Pansclerotic Morphea with Features of Eosinophilic Fasciitis: Distinct Entities or Part of a Continuum?

Abstract

Scleroderma is a highly complex disorder in its clinical manifestations and pathogenesis. It has a wide range of clinical manifestations due to varying degrees of vasculopathy, autoimmunity, altered endothelium function, and abnormal fibrosis. The most widely used classification system grouped eosinophilic fasciitis and disabling pansclerotic morphea of childhood into the category of deep morphea. This previous classification does not include a category for overlapping conditions. A proposed new classification includes a new mixed subtype in which a combination of two or more of the previous subtypes is present in the same individual, although eosinophilic fasciitis has been excluded. We present the case of a 4-year-old boy who presented with features of disabling pansclerotic morphea and eosinophilic fasciitis simultaneously, which to our knowledge has not been previously reported. This suggests that these diseases are part of a more closely related continuum rather than separate disorders, as currently classified.

Eosinophilic fasciitis: clinical characteristics and response to methotrexate

“…Terms like generalized morphea or deep morphea have been used sometimes to describe patients with EF, especially in dermatology. Also, there are case reports of patients with co‐existent lesions of EF and deep morphea . All our patients presented with superficial morphea lesions and they responded to therapy with steroids and MTX used to treat EF.…”

Granulomatous fasciitis followed by morphea profunda: Is granulomatous fasciitis part of a spectrum of deep morphea? A case report and review of the literature

“…Deep morphea, or morphea profunda, is one of the more rare clinical variants of morphea and can extend into the fascia and muscle . Morphea has been associated with both eosinophilic fasciitis and Lyme disease . The pathogenesis of morphea is poorly understood, but environmental factors such as radiation, trauma, or infection have been proposed as a trigger.…”

“…Of interest to our case, there is also an ongoing discussion as to whether EF is a variant of morphea. There have been cases in which morphea and EF coexist; however, the combination is considered to be rare . The first association was reported by Lakhanpal in which 15 of 52 patients with EF also had morphea .…”

Metastatic Squamous Cell Carcinoma in a Patient with Disabling Pansclerotic Morphea of Childhood

“…Scleroderma is a spectrum of fibrosing diseases ranging from localized morphea to systemic sclerosis. Within the first group is disabling pansclerotic morphea of childhood ( DPMC ), with approximately 30 cases reported in the literature . DPMC generally begins before the age of 14 years, typically presenting with sclerotic plaques on the trunk and extensor areas progressing to the entire skin surface, eventually affecting muscle and bone .…”

“…DPMC generally begins before the age of 14 years, typically presenting with sclerotic plaques on the trunk and extensor areas progressing to the entire skin surface, eventually affecting muscle and bone . Walking becomes impossible because of contractures and mutilation caused by sclerosis, and patients often have joint contractures, calcinosis cutis, and nonhealing ulcers . The prognosis is poor, with severe orthopedic, psychological, and cosmetic disturbances, and the condition is often refractory to any treatment .…”