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Rheumatology
Published in Paul Bentley, Ben Lovell, Memorizing Medicine, 2019
Eosinophilic fasciitis: Acute inflammation, and cobblestone-like induration of all extremities, after exertionFlexion contractures, carpal tunnel syndrome, myositis (CK normal)Aplastic anaemia, myelodysplasia
Differential diagnoses of systemic sclerosis
Published in Aparna Palit, Arun C. Inamadar, Systemic Sclerosis, 2019
Eosinophilic fasciitis is a disorder of unclear etiopathogenesis characterized by acute or subacute onset erythema and edema of the extremities, with or without involvement of the trunk. The acral areas are typically spared. The edematous stage is succeeded by induration and fibrosis mimicking scleroderma.
Clinical guide to eosinophilic fasciitis: straddling dermatology and rheumatology
Published in Expert Review of Clinical Immunology, 2022
Eosinophilic fasciitis (EF) has originally been described by Shulman in 1974 as ‘diffuse fasciitis with eosinophilia.’ The two patients in the original observation provided a history of strenuous exercise a few days before the onset of the circumferential swelling and induration of the skin in the distal areas of the extremities. The patients had no Raynaud’s phenomenon. En-bloc biopsies skin-to-fascia of the clinically involved tissues showed marked fascial thickening and inflammatory cell infiltration by lymphocytes and plasma cells. Peripheral eosinophilia in these patients was not matched by eosinophilic tissue infiltration. The patients responded well to oral corticosteroid therapy. Rodnan proposed for this disorder the name ‘eosinophilic fasciitis,’ the American College of Rheumatology proposed ‘diffuse fasciitis with or without eosinophilia,’ others used the label ‘Shulman syndrome,’ yet ‘eosinophilic fasciitis’ became the most widely accepted name for this disorder.EF begins with the acute or subacute onset of symmetrical erythema, edema, and induration of extremities, often also of the trunk, accompanied by elevated inflammatory markers. The lesions may progress to sclerosis of the subcutaneous tissue and fascia, complicating with flexion contracture of the fingers along with carpal tunnel syndrome. Visceral involvement is uncommon. Increased erythrocyte sedimentation rate may be present with disease activity.
Mycophenolate mofetil and mycophenolic acid for the treatment of eosinophilic fasciitis: report of two cases and literature review
Published in Journal of Dermatological Treatment, 2022
Ilse Andrea Moreno-Arquieta, Jesus Alberto Cardenas-de la Garza, Jorge Antonio Esquivel-Valerio, Janett Riega-Torres, Adrian Cuellar-Barboza, Maira Elizabeth Herz-Ruelas, Jorge Ocampo-Candiani, Dionicio Angel Galarza-Delgado
We identified cases of eosinophilic fasciitis by searching the medical records of the Dermatology Department from 2016 to 2020. The diagnosis of EF was made based on clinical presentation, histopathology, and imaging. A form was used to record clinical features, laboratory, and histopathologic findings at the time of the initial evaluation. Baseline variables included age, gender, pertinent physical exam findings, and the extent of skin involvement. The presence of peau d'orange/dimpling (induration of the skin with a dimpling/rippling/puckering or “pseudo-cellulite” appearance) and the groove sign (a linear depression where veins appear to be sunken within the indurated skin) were recorded. Laboratory studies included complete blood count, peripheral eosinophilia, erythrocyte sedimentation rate (ESR), and serum autoantibodies if performed. All medications used for the treatment of EF were recorded. The progress of the disease was evaluated at each subsequent visit and the clinical response was defined as complete (halted disease progression, resolution of erythema and edema, and improvement of induration), partial (halted disease progression with incomplete improvement of erythema and edema), or no response (continued disease progression).
Evaluating the use of JAK inhibitors in inflammatory connective tissue diseases in pediatric patients: an update
Published in Expert Review of Clinical Immunology, 2022
Jane Chuprin, Lindsay McCormack, Jillian M. Richmond, Mehdi Rashighi
Sclerodermiform syndromes are a group of autoinflammatory diseases that may be systemic or localized (also known as morphea, Figure 4). Both systemic sclerosis (SSc) and localized scleroderma (LS) are characterized by an autoinflammatory fibrotic process. In short, there is an initial inflammatory event, which triggers the deposition of excess collagen, ultimately leading to the pathologic hardening and thickening of affected tissue. Although less than 5% of SSc patients are children, LS is more likely to be present in childhood [43,61]. SSc is a potentially life-threatening, multi-system disease with a childhood incidence of 0.27 per million children per year in the UK and Ireland [62]. LS is limited to affecting the skin or underlying tissue, without any organ or vascular involvement, with a childhood incidence of 3.4 per million children per year in the UK and Ireland [62]. Eosinophilic fasciitis is another rare autoinflammatory disease thought to be related to LS. Unlike LS, eosinophilic fasciitis has an acute onset with painful, rapid, and symmetric scleroderma-like manifestation in extremities alongside eosinophilia in peripheral blood [43]. The LS Cutaneous Assessment Tool (LoSCAT) is a scoring index for LS and eosinophilic fasciitis and is a useful metric for clinical trial design studying pediatric populations [63].