Rheumatoid Arthritis Associated With Pemphigus Foliaceus in a Patient Not Taking Penicillamine

Authors

  • Marimar Sáez-de-Ocariz MD,

    1. From the Department of Dermatology, Hospital “Dr Manuel Gea González”;1the Department of Dermatology, Instituto Nacional de Pediatría;2 and the Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición “Dr Salvador Zubirán”;3Mexico City, Mexico
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  • 1,2 Julio Granados MD,

    1. From the Department of Dermatology, Hospital “Dr Manuel Gea González”;1the Department of Dermatology, Instituto Nacional de Pediatría;2 and the Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición “Dr Salvador Zubirán”;3Mexico City, Mexico
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  • 3 Jesús K. Yamamoto-Furusho MD, MSc,

    1. From the Department of Dermatology, Hospital “Dr Manuel Gea González”;1the Department of Dermatology, Instituto Nacional de Pediatría;2 and the Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición “Dr Salvador Zubirán”;3Mexico City, Mexico
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  • 3 Alondra López-Martínez MD,

    1. From the Department of Dermatology, Hospital “Dr Manuel Gea González”;1the Department of Dermatology, Instituto Nacional de Pediatría;2 and the Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición “Dr Salvador Zubirán”;3Mexico City, Mexico
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  • and 3 María Elisa Vega-Memije MD 1

    1. From the Department of Dermatology, Hospital “Dr Manuel Gea González”;1the Department of Dermatology, Instituto Nacional de Pediatría;2 and the Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición “Dr Salvador Zubirán”;3Mexico City, Mexico
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Marimar Sáez-de-Ocariz, MD, Department of Dermatology. Instituto Nacional de Pediatráa, Insurgentes Sur 3700 C, Insurgentes Cuicuilco, 04530, Mexico City, Mexico
E-mail: mariadelmars@prodigy.net.mx

Abstract

A 50-year-old-woman presented with a history of crusted plaques on her face and trunk. On physical examination, she had a disseminated dermatosis involving the face, chest, trunk, abdomen, and both ankles. She had erythematous and scaly plaques on both cheeks (Figure 1). In both ears and on the rest of the body, she presented with scarce intact vesicles and blisters, a few eroded and crusted vesicles, and residual hyperpigmented macules. The onset of the dermatosis occurred 6 months before diagnosis, affecting the chest, followed by further spreading of vesicles on the body. She denied ever having developed ulcerative or erosive lesions on mucosal surfaces. The presence of blisters were followed by the development of crusts and hyperpigmented macules. One month before visiting our service, the patient had been receiving treatment with dapsone, with slight improvement of the cutaneous lesions. Biopsy results of one of the scaly plaques on the cheeks demonstrated a subcorneal blister with a few acantholytic cells and a scarce inflammatory infiltrate in the papillary dermis (Figure 2). Because of the clinical picture, especially the erythematous lesions on both cheeks, tests for antinuclear antibodies were performed to rule out pemphigus erythematosus, and the results were negative. Besides the cutaneous manifestations, the patient had had signs and symptoms of arthritis for at least 11 years; she had received sporadic relief with the nonsteroidal anti-inflammatory drug ketoprofen. She reported generalized morning rigidity lasting for 2 hours and arthralgias in the temporomandibular joints, elbows, wrists, metacarpophalangeal joints, knees, and ankles. On examination, she had phlogosis and synovial thickening in the elbows, wrists, metacarpophalangeal joints, knees, and ankles. She also had flexion contractures on both elbows as well as ulnar deviation of the left hand and swan-neck deformity in several fingers. Radiographic examination of the hands demonstrated generalized osteopenia, decreased articular spaces in metacarpophalangeal and proximal interphalangeal joints, and slight erosion in the carpal bones (Figure 3). Results of rheumatoid factor testing were positive (521 IU/mL). The diagnoses of both pemphigus foliaceus and rheumatoid arthritis were made. We performed high-resolution DNA typing of HLA-DRB1 polymorphism using sequence-specific oligonucleotides,1 and found that the patient carried HLA DRB1*0404 and *1406.

Figure 1.

Figure 1.

The patient had erythematous and scaly plaques on both cheeks.

Figure 2.

Figure 2.

Biopsy revealed a subcorneal blister with a few acantholytic cells and a scarce inflammatory infiltrate in the papillary dermis.

Figure 3.

Figure 3.

Radiography of the hands, revealed generalized osteopenia, decreased articular spaces in metacarpophalangeal and proximal interphalangeal joints, and slight erosion in the carpal bones.

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