A case of eosinophilic fasciitis and generalized morphea overlap

Watanabe, Yuzuru; Yamamoto, Miyuki; Yamamoto, Toshiyuki

doi:10.5070/d3262047425

Cited by 3 publications

(4 citation statements)

References 9 publications

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“…Luo G et al report a case of overlap of the two diagnosed on biopsy in a 45-year-old patient with long-standing skin sclerosis and also found to have IgA nephropathy [ 14 ]. Lakjiri et al report a 45-year-old with twenty years of vitiligo who got diagnosed with this overlap syndrome [ 15 ]. Watanabe et al also reported a case of a 60-year-old male with differential expression of CD34 in both types of lesions, discussing the origin from different mesenchymal cells and emphasizing that the two conditions are independent entities [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

A Severe Case of Overlap of Morphea and Eosinophilic Fasciitis after Burn Injuries

Sami,

Sami

et al. 2024

Case Reports in Rheumatology

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Background. Generalized morphea is a rare fibrosing skin illness that progresses from erythematous, violet-colored skin patches to sclerotic plaques. Another uncommon immune-mediated connective tissue disease called eosinophilic fasciitis (EF) evolves to cause sclerosis and woody skin induration. The coexistence of the two is extremely rare and has a poorer prognosis. Our case report is one of the first to report burn injuries as a trigger factor for EF and generalized morphea overlap. Case Presentation. A 36-year-old man presented with acute onset of rapidly progressing skin thickening, tender edema, and skin contractures involving all extremities, shortly after enduring burn injuries from a gasoline explosion. Workup was remarkable for peripheral eosinophilia, hypergammaglobulinemia, and elevated C-reactive protein. Skin biopsy demonstrated sclerodermoid changes and sclerotic thickening of subcutaneous fibrous septa associated with stromal mucin, dermal perivascular, diffuse lymphoplasmacytic infiltrate with eosinophils, decreased CD34 expression, and increased factor XIIIa. He was subsequently diagnosed with an overlap of generalized morphea and eosinophilic fasciitis. The patient had only limited improvement with steroids, methotrexate, mycophenolate mofetil, and intralesional triamcinolone acetonide injections. Conclusion. Generalized morphea with concomitant EF indicates some degree of therapeutic resistance and poor prognosis with a low quality of life. Burn injuries can be a trigger factor for this overlap syndrome. Prompt identification of at-risk individuals and initiating aggressive management are necessary.

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Section: Discussionmentioning

confidence: 99%

A Severe Case of Overlap of Morphea and Eosinophilic Fasciitis after Burn Injuries

Sami,

Sami

et al. 2024

Case Reports in Rheumatology

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“…Es más frecuente entre los 40 y 50 años, por igual en ambos sexos 2,4 o con leve predominio en los hombres 3,5 .…”

Section: Comentariosunclassified

“…También se puede hacer diagnóstico diferencial con la morfea 1,5 . En la FE hay afectación simétrica, una fase inicial inflamatoria y eosinofilia periférica y una diferente distribución de la esclerosis cutánea.…”

Section: Comentariosunclassified

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Cuando unos edemas no son lo que parecen

Fornés Ollé,

Boada Peiró,

Cuberas Mas

et al. 2023

Med Gen Fam

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Historia del artículo:Recibido el 24 de octubre de 2022 Aceptado el 30 de abril de 2023On-line el 7 de mayo de 2023 La fascitis eosinofílica es una patología poco frecuente del tejido conectivo de etiología desconocida que requiere de un rápido diagnóstico porque el pronóstico mejora si se inicia el tratamiento precozmente y porque un 10 % puede asociarse a enfermedades hematooncológicas.Debemos sospechar la presencia de una fascitis eosinofílica ante un cuadro de eritema y edema generalizado en las extremidades, que no responde a diuréticos, que progresa a induración de la piel, acompañado de dolor y rigidez articular, eosinofilia periférica, elevación de reactantes de fase aguda e hipergammaglobulinemia.Procede remitir a Dermatología de forma urgente para confirmar mediante biopsia profunda de la piel y así iniciar tratamiento precoz con immunosupresores para lograr un curso más favorable y con menos secuelas articulares.

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