Which histologic findings are characteristic of scleroderma?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Answer

The histopathological findings in the skin include marked thickening of the dermis with massive accumulation of dense collagen causing epidermal atrophy, flattening of the rete pegs, and replacement of sebaceous and sweat glands, as well as hair follicles (see image below). A prominent inflammatory infiltrate is often present at the dermal-adipose tissue interphase, especially in early lesions. The small vessels of the lower dermis show fibrous thickening but evidence of vasculitis is absent.

Skin biopsy showing extensive fibrosis. The biopsy Skin biopsy showing extensive fibrosis. The biopsy has a square morphology, which reflects the rigidity of the tissue biopsy specimen due to striking pan-dermal sclerosis. In addition, the fibrosing reaction extends into the panniculus. The number of adnexal structures is reduced, another characteristic feature of scleroderma. A significant inflammatory cell infiltrate is not observed. This is in contradistinction to morphea, in which a prominent inflammatory cell infiltrate is present.
Skin biopsy showing severe fibrosis. The fibrosis Skin biopsy showing severe fibrosis. The fibrosis reflects a widening of collagen bundles in concert with an increase in the number of collagen fibers. Note the superimposed deposition of the newly synthesized delicate collagen bundles interposed between the preexisting collagen bundles, the latter appearing wide and manifesting a hyalinized morphology.

In the lungs, fibrosis of the alveolo-capillary membrane and the parenchymal interstitium accompanied by severe mononuclear cell infiltration is present frequently, resulting in marked disruption of their architecture. Prominent vascular abnormalities with intimal proliferation causing narrowing or complete obliteration of small vessels are frequent (see image below).

Lung biopsy demonstrating severe interstitial fibr Lung biopsy demonstrating severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of a pulmonary arteriole compatible with pulmonary hypertension.
Lung biopsy demonstrating expansion of the interst Lung biopsy demonstrating expansion of the interstitium of the lung by fibrous tissue along with chronic inflammatory cells.

The renal lesions display severe narrowing and obliteration of the medium-size arterioles due to sub-intimal accumulation of loose connective tissue and intimal and perivascular fibrosis. The glomeruli frequently appear ischemic and there is no evidence of glomerulitis. Severe interstitial, perivascular, and periglomerular fibrosis may be present in cases of long duration.

Other affected organs display variable degrees of fibrosis, mononuclear cell infiltration and microvascular obliteration and fibrosis.


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