1 - Unidade de Reumatologia Pediátrica, Centro Hospitalar Universitário Lisboa Central, Lisboa
2 - Unidade de Radiologia, Centro Hospitalar Universitário Lisboa Central, Lisboa
- 28th European Paediatric Rheumatology Congress,PRES2022, Praga, Setembro 2022. Publicação sob a forma de poster com apresentação
Introduction: Eosinophilic fasciitis is a rare disorder presenting with skin edema and erythema, followed by collagenousn thickening of the subcutaneous fascia. Its pathogenesis is poorly understood and been associated with multiple conditions, such as strenuous exercise, exposure to certain medications as well as hematological and autoimmune diseases.
Objectives: Increase awareness for the diagnosis of eosinophilic fasciitis.
Methods: Report of a patient with eosinophilic fasciitis and juvenile systemic lupus erythematosus
Results: A 17-year-old girl was referred to a tertiary hospital with a 9-month history of fatigue and migratory arthralgias. In the previous month, she also reported limb edema and a generalized photosensitive rash.Her medical history was unremarkable, with no regular medication. Her sister had been diagnosed with juvenile systemic lupus erythematosus. Physical examination revealed a disseminated maculopapular lupus rash with scaly appearance, as well as a malar rash. There was also bilateral erythema, edema and thickening of the skin of the four limbs and trunk, sparing the fingers and toes. Laboratory investigations showed non regenerative anemia, eosinophilia, elevated erythrocyte sedimentation rate, elevated transaminases and LDH; positive ANA, anti-double-stranded DNA, anti-nucleosome, antihistone, RF and CCP; low levels of C3 and functional CH50 complement. Direct Coombs test, creatine-kinase, creatinine, urea, electrolytes, urine analysis and thyroid hormones were within normal limits. Systemic sclerosis antibody panel and antiphospholipid antibodies were negative. Diffusing capacity for carbon monoxide, cardiac ultrasound and ECG were normal. Nailfold capillaroscopy revealed a normal pattern. Skin biopsy revealed an infiltrate of lymphocytes and plasma cells surrounding the superficial and deep vessels, the adipocytes and the skin derivatives in the dermis. In immunofluorescence, a dense linear pattern of IgM was found in the dermoepidermal junction. Whole-body MRI showed edema and thickening of the fascia and subcutaneous tissue, affecting the trunk and the limbs. The diagnosis of juvenile erythematous lupus and eosinophilic fasciitis was established and treatment with high dose steroids and hydroxychloroquine was started. During the following months, clinical stability was achieved and steroids were gradually tapered. However, skin edema recurred and she developed an irregular, peau d'orange texture and a “groove sign” of the arms. Methotrexate was added after nine months of the diagnosis. The patient responded favorably and steroids were suspended. During the last 2 years of follow-up there was no recurrence of the eosinophilic fasciitis.
Conclusion: Eosinophilic fasciitis is a scleroderma-like syndrome predominantly affecting the extremities, sporadically seen in children. In contrast to systemic sclerosis, sclerodactyly, Raynaud's phenomenon, internal organ involvement and nailfold capillaries abnormalities are uncommon. Early recognition and treatment are essential in order to prevent longterm disabling outcomes in eosinophilic fasciitis. Myalgias, fatigue and, less frequently, arthritis are commonly associated symptoms. Eosinophilia is a prominent laboratory finding in the early phase of this disease. MRI is an important tool for the diagnosis, particularly when the skin biopsy is inconclusive. Eosinophilic fasciitis has been described in association with collagen tissue diseases, such as Sjögren's syndrome and systemic lupus erythematosus. To the best of our knowledge, this is the first reported case of juvenile eosinophilic fasciitis associated with inaugural systemic lupus erythematosus.