BACKGROUNDLeprosy, a chronic infectious disease caused by Mycobacterium leprae, affects the skin, peripheral nerves, upper respiratory tract, musculoskeletal system and eyes. As it presents a wide spectrum of clinical manifestations, it can be a diagnostic challenge, especially in early stages of disease. Some of these manifestations resemble pictures of rheumatic diseases that affect adults and children. Among the musculoskeletal manifestations of childhood leprosy are inflammatory chronic arthritis, mimicking juvenile idiopathic arthritis or spondyloarthritis, inflammatory swelling of the hands and feet, neuropathic arthritis, septic arthritis, arthralgias/ myalgias, soft tissue rheumatism and multisystem involvement similar to collagenases, including vasculitis and myositis. CASE REPORTWe report the case of a previously healthy 8-year-old boy admitted to the pediatric rheumatology unity due to the presence of puffy hands and fingers and hard and shiny edema of the lower limbs, suggesting systemic scleroderma diagnosis. He had an 11-month history of recurrent episodes of intermittent fever, plaque-like, erythematous, nonpruritic facial skin lesions and joint pain in the knees and ankles. On physical examination, he presented, in addition to the aforementioned findings, purpuric lesions on the toes, hepatosplenomegaly, arthralgias (wrist, small joints of the hands, knees and ankles) and erythematous plaques infiltrated in the bilateral malar region, nasal region and auricular pavilion. Complementary tests showed normocytic and normochromic anemia, leukocytosis, neutrophilia, thrombocytosis, elevation of inflammatory tests and negativity of ANA, RF, ANCA, anti-RNP and anti-Scl70. Due to the infiltrated skin lesions, lymph smear testing was performed, which was strongly positive, and a diagnosis of Virchowian leprosy associated with mixed leprosy reaction was made. There was clinical and laboratory improvement after initiation of multidrug therapy and systemic corticosteroid therapy. CONCLUSIONLeprosy is known as a great mimic of rheumatic diseases, often fulfilling diagnostic criteria for many of them. The multibacillary forms present greater musculoskeletal involvement. It should be considered in differential diagnosis of children with musculoskeletal symptoms, autoantibody positivity and cutaneous and/or neurological involvement.
BACKGROUNDCatastrophic antiphospholipid syndrome (APS) is a severe acquired thrombophilia characterized by rapid development of thromboses in several organs, in presence of antiphospholipid antibodies. For histopathologic confirmation, thrombosis should be present without significant evidence of vessel wall inflammation. APS is a known association of systemic lupus erythematosus (SLE) and, when present with lupus, has the worst outcome. Peripheral vascular disease leading to digital gangrene is a well-recognized complication of APS, particularly in patients with SLE. Digital gangrene in SLE is a rare form of vascular injury and generally leads to digital amputation. CASE REPORTA previously healthy 12-year-old girl is brought to the emergency department because of a 3-day history of purpuric lesions in the lower limbs and digital gangrene in all right toes and in the second, third and fourth fingers on the left hand. She had rapid progression to gangrene in all the aforementioned digits and ulceration and gangrene of purpuric lesions. Arterial Doppler ultrasound of the four limbs showed no changes. Brain magnetic resonance imaging (MRI) showed lacunar infarcts of probable microembolic etiology. Laboratory findings included triple positivity in high titers of antiphospholipid antibodies, ANA and anti-DNA and hypocomplementemia, suggesting SLE and probable catastrophic APS diagnosis. Biopsy of skin lesions showed nonleukocytoclastic cutaneous small vessel vasculitis, leading to the hypothesis of lupus vasculitis. Methylprednisolone pulse therapy, human immunoglobulin, vasodilators and anticoagulation were performed. Due to the presence of lupus vasculitis, it was chosen to use cyclophosphamide. There was a halt in the progression of the vascular condition, but the patient evolved with self-amputation of some of the affected distal phalanges of the right toes and the left hand. After six doses of cyclophosphamide in a biweekly schedule, azathioprine was started, and the patient is currently asymptomatic with a SLEDAI-2K score of zero. CONCLUSIONDigital gangrene may be secondary to thrombosis or small vessel vasculitis. This distinction can be difficult, especially in patients with lupus-associated antiphospholipid syndrome. Only 0.2% of patients with SLE presented initially as digital necrosis and less than 10% of pediatric APS patients present with small-vessel thrombosis. Despite the rarity of presentation and the difficulty in distinguishing the diagnosis, early recognition and treatment of both diseases are essential to prevent progression and reduce their morbidity and mortality.
BACKGROUNDJuvenile spondyloarthritis is a chronic inflammatory disease that mainly affects boys over the sixth and under the 18 th year of life, accompanied by overlapping genetic susceptibility. It usually courses with arthritis, which is most commonly asymmetric, oligoarticular, involving the lower extremity large joints, associated with enthesitis, and may also progress with axial disease, especially sacroiliitis. In refractory cases, use of immunobiologicals of the tumor necrosis factor inhibitors (TNFi) class is mandatory. An increased risk infection is observed in patients undergoing biological treatment, including mycobacterial, fungal and viral infections. CASE REPORTA 17-year-old boy was previously diagnosed with juvenile spondyloarthritis 5 years ago with a history of chronic oligoarthritis of the lower limbs, including tarsitis, positive HLA-B27 and family history of ankylosing spondylitis, currently in use of adalimumab due to refractoriness to traditional disease-modifying antirheumatic drugs. He was admitted to the pediatric rheumatology ward due to worsening of joint condition and loss of 11 kg for 3 months. He was febrile, pale, with arthritis of the knees and ankles and deformity with rigid flat feet and flexion of toes. Complementary exams showed microcytic anemia, thrombocytosis and elevation of inflammatory tests. Initial infectious and neoplastic screening was negative, including tuberculin skin test. Tomography of chest and abdomen showed multiple calcified nodules in the hilum and paratracheal, mediastinal and subcarinal chains, pulmonary opacification suggestive of a tree-in-bud pattern, multiple foci of calcification in the liver and splenic parenchymas. Left ankle and knee magnetic resonance imaging (MRI) showed small oval lesions with postcontrast peripheral enhancement, identified in different bones of the ankle and in the anterior aspect of the tibia and fibula suggestive of dot circle sign. After discussion with an infectiologist, a hypothesis of disseminated fungal disease was raised. Serology for histoplasmosis was positive, amphotericin B deoxycholate was started with a good response and the use of adalimumab was discontinued.
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