Guttate morphea in a scleroderma spectrum disorder patient with anticentromere antibody

ARTICLE

Auteur(s) : Masayoshi Yamanaka, Osamu Ishikawa

Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan

Guttate morphea is a rare varient of localized scleroderma and accounts for only 0.13%-1.2% of patients with morphea [1, 2]. We herein present a case of guttate morphea in a scleroderma spectrum disorder (SSD) [3, 4] with ACA.

A 38-year-old Japanese woman presented with a 4-year history of erythematous skin lesions on her extremities. She had suffered from atopic dermatitis since the age of 15 and often scratched her skin. At the age of 34, she experienced asymptomatic small eruptions around the scratched areas on her extremities. In December 2007, she visited our hospital. Multiple, small (< 1 cm), reddish erythematous plaques were scattered on the extensor surfaces of the forearms and lower legs, and lesions on the lateral aspects of the thighs and buttocks (figure 1A). The central part of the plaques was slightly atrophic. Nail fold bleeding and periungal erythema were observed on her fingers (figure 1B), sclerodactyly was not present. There was no history of Raynaud’s phenomenon, nor systemic symptoms suggestive of SSc. Laboratory studies revealed a positive ANA (1:1280) and ACA (209.6 Index). Anti-topoisomerase I, anti-SSA, anti-SSB, anti-Sm, anti-RNP, anti-single-stranded-DNA, anti-double-stranded-DNA, antihistone and antimitochondrial antibodies were all negative. A skin biopsy specimen from a forearm lesion showed thickened collagen bundles in the dermis and moderate infiltrates of lymphocytes, histiocytes and plasma cells around blood vessels and appendages (figure 1C). Taken together, the diagnosis of guttate morphea in ACA-positive SSD was established.

The concept of SSD was proposed by Maricq et al. [3] to unify typical scleroderma, early forms of scleroderma and closely related disorders, including mixed connective tissue disease (MCTD). Our patient did not show sclerodactyly but nail fold bleeding in all fingers and positive-ACA. According to the diagnostic method of SSD by Ihn et al. [4], these findings indicate that she has high risk of future development to SSc and requires careful follow-up.

Localized scleroderma is considered to have an autoimmune background, because of a high frequency of autoantibodies [1, 2]. In general, anti-topoisomerase I antibody and ACA, which are representative autoantibodies for SSc, have been considered not to be detected in localized scleroderma. However, ACA-positive patients with localized scleroderma have been reported in recent years [5, 6]. Zulian et al. [1] analyzed 750 patients with juvenile localized scleroderma and found that ACA were positive in 2% of cases, respectively. Marzano et al. [2] also examined 239 patients with localized scleroderma (113 adults and 126 children) and reported that ACA were positive in 5% of adults but not in children. Although none of their patients developed SSc, four of the six ACA-positive patients had Raynaud’s phenomenone and capilloscopic changes. These patients may have SSD like our patient. Thus, ACA can be detected with a certain frequency in localized scleroderma.

Trauma is considered to be a possible trigger of morphea. Zulian et al. [1] reported that a history of local mechanical factors, including accidental trauma, insect bite and vaccination, were present in approximately 10% of patients. In our case, skin scratching seems to be a trigger of morphea. Recently, Ehara et al. [6] reported two patients who had morphea lesions in areas mechanically-compressed by underclothes. Interestingly, one patient had Raynaud’s phenomenon and positive detection of ACA. Certainly, trauma itself is associated with local inflammation and release of cytokines and growth factors, however, most traumas do not result in morphea. For developing trauma-induced morphea, some predisposition should be required. In patients with ACA, there may be an immunological predisposition to develop morphea. Further clinical studies are warranted to clarify the relationship between the development of morphea and ACA.

Acknowledgements

References

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4 Ihn H, Sato S, Tamaki T, et al. Clinical evaluation of scleroderma spectrum disorders using a points system. Arch Dermatol Res 1992; 284: 391-5.

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