Histopathological changes in morphea and their clinical correlates: Results from the Morphea in Adults and Children Cohort V

Abstract

Background Histopathological features in morphea (localized scleroderma) and their clinical correlates are poorly described. Objective Systematically describe histologic changes of morphea in a large, well annotated cohort and determine the association between histopathology and clinical features. Methods Cross-sectional study of 83 patients enrolled in the Morphea in Adults and Children (MAC) cohort. The main outcome measure was the association of microanatomical location and degree of sclerosis and inflammation seen on histologic samples with patient reported symptoms and physician-based measures of severity. Results Pattern of sclerosis was associated with morphea subtype, the presence of patient reported symptoms, and functional limitation. A bottom heavy pattern of sclerosis was associated with pain and tightness (P = 0.0039, 0.001 respectively). These symptoms were not associated with a top heavy pattern. Severe inflammation may be associated with pain and functional limitation (p = 0.073 for both). Limitations Small sample size limits ability to detect associations, particularly in subgroups. Conclusions Histopathological examination of morphea may assist in identifying patients who may require additional monitoring and treatment. Features such as patterns of sclerosis and severity of inflammation should be included in pathology reports to help aid in clinical management.

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

“…In ECDS, sclerotic changes of the skin and hypoderma due to excessive collagen deposition, adnexal atrophy, and mononuclear cell infiltrates were revealed as well ( 6 , 61 ). Walker and colleagues underlined that severe inflammation might lead to pain and functional limitation in morphea ( 62 ). Perineural inflammation is common in morphea ( 29 ).…”

Panniculitides of particular interest to the rheumatologist

“…Deep morphea Morphea, or localized scleroderma, is a rare fibrosing disorder of the skin resulting from inflammation and deposition of collagen-rich extracellular matrix [19]. When sclerotic changes extend beyond the dermis to involve the subcutaneous tissue, the fascia or the superficial muscle, we refer to deep variant of circumscribed morphea (previously known as subcutaneous morphea or morphea profunda).…”

“…The overlying skin may feel hardened and adherent to the underlying tissues; however, acute inflammatory signs, such as edema and erythema, are rarely observed [21][22][23]. The presence of deep sclerosis, in some cases perineural, may account for the presence of pain [19].…”

Systemic Sclerosis Mimics

“…Systemic Sclerosis Mimics DOI: http://dx.doi.org /10.5772/intechopen.88546 demonstrates homogenization of the papillary dermis and thickened collagen bundles extending into the reticular dermis or beyond (Figure 8). In biopsy from deep morphea, the deep reticular dermis, subcutis, and fascia show sclerotic changes [14].…”

“…Furthermore, we need to think of lipodermatosclerosis as fibrosing panniculitis with typical localization on the lower extremities or eosinophilic fasciitis. In some cases of limb involvement, pretibial myxedema or Lyme disease (acrodermatitis atrophicans) must be excluded [1,13,14].…”