Clinical Review & Education
JAMA Clinical Challenge
Painful Rash in a Patient With Bronchiectasis Sruthi Renati, BS; Adam Raff, MD, PhD; Susan Burgin, MD
Figure. Left, Axial computed tomography scan of the chest revealing mild bronchiectasis and ground glass inflammation. Right, Purpuric plaque on the right medial ankle and dorsal foot, with central vesicles.
A 71-year-old man with a history of bronchiectasis presents with a painful rash and swelling of his right foot for 1 day. He has had no fevers, joint pain, recent trauma, or new exposures to the area. He was diagnosed with bronchiectasis of unknown etiology 6 years ago and notes a worsenQuiz at jama.com ing of his cough and dyspnea over the past few months. A recent computed tomography scan of the chest revealed mild bronchiectasis and ground glass inflammation (Figure, left), and recent pulmonary function tests showed combined obstructive and restrictive defects. His medications include albuterol inhaler, fluticasonesalmeterol inhaler, guaifenesin, and loratadine. On examination, the patient’s vital signs are normal. He has a purpuric plaque on the right medial ankle and dorsal foot, with central vesicles (Figure, right). The rash is tender to palpation, and there is associated nonpitting edema of the right foot and ankle. Laboratory values are notable for a leukocyte count of 15 700 cells/μL, with 38% eosinophils (absolute count, 5950 cells/μL; normal, 65 years and renal insufficiency). The patient began oral prednisone (60 mg daily) and cyclophosphamide (2 mg/kg [125 mg] daily). Within 3 days both his painful rash and pulmonary symptoms substantially improved. He continues to do well as the prednisone dose is slowly tapered down.
2. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol. 2009;23(3):355-366. 3. Comarmond C, Pagnoux C, Khellaf M, et al; French Vasculitis Study Group. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Arthritis Rheum. 2013;65(1):270-281. 4. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011; 90(1):19-27. 5. Kawakami T, Soma Y, Kawasaki K, et al. Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome. Arch Dermatol. 2005;141(7):873-878.
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6. Sablé-Fourtassou R, Cohen P, Mahr A, et al; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143(9):632-638. 7. Ribi C, Cohen P, Pagnoux C, et al; French Vasculitis Study Group. Treatment of Churg-Strauss syndrome without poor-prognosis factors. Arthritis Rheum. 2008;58(2):586-594. 8. Gayraud M, Guillevin L, le Toumelin P, et al; French Vasculitis Study Group. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Arthritis Rheum. 2001;44(3):666-675. 9. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. Medicine (Baltimore). 1996;75(1):17-28.
(Reprinted) JAMA April 7, 2015 Volume 313, Number 13
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://jama.jamanetwork.com/ by a New York University User on 05/15/2015
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JAMA Clinical Challenge
Painful Rash in a Patient With Bronchiectasis Sruthi Renati, BS; Adam Raff, MD, PhD; Susan Burgin, MD
Figure. Left, Axial computed tomography scan of the chest revealing mild bronchiectasis and ground glass inflammation. Right, Purpuric plaque on the right medial ankle and dorsal foot, with central vesicles.
A 71-year-old man with a history of bronchiectasis presents with a painful rash and swelling of his right foot for 1 day. He has had no fevers, joint pain, recent trauma, or new exposures to the area. He was diagnosed with bronchiectasis of unknown etiology 6 years ago and notes a worsenQuiz at jama.com ing of his cough and dyspnea over the past few months. A recent computed tomography scan of the chest revealed mild bronchiectasis and ground glass inflammation (Figure, left), and recent pulmonary function tests showed combined obstructive and restrictive defects. His medications include albuterol inhaler, fluticasonesalmeterol inhaler, guaifenesin, and loratadine. On examination, the patient’s vital signs are normal. He has a purpuric plaque on the right medial ankle and dorsal foot, with central vesicles (Figure, right). The rash is tender to palpation, and there is associated nonpitting edema of the right foot and ankle. Laboratory values are notable for a leukocyte count of 15 700 cells/μL, with 38% eosinophils (absolute count, 5950 cells/μL; normal, 65 years and renal insufficiency). The patient began oral prednisone (60 mg daily) and cyclophosphamide (2 mg/kg [125 mg] daily). Within 3 days both his painful rash and pulmonary symptoms substantially improved. He continues to do well as the prednisone dose is slowly tapered down.
2. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol. 2009;23(3):355-366. 3. Comarmond C, Pagnoux C, Khellaf M, et al; French Vasculitis Study Group. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Arthritis Rheum. 2013;65(1):270-281. 4. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011; 90(1):19-27. 5. Kawakami T, Soma Y, Kawasaki K, et al. Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome. Arch Dermatol. 2005;141(7):873-878.
jama.com
6. Sablé-Fourtassou R, Cohen P, Mahr A, et al; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143(9):632-638. 7. Ribi C, Cohen P, Pagnoux C, et al; French Vasculitis Study Group. Treatment of Churg-Strauss syndrome without poor-prognosis factors. Arthritis Rheum. 2008;58(2):586-594. 8. Gayraud M, Guillevin L, le Toumelin P, et al; French Vasculitis Study Group. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Arthritis Rheum. 2001;44(3):666-675. 9. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. Medicine (Baltimore). 1996;75(1):17-28.
(Reprinted) JAMA April 7, 2015 Volume 313, Number 13
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://jama.jamanetwork.com/ by a New York University User on 05/15/2015
1367
