Ischemic fasciitis M72.40; M72.41; M 72.42;

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

atypical decubital fibroplasia; Atypical decubital fibroplasia; ischemic fasciitis

History
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Montgomery EA et al. 1992

Definition
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Pseudosarcomatous (reactive?), fibrohistiocytic tumor described in 1992 by Montgomery et al. as "atypical decubital fibroplasia".

Etiopathogenesis
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Unknown; recurrent, pressure-related, intermittent ischemia may play a pathogenetic role (Lehmer LM et al. 2016). This mechanism could explain the preferred localisation of the pseudosarcomatous tumour and the coexistence of regenerative and degenerative connective tissue reactions.

Manifestation
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Mainly immobile patients in the 8th and 9th decade of life.

Localization
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Shoulder, posterior thoracic wall, greater trochanter, elbow, arms and legs more rarely.

Clinical features
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Clinically, the "reactive" tumour presents as a slow-growing, painless, skin-coloured, firm, subcutaneous swelling, which generally does not exceed 6.0-7.0 cm in size (Lehmer LM et al. 2016). A decubitus is often detectable.

Histology
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Lobular change in the deep subcutis involving the adjacent muscles with fibrinoid necrosis zones and a myxoid stromal. Large atypical fibroblasts with hyperchromatic nuclei are detectable. The proliferating cells were positive for vimentin, collagen type IV, CD68, but negative for CAM 5.2, epithelial membrane antigen, desmin, smooth muscle actin, muscle actin, S-100 protein and CD3e and showed a fibroblastic nature by electron microscopy. A cytogenetic translocation of t (1;2) (p36.1; q23) and t (7;19) (q32; q13.3) can be detected (Sachak T et al.2018).

Differential diagnosis
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Therapy
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Surgical removal. High risk of recurrence.

Literature
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  1. Baldassano MF et al (1998) Atypical decubital fibroplasia: a series of three cases.J Cutan catheter 25:149-152.
  2. Fukunaga M (2001) Atypical decubital fibroplasia with unusual histology. APMIS 109:631-635.
  3. Lehmer LM et al (2016) Ischemic fasciitis: enhanced diagnostic resolution through clinical, histopathologic and radiologic correlation in 17 cases. J Cutan Pathol 43:740-748.
  4. Montgomery EA et al (1992) Atypical decubital fibroplasia. A distinctive fibroblastic pseudotumor occurring in debilitated patients. On J Surg pathogen 16:708-715.
  5. Sachak T et al.(2018) Novel t(1;2)(p36.1;q23) and t(7;19)(q32;q13.3) chromosomal translocations in ischemic fasciitis: expanding the spectrum of pseudosarcomatous lesions with clonal pathogenetic link. Diagn Pathol 13:18.

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Last updated on: 29.10.2020