Dactylitis and Bone Lesions at the Onset of Sarcoidosis: a Case Report
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CASE REPORT Dactylitis and bone lesions at the onset of sarcoidosis: a case report Daniela Fodor1, Laura Poanta2, Liliana Rogojan2 1 2nd Internal Medicine Clinic, University of Medicine and Pharmacy, Cluj‑Napoca, Romania 2 Histopathology Department, University of Medicine and Pharmacy, Cluj‑Napoca, Romania KEY WORDS AbSTRACT bone cysts, dactylitis, Dactylitis and bone lesions are rare complications of sarcoidosis that occur in the chronic disease sarcoidosis and they are unusual features of the disease at presentation. The present paper describes a case of a 28-year-old woman with dactylitis (due to tenosynovitis and soft tissue granulomas) and pha- langeal bone lesions in 2 fingers at the onset of sarcoidosis. She also had asymptomatic pulmonary type I sarcoidosis (bihilar lymph node enlargement with no involvement of the lung parenchyma). The response to treatment (prednisone 30 mg/day, tapered to 5 mg over 2 months for a 12-month period) was very good, with no relapse at 6 months after the end of systemic treatment. INTRODUCTION Sarcoidosis is an inflamma‑ response to non‑steroid anti‑inflammatory treat‑ tory disorder of unknown cause, characterized ment. Prior to the onset she had never had symp‑ by the presence of non‑caseating granulomas toms about the hands or other joints. She had no in the tissues. The disease affects multiple or‑ constitutional symptoms, like cough, fever, short‑ gans, most commonly the lung, the lymph nodes, ness of breath, and weight loss. the skin and the eyes, but any other organ in‑ The clinical examination revealed the swelling volvement is possible. Skeletal involvement has of the right index, from meta carpophalangeal been reported in 1–13% of patients with sarcoid‑ joint to the finger tip on the flexor part and osis, with an estimated average of 5%. The mus‑ along the second phalanx on the extensor part. culoskeletal involvement in sarcoidosis usually At the flexor side of the left thumb there was occurs in patients with a generalized (systemic) a swelling distal to the meta carpophalangeal joint. disease and it is believed to be a sign of a chronic The involved areas were slightly warm and tender and prolonged clinical course.1 While bone lesions and there was an important restriction of the joint of the phalanges are more common, dactylitis is motion in the affected joints (FIGURE 1). The find‑ a rare rheumato logic complication of sarcoidosis, ings of the rest of clinical examinations (the re‑ and an association of sarcoidosis of soft tissues maining musculoskeletal system, the respirato‑ Correspondence to: and bones of the hand is infrequent. ry and the cardiac systems, the abdomen and Assistant Professor Laura Poanta, MD, PhD, II Internal Medicine Clinic, We present a case of sarcoidosis with soft tis‑ the skin) were normal. University of Medicine and Pharmacy, sue manifestations (dactylitis due to tenosynovi‑ The antero‑posterior and lateral radiographs “Iuliu Hatieganu”, 2–4 Clinicilor Street, tis and soft tissue granulomas) and phalangeal of the patient’s hands showed the pres ence 400006, Cluj‑Napoca, Romania, phone: +40‑744‑894‑190, bone lesions that appeared as early as at the on‑ of cysts in the thumb’s 1st phalanx head and fax: +40‑264‑596‑912, set of the disease. the index’s 1st and 2nd phalanx head, with no e‑mail: [email protected] significant cortical disruption, a minor narrowing Received: August 5, 2008. CASE REPORT The patient, a 28‑year‑old Cau‑ of the proximal inter phalangeal spaces of both fin‑ Revision accepted: September 9, 2008. casian woman, working as a PC operator, with‑ gers and the swelling of soft tissue (FIGURE 2). Conflict of inter est: none declared. out a previous medical history, nonsmoker, pre‑ The ultrasonography showed severe tenosyno‑ Pol Arch Med Wewn. 2008; sented with a 2‑month history of pain, swell‑ vitis of the flexor sheath of both fingers, with hy‑ 118 (12): 774‑777 Copyright by Medycyna Praktyczna, ing and severe disability of the right index and pervascularisation in, and mostly around����������, ��������the ten‑ Kraków 2008 left thumb, with progressive worsening and no don, and a clear definition of the tendon sheath 774 POLSKIE ARCHIWUM MEDYCYNY WEWNĘTRZNEJ 2008; 118 (12) The pulmonary radiography showed bihilar lymph nodes enlargement with no radio logical in‑ volvement of the lung parenchyma, inter preted as type I sarcoidosis. The absence of pulmonary pa‑ renchyma involvement was confirmed on the com‑ puted tomography examination. The patient received systemic corticosteroids, prednisone 30 mg/day, tapered to 5 mg over 2 months for a 12‑month period. She was called for a check‑up on every 3 months. The fingers returned to the normal clinical appearance and the pulmonary aspect became normal. Six months after the end of the systemic treatment there was no relapse. DISCUSSION Joint involvement in sarcoidosis often manifests itself as arthralgia, but deforming arthritis is rare.1,2 There are 2 patterns of sarcoid‑ al arthropathy. The early pattern is encountered in Löfgren syndrome, the acute type of the on‑ FIGURE 1 Clinical from the surroundings tissues. At the extensor set of sarcoidosis occurring in the first 6 months aspect of the right index part of the index phalanx a hypoechoic mass sit‑ of symptoms. Polyarthralgia (of ankles, knees, uated between the bone and the extensor tendon proximal inter phalangeal joints, wrists, and el‑ was identified. Some irregularities of bone con‑ bows) is common, but conventional radiographs tours were present, but with no fluid or synovi‑ of the symptomatic joints show normal joints tis inside the joints. or only osteoporosis and the swelling of soft tis‑ Laboratory tests revealed a normal peripher‑ sue2. The 2nd form is observed after 6 months or al blood count, C‑reactive protein and serum fi‑ more from the diagnosis and it usually involves brinogen levels and a normal erythrocyte sedi‑ 2 or 3 joins (ankles, knees, proximal inter‑ mentation rate. Blood chemistry was normal (in‑ phalangeal joints, wrists or shoulders). In this cluding calcium and alkaline phosphatase levels) form, dactylitis can occur. Radiographic chang‑ and the serology revealed a negative rheumatoid es usually consist of cystic bone lesions.2,3 Bone factor and anti‑cyclic citrullinated peptide anti‑ involvement is reported to have a worse progno‑ bodies. The tuberculin skin test, the serum angio‑ sis, with 4 times higher mortality rate compared tensin‑converting enzyme level and the human to the patients with normal bone findings, but al‑ leukocyte antigens type were not evaluated. most half of the patients have no symptoms re‑ A surgical bio psy from the extensor part lated to bone involvement in spite of abnormal FIGURE 2 Radiography of the index was performed. The patho logic tis‑ radiographic findings.3 of the left thumb and sue had a yellowish appearance with a good de‑ In the discussed patient’s case the onset right index. Bone cysts, lineation from the skin, the extensor tendon of the disease, with asymmetric dactylitis that slightly narrowing of and the bone. Histopatho logical findings were developed in a short period of time and with no proximal inter phalangeal of non‑caseating granuloma composed of epi‑ constitutional complains, was the starting point joints spaces, soft-tissue thelial cells, lymphocyte and occasional Langer‑ of the diagnosis. tumefaction are seen hans giant cells. The term of dactylitis is used to describe the in‑ flammation of a finger or a toe. In clinical practice, dactylitis is considered a hallmark feature of spon‑ dyloarthritis especially of psoriatic arthritis. Dac‑ tylitis is so specific for spondyloarthritis that it was included in the clinical criteria for the clas‑ sification and the diagnosis of the whole disease group.4 In spondyloarthritis the aspect of “sau‑ sage‑like” digit is due to the flexor tenosynovitis and sometimes to the enlargement of the joint. In spite of this high specificity of dactylitis for spondyloarthritis a differential diagnosis should be carefully considered. In tuberculosis dactyli‑ tis, short tubular bones of hands and feet are af‑ fected by tuberculous osteomyelitis (the aspect of spina ventosa on the radiography). Syphilitic dactylitis is encountered in congenital syphilis and the manifestations are bilateral and symmet‑ ric. Blistering distal dactylitis is attributed to in‑ fection with group A β‑hemolytic Streptococcus or Staphylococcus aureus, involving the volar fat pad CASE REPORT Dactylitis and bone lesions at the onset of sarcoidosis: a case report 775 of the distal phalanx of the digits, and it usually bone involvement and a good prognosis. The pa‑ presents as a fluid‑filled blister. In sickle cell dac‑ tient discussed here had dactylitis as the 1st mani‑ tylitis, (“hand‑foot syndrome”) bone marrow in‑ festation of the disease, her response to the treat‑ farction of the carpal and tarsal bones and pha‑ ment was very good and she also had a good prog‑ langes develops. Gouty dactylitis is a rare finding, nosis. The different course of dactylitis at the on‑ seen in a chronic polyarticular disease.4,5 set compared to dactylitis that develops during The current patient did not fulfill the diagnos‑ sarcoidosis is more likely to be related to caus‑ tic criteria for all these diseases. Thus the deci‑ ative factors than to the immuno logic pathogen‑ sion was made to perform a surgical bio psy from esis of the disease. the extensor part of the index, and this area was chosen for 2 reasons: first, because it was the most REFERENCES painful region, with a great difficulty in extension 1 Koyama T, Ueda H, Togashi K, et al. Radio logic manifestations of sarcoi- of the finger, and second, in order to avoid an un‑ dosis in various organs. RadioGraphics. 2004; 24: 87-104. necessary tendon sheath dissection. The histo‑ 2 Costabel C.