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Correspondence |

A Case of Lichen Sclerosus of the Scalp Associated With Autoantibodies to Extracellular Matrix Protein 1

Yoshio Kawakami, MD, PhD; Noritaka Oyama, MD, PhD; Yuka Hanami, MD; Tetsunori Kimura, MD; Kazuhiro Kishimoto, MD, PhD; Toshiyuki Yamamoto, MD, PhD
Arch Dermatol. 2009;145(12):1458-1460. doi:10.1001/archdermatol.2009.313.
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Lichen sclerosus (LS) is a chronic inflammatory skin disease of unknown cause with a predilection for the anogenital area. Recent investigation has identified serum IgG autoantibodies specific to extracellular matrix protein 1 (ECM1) in 74% of patients with anogenital LS1; however, the rationale for humoral autoimmunity to ECM1 in the extragenital disease has been enigmatic. Herein, we describe the first case to our knowledge of extragenital LS associated with IgG autoantibodies to ECM1.

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Figure 1. Clinical and histopathologic images from the subject case. A, The right occipital lesion with scarring alopecia accompanied by red violaceous papulonodules. B, Histologic analysis of a skin biopsy specimen shows hyperkeratosis, epidermal atrophy, edematous dermal papillae, diffuse hyalinization, and homogenized collagen bundles in the dermis (hematoxylin- eosin, original magnification ×25; scale bar indicates 500 μm). C, In addition to pigmentary incontinence, there is capillary dilatation and congestion just beneath the epidermis (hematoxylin-eosin, original magnification ×100; scale bar indicates 100 μm).

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Figure 2. Immunoblotting using a series of bacterially expressed recombinant extracellular matrix protein 1 (ECM1)–glutathione S-transferase fusion fragments. Three distinct recombinant proteins (ΔNH2, ΔS/ex7, and ΔDs/carboxylic acid [COOH]), which cover the entire ECM1 sequence, were immunoblotted independently with the panel of the serum samples, as detailed elsewhere.2 Samples of control genital lichen sclerosus (LS) serum (lane 1), the patient's serum at 1:10 (lane 2) and 1:100 dilutions (lane 3), and anti-ECM1 rabbit serum (lane 5)1 reacted specifically with the ΔDs/COOH recombinant, which is the distal COOH-terminus of ECM1 (amino acids 359-559),2 whereas a healthy volunteer's serum sample (lane 4) did not show any positive reactivity. The patient's serum never reacted with the remaining 2 ECM1 recombinants (data not shown). Arrow indicates the positive signals.

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