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Arthropathy and Cutaneous Eruption in a Patient With Cystic Fibrosis

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

An elementary school-aged girl with cystic fibrosis (CF) presented with a 2-year history of a relapsing-remitting, evanescent, salmon-pink maculopapular cutaneous eruption associated with joint pain. Each episode of joint pain and cutaneous eruption lasted 24 to 48 hours with episodes occurring anywhere from every other day to once weekly. The patient’s mother was unable to identify any possible triggers because the episodes occurred independent of pulmonary CF exacerbations or antibiotic use. The patient’s joint pain and cutaneous symptoms resolved completely between each episode and were not associated with fever. Review of photos taken during the patient’s flares revealed a variable eruption consisting of oval-shaped, salmon-pink macules on the trunk, urticarial-appearing papules on the dorsal hands, and flagellate arrays of petechial macules on the upper medial arms (Figure 1). This eruption was intermittently pruritic but otherwise was asymptomatic. Her joint pain recurred with each episode of cutaneous eruption and primarily affected the wrist, knee, and ankle joints bilaterally. She had been treated with naproxen during these flares, which improved her joint pain but not her cutaneous symptoms. A complete blood cell count and comprehensive metabolic panel as well as C-reactive protein and erythrocyte sedimentation rate obtained during a flare were normal. Titers for parvovirus, Epstein–Barr virus, and antistreptolysin O were also negative.

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B. Cystic fibrosis-associated episodic arthritis

Histopathologic examination revealed a normal epidermis overlying a mild perivascular and interstitial lymphocytic infiltrate with rare neutrophils (Figure 2). Examination also demonstrated negative direct immunofluorescence results for IgG, IgM, IgA, C3, and fibrinogen.

Cystic fibrosis-associated episodic arthritis (CFEA) is the more common of 2 arthropathies in patients with CF; the less common variety is termed hypertrophic pulmonary osteoarthropathy (HPOA).13 Cystic fibrosis-associated episodic arthritis is frequently associated with cutaneous manifestations including an erythematous maculopapular eruption, purpura of the legs, erythema nodosum, and cutaneous vasculitis.25 Patients have joint pain, swelling, and limited movement. Cystic fibrosis-associated episodic arthritis can be quite disabling, but causes no radiographic evidence of joint destruction.14 Cystic fibrosis-associated episodic arthritis flares typically occur independent of pulmonary CF exacerbations. In addition, they have a sudden onset and last 5 to 7 days before remitting spontaneously. Throughout the course, patients remain afebrile and constitutionally well.1,2 Rheumatoid factor and antinuclear antibodies are negative.2 Though circulating immune complexes detected by C1q binding are found in up to 30% of adult patients with CF, they are largely undetectable in the peripheral blood during CFEA flares.1,2,5 In addition, synovial fluid from CFEA-affected joints is sterile with little evidence of inflammation.2 However, synovial biopsies from several cases have shown IgM on immunofluorescence results, sometimes also with IgG, C3, and fibrinogen.2

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Article Information

Corresponding Author: Barrett J. Zlotoff, MD, Associate Professor of Dermatology, University of Virginia, PO Box 800718, Charlottesville, VA 22908 (bjz3a@virginia.edu).

Published Online: January 23, 2019. doi:10.1001/jamadermatol.2018.5018

Conflict of Interest Disclosures: The authors have no potential conflicts of interest to disclose.

Additional Contributions: We thank the patient’s mother for granting permission to publish this information.

References
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Wulffraat  NM, de Graeff-Meeder  ER, Rijkers  GT, van der Laag  H, Kuis  W.  Prevalence of circulating immune complexes in patients with cystic fibrosis and arthritis.  J Pediatr. 1994;125(3):374-378. doi:10.1016/S0022-3476(05)83279-2PubMedGoogle ScholarCrossref
2.
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Dixey  J, Redington  AN, Butler  RC,  et al.  The arthropathy of cystic fibrosis.  Ann Rheum Dis. 1988;47(3):218-223. doi:10.1136/ard.47.3.218PubMedGoogle ScholarCrossref
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Schidlow  DV, Goldsmith  DP, Palmer  J, Huang  NN.  Arthritis in cystic fibrosis.  Arch Dis Child. 1984;59(4):377-379. doi:10.1136/adc.59.4.377PubMedGoogle ScholarCrossref
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Schidlow  DV, Panitch  HB, Zaeri  N, Zenel  J, Alpert  BE.  Purpuric rashes in cystic fibrosis.  Am J Dis Child. 1989;143(9):1030-1032.PubMedGoogle Scholar
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Mahadeva  R, Dunn  AC, Westerbeek  RC,  et al.  Anti-neutrophil cytoplasmic antibodies (ANCA) against bactericidal/permeability-increasing protein (BPI) and cystic fibrosis lung disease.  Clin Exp Immunol. 1999;117(3):561-567. doi:10.1046/j.1365-2249.1999.01006.xPubMedGoogle ScholarCrossref
7.
Stringer  T, Gittler  J, Meehan  S, Kahn  P, Oza  VS.  Neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis.  Pediatr Dermatol. 2018;35(3):e170-e172. doi:10.1111/pde.13464PubMedGoogle ScholarCrossref
8.
Rye  PJ, Roberts  G, Staugas  RE, Martin  AJ.  Coagulopathy with piperacillin administration in cystic fibrosis: two case reports.  J Paediatr Child Health. 1994;30(3):278-279. doi:10.1111/j.1440-1754.1994.tb00635.xPubMedGoogle ScholarCrossref
9.
Dávila-Seijo  P, Hernández-Martín  A, Torrelo  A.  Autoinflammatory syndromes for the dermatologist.  Clin Dermatol. 2014;32(4):488-501. doi:10.1016/j.clindermatol.2014.02.004PubMedGoogle ScholarCrossref
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Nguyen  TV, Cowen  EW, Leslie  KS.  Autoinflammation: From monogenic syndromes to common skin diseases.  J Am Acad Dermatol. 2013;68(5):834-853. doi:10.1016/j.jaad.2012.11.002PubMedGoogle ScholarCrossref
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