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BACKGROUNDJuvenile localized scleroderma (JLS), also known as morphea, is a spectrum of rare inflammatory pediatric disorders associated with skin and underlying tissue thickening and sclerosis. Clinical manifestations range from skin lesions to extracutaneous involvement that often vary with its subtype. Early recognition is important to reduce disease-related functional impact. METHODSReview of medical records of children and adolescents with diagnosis of JLS from 2015 to May 2022 and regularly followed up in the Pediatric Rheumatology Unit of a tertiary hospital in the state of Ceará was performed. Data included demographics, clinical manifestations, treatment and disease course. RESULTSTwenty patients were included and 13 (65%) were female. The median age at onset, age at diagnosis and time elapsed from symptom onset to diagnosis were, respectively, 6.60 (2-16.4), 9.05 (3.2-17) and 2.45 (0-10.5) years. The most prevalent subtype was mixed morphea (35%), followed by linear form (25%). Involvement was predominantly appendicular, distributing in upper and/or lower limbs in 75%. Five patients had disease restricted to the face, two with en coup de sabre variant and three with Parry-Romberg syndrome. Most of the patients (75%) already had characteristics of long-standing skin disease at diagnosis. Seventeen patients (85%) had extracutaneous manifestations, with a predominance of joint involvement (arthritis/arthralgia of small joints of the hands, wrists and knees) in 13 (65%) of them, followed by gastrointestinal tract (20%). Autoantibodies were positive in 9 (45%) patients, of which ANA (78%) and RF (22%) were the most prevalent. Eighteen patients used corticosteroids at diagnosis, with an average of 8.5 months treatment, and a partial response in 15 (83.3%) of them. Methotrexate was associated in 19 cases, allowing 12 (63.1%) patients to achieve remission. In refractory cases (35%), six received mycophenolate mofetil and two cyclophosphamide. After approximately 3 years of follow-up, 85% of patients had good disease control. CONCLUSIONThe female-to-male ratio (1.85) and the age at onset of symptoms (6.60) are in line with literature (1.7:1 to 3.7:1 and 6.1 to 8.1, respectively). Our study differs in the higher prevalence of mixed morphea subtype in relation to the linear one. Time until diagnosis was long, with most patients presenting already with chronic skin changes, warning about the lack of knowledge about the disease. Immunosuppressive standard treatment, mainly with methotrexate and corticosteroids, shows promising results in controlling the disease progression.

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.

BACKGROUND Pediatric rheumatology (PR) has been formally recognized for a few decades in Brazil, with medical training centers concentrated in southeast of country. The need to expand specialists number in area, as well as to improve health care of pediatric patients with rheumatic diseases in region, was the reason for creation of a new unit in Ceará. MATERIALS AND METHODS Descriptive and observational study about implementation of a PR unit in a tertiary public hospital in Ceará. Data were collected based on internal reports and electronic spreadsheets filled monthly since May 2010 and analyzed in May 2019. RESULTS This hospital has reference services in rheumatology and pediatrics, as well as medical residences in both areas, solid bases for creation of a well-structured PR unit. Outpatient care started in May 2010 and in March 2013 the PR medical residency was founded, being only one in North and Northeast of Brazil. This made it possible to increase specialists number from two in 2013 to six in 2019, performing in main public and private hospitals. The unit receives medical graduation students and medical residents of pediatrics and rheumatology, being currently a required practice scenario. The resident physician training is based on patients follow-up in inpatient and outpatient clinic of PR, but also has other action fields, such as infusion center of immunobiologics, infiltration outpatient clinic, outpatient clinics of orthopedic, immunology and dermatology , autoimmunity laboratory, among others. Regarding clinical research, the unit is included in multicentric Brazilian studies and counts on own scientific productions. It actively participates in local, national and international events, having already promoted two regional scientific ones (2017 and 2019) aimed at general rheumatologists and pediatric rheumatologists from the north and northeast, establishing as one of centers responsible for continuing education. With regard to health care, from May 2010 to May 2019, 2,168 patients were evaluated in 12,156 visits (1,350 visits / year), and currently 978 patients are in regular follow-up. Since 2013 to the present time, 928 hospital admissions were performed on the inpatient unit (154 / year).