Ann Dermatol.  2017 Jun;29(3):268-275. 10.5021/ad.2017.29.3.268.

Periostin in Mature Stage Localized Scleroderma

• Kim MW
• Park JT
• Kim JH
• Koh SJ
• Yoon HS
• Cho S
• Park HS

• KMID: 2378524
• DOI: http://doi.org/10.5021/ad.2017.29.3.268

Abstract

BACKGROUND
Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare.
OBJECTIVE
To investigate the expression of periostin and other molecules in localized scleroderma.
METHODS
A retrospective study of 14 patients with confirmed mature stage localized scleroderma was undertaken. Fourteen age-matched and biopsy site-matched subjects with normal skin were included as controls. Collagen fiber deposition, periostin, procollagen, transforming growth factor-β, and matrix metalloproteinase (MMP)-1 expression were assessed and compared between the two groups. Co-localization of α-smooth muscle actin and periostin was evaluated using confocal microscopy.
RESULTS
Periostin was predominantly expressed along the dermo-epidermal junction in the controls. Conversely, patients with localized scleroderma demonstrated increased collagen fiber deposition and periostin expression that was more widely distributed along the entire dermis. MMP-1 staining showed increased expression in the epidermis and dermis of patients compared to scanty expression in the controls. A semi-quantitative evaluation showed a higher proportion of excessive collagen bundle deposition (57.1% vs. 7.1%, p=0.013), diffuse periostin positivity (42.9% vs. 0%, p=0.016), and moderate MMP-1 positivity (71.4% vs. 7.1%, p=0.001) in patients than in the controls.
CONCLUSION
Compared to the controls, patients with localized scleroderma had enhanced periostin expression corresponding to increased collagen fiber deposition and unexpected overexpression of MMP-1. The results of this human in vivo study may implicate the pathogenesis of localized scleroderma.

Keyword

Collagen; Localized scleroderma; Pathogenesis; Periostin; Sclerosis

MeSH Terms

Actins
Biopsy
Collagen
Dermis
Epidermis
Humans
Microscopy, Confocal
Periodontal Ligament
Procollagen
Retrospective Studies
Scleroderma, Localized*
Sclerosis
Skin
Actins
Collagen
Procollagen

Figure

• 

  Fig. 1 (A) A representative image of localized scleroderma specimen revealing thick and hyalinized collagen bundles (H&E, ×50). (B) A representative image of control skin without evidence of pathologic findings (H&E, ×50). (C) Excessive collagen bundle deposition in the same scleroderma specimen (Masson trichrome, ×50). (D) Moderate amount of collagen fibers in the same control (Masson trichrome, ×50). (E) Diffuse periostin positivity through the whole dermis in the same scleroderma specimen (anti-periostin antibodies, ×50). (F) Focal positivity of periostin mainly along dermo-epidermal junction in the same control (anti-periostin antibodies, ×50). (G) Moderate matrix metalloproteinase (MMP)-1 positivity in the epidermis and dermis in the same scleroderma specimen (anti-MMP-1 antibodies, ×50). (H) Scanty MMP-1 positivity in the same control (anti-MMP-1 antibodies, ×50). (I) Scanty TGF-β expression in the scleroderma specimen (anti-TGF-β antibodies, ×50). (J) Scanty TGF-β expression in the control (anti-TGF-β antibodies, ×50). (K) Scanty procollagen expression in the scleroderma specimen (anti-procollagen 1 antibodies, ×50). (L) Scanty procollagen expression in the control (anti-procollagen 1 antibodies, ×50).

• 

  Fig. 2 (A) Scleroderma patient specimen sections are stained with 4′,6-diamidino-2-phenylindole (DAPI), periostin and α-smooth muscle actin (α-SMA). Periostin showed diffuse positivity through the whole dermis. α-SMA expression is mainly located at pericyte and is not colocalized with periostin expression. (B) Control specimen sections are stained with DAPI, periostin and α-SMA. Periostin showed focal positivity along dermo-epidermal junction and perivascular area. α-SMA expression is mainly located at pericyte and is somewhat colocalized with perivascular periostin positivity.

• 

  Fig. 3 (A) Significantly higher proportion of excessive collagen bundle deposition (57.1% vs. 7.1%, p=0.013). (B) Diffuse periostin expression (42.9% vs. 0%, p=0.016) in the localized scleroderma specimens compared to controls. (C) Moderate matrix metalloproteinase (MMP)-1 expression (71.4% vs. 7.1%, p=0.001) in the localized scleroderma specimens compared to controls. *p<0.05, **p<0.01.

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