A 52-year-old man comes to the physician with leg ulcers. Two months ago a small ulcer appeared on the right medial malleolus, and now there are 2 additional ulcers on both legs. He was diagnosed with seropositive rheumatoid arthritis 15 years ago and has been on oral prednisone, sulfasalazine, and hydroxychloroquine since then. Eight years ago methotrexate was discontinued due to liver toxicity. Five years ago he received etanercept for a few months, but this was discontinued due to frequent sinus infections.
The patient's other medical problems include gastritis, type 2 diabetes mellitus, and colon polyps. He is a cattle farmer and does not use tobacco, alcohol, or illicit drugs.
He is afebrile and vital signs are normal. Skin examination is notable for several nodules on the extensor surface of the elbows. There are 3 shallow ulcers on both legs in various stages of healing. Heart and lung sounds are normal. Spleen is palpable 2 cm below the left costal margin. There are rheumatoid deformities of both hands and feet with decreased range of motion in wrists, elbows, and ankle joints. Neurologic examination is normal.
Laboratory results are as follows:
Which of the following conditions is the most likely diagnosis?

Question

A 52-year-old man comes to the physician with leg ulcers.  Two months ago a small ulcer appeared on the right medial malleolus, and now there are 2 additional ulcers on both legs.  He was diagnosed with seropositive rheumatoid arthritis 15 years ago and has been on oral prednisone, sulfasalazine, and hydroxychloroquine since then.  Eight years ago methotrexate was discontinued due to liver toxicity.  Five years ago he received etanercept for a few months, but this was discontinued due to frequent sinus infections.
The patient's other medical problems include gastritis, type 2 diabetes mellitus, and colon polyps.  He is a cattle farmer and does not use tobacco, alcohol, or illicit drugs.
He is afebrile and vital signs are normal.  Skin examination is notable for several nodules on the extensor surface of the elbows.  There are 3 shallow ulcers on both legs in various stages of healing.  Heart and lung sounds are normal.  Spleen is palpable 2 cm below the left costal margin.  There are rheumatoid deformities of both hands and feet with decreased range of motion in wrists, elbows, and ankle joints.  Neurologic examination is normal.
Laboratory results are as follows:
 Which of the following conditions is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_9937_52f3_853d_ad62f29ec6d8_MD0002_00 This patient has rheumatoid arthritis, neutropenia, and splenomegaly, a triad known as Felty's syndrome (FS). It occurs in about 1% of patients with seropositive, advanced rheumatoid arthritis (usually > 10 years). Almost all patients have positive rheumatoid factor and anti-CCP antibodies. Patients can develop extra-articular manifestations such as mononeuritis multiplex, necrotizing skin lesions, pleuropericarditis, lymphadenopathy, and episcleritis. Patients are at increased risk for skin and respiratory tract infections due to neutropenia. Diagnosis requires bone marrow biopsy to rule out other causes of neutropenia, such as large granular lymphocyte syndrome. Treatment of FS is methotrexate and other disease-modifying antirheumatic drugs. Granulocyte colony-stimulating factor can be given to patients with life-threatening or refractory infections. (Choice A) Absence of renal manifestations and the presence of splenomegaly make polyarteritis nodosa unlikely. (Choice C) Necrobiosis lipoidica diabeticorum is a chronic, inflammatory skin condition most commonly seen in diabetics. The lesions typically appear as indurated plaques with central atrophy and yellow-brown pigmentation. (Choice D) Pyoderma gangrenosum is a skin condition frequently associated with systemic disorders such as inflammatory bowel disease. Patients present with painful, necrotic skin ulcerations. Pyoderma gangrenosum is not associated with splenomegaly. Pyoderma gangrenosum (Choice E) Sweet's syndrome is neutrophilic dermatoses associated with malignancy and autoimmune diseases (eg, inflammatory bowel disease). It presents with abrupt onset of erythematous plaques with pseudoblistering and pustules on the face, neck, chest, and extremities. It is frequently preceded by flu-like symptoms, and laboratory results show leukocytosis (not present in this patient). Educational objective: Felty's syndrome is a triad of seropositive rheumatoid arthritis, neutropenia, and splenomegaly. It is seen in patients with advanced rheumatoid arthritis (usually > 10 years). __________ References: Felty's syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/15454123) Cutaneous polyarteritis nodosa. (http://www.ncbi.nlm.nih.gov/pubmed/17544958) Etiology and management of pyoderma gangrenosum: a comprehensive review. (http://www.ncbi.nlm.nih.gov/pubmed/22356259)

A 52-Year-Old Man Comes to the Physician with Leg Ulcers