and legs followed by the development of diffuse skin thickening spreading to her entire body including the face (A). At that time, she denied experiencing any symptoms of Raynaud’s phenomenon, and antinuclear antibodies were absent. Skin biopsy at that time revealed superficial perivascular lymphocytic infiltrates with dermal fibrosis, and protein electrophoresis showed IgG monoclonal gammopathy; bone marrow examination results were normal. Her disease progressed without a conclusive diagnosis or treatment, and she was lost to followup. After 4 years, she returned with severe facial papules (B), dermal induration with tight skin turgor, hyperpigmentation, increased erythema, and reduced joint motility, as well as worsening fatigue. She was diagnosed as having scleromyxedema. Scleromyxedema, a primary cutaneous mucinosis, should be considered in patients with diffuse skin thickening, papules, and monoclonal gammopathy. Treatment should be initiated promptly to avoid potential long-term disfigurement. Jin Kyun Park, MD Jeong Seok Lee, MD Yeong Wook Song, MD, PhD Eun Bong Lee, MD, PhD Seoul National University Hospital Seoul, South Korea
DE BOYSSON ET AL
of the central nervous system: experience of a Victorian tertiaryreferral hospital. Intern Med J 2013;43:685–92. 29. Volcy M, Toro ME, Uribe CS, Toro G. Primary angiitis of the central nervous system: report of five biopsy-confirmed cases from Colombia. J Neurol Sci 2004;227:85–9. 30. Yin Z, Li X, Fang Y, Luo B, Zhang A. Primary angiitis of the central nervous system: report of eight cases from southern China. Eur J Neurol 2009;16:63–9. 31. El M’Kaddem B, Clapuyt P, de Bruycker JJ, Lauwerys B, Raftopoulos C, Godfraind C, et al. Primary cerebral arteritis in a young girl: an unusual cause of acute recurrent neurological deterioration. Eur J Paediatr Neurol 2012;16:196–9.
32. Hutchinson C, Elbers J, Halliday W, Branson H, Laughlin S, Armstrong D, et al. Treatment of small vessel primary CNS vasculitis in children: an open-label cohort study. Lancet Neurol 2010;9:1078–84. 33. Concato J, Peduzzi P, Holford TR, Feinstein AR. Importance of events per independent variable in proportional hazards analysis. I. Background, goals, and general strategy. J Clin Epidemiol 1995;48:1495–501. 34. Peduzzi P, Concato J, Feinstein AR, Holford TR. Importance of events per independent variable in proportional hazards regression analysis. II. Accuracy and precision of regression estimates. J Clin Epidemiol 1995;48:1503–10.
DOI 10.1002/art.38350
Clinical Images: Diffuse papules and skin tightening
The patient, a 41-year-old woman, presented (current presentation) with a history of progressive skin thickening on the entire body, and chronic fatigue. She had initially presented 4 years previously, with waxy papules on her arms and legs followed by the development of diffuse skin thickening spreading to her entire body including the face (A). At that time, she denied experiencing any symptoms of Raynaud’s phenomenon, and antinuclear antibodies were absent. Skin biopsy at that time revealed superficial perivascular lymphocytic infiltrates with dermal fibrosis, and protein electrophoresis showed IgG monoclonal gammopathy; bone marrow examination results were normal. Her disease progressed without a conclusive diagnosis or treatment, and she was lost to followup. After 4 years, she returned with severe facial papules (B), dermal induration with tight skin turgor, hyperpigmentation, increased erythema, and reduced joint motility, as well as worsening fatigue. She was diagnosed as having scleromyxedema. Scleromyxedema, a primary cutaneous mucinosis, should be considered in patients with diffuse skin thickening, papules, and monoclonal gammopathy. Treatment should be initiated promptly to avoid potential long-term disfigurement. Jin Kyun Park, MD Jeong Seok Lee, MD Yeong Wook Song, MD, PhD Eun Bong Lee, MD, PhD Seoul National University Hospital Seoul, South Korea
