Table of Contents Author Guidelines Submit a Manuscript
Case Reports in Dermatological Medicine
Volume 2014 (2014), Article ID 418257, 3 pages
http://dx.doi.org/10.1155/2014/418257
Case Report

Circumscribed Morphea and Breast Asymmetry in an Adolescent

1Department of Dermatology, Centro Hospitalar de V.N.Gaia/Espinho, EPE, 4430 Vila Nova de Gaia, Portugal
2Department of Pathology, Centro Hospitalar de V.N.Gaia/Espinho, EPE, 4430 Vila Nova de Gaia, Portugal

Received 16 October 2013; Accepted 18 December 2013; Published 8 January 2014

Academic Editors: T. Hoashi, M. Jinnin, B. Kumar, N. Oiso, and G. E. Piérard

Copyright © 2014 António Augusto Fernandes Massa et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Morphea is a rare fibrosing disorder of the skin and underlying tissues. Circumscribed morphea presents with less than three discrete indurated plaques and breasts are commonly affected in women. We report the case of a 12-year-old female with a right infra-areolar, nontender, brownish patch and asymmetry of the right breast with 2 years of evolution. Skin biopsy showing thickening of the dermal collagen bundles confirmed the clinical diagnosis of morphea. After a 3-year follow-up period without progression of disease, reconstructive surgery is scheduled. Plaque morphea can involve all layers of the skin but associated breast deformity is rare. It can mimic benign and malignant breast disorders justifying the benefit for early tissue biopsy. Breast morphea generally has a good prognosis but hyperpigmentation and breast deformity in young girls have been rarely described. An early diagnosis can possibly lead to a therapeutic intervention with a different outcome, as it can be the source of severe psychological and social issues in a delicate period of development such as adolescence.

1. Introduction

Morphea is a rare fibrosing disorder of the skin and underlying tissues, with equal prevalence both in adults and children, female predominance, and greater prevalence in caucasians. Circumscribed morphea presents with less than three discrete indurated plaques, predominantly on the trunk, and it can be superficial or deep [13]. Breasts are commonly affected in women, uniformly sparing the nipples [4].

2. Case Presentation

We report the case of a 12-year-old female patient who was referred to our outpatient clinic for evaluation of a patch in her right breast with 2 years of evolution. Pruritus and pain were denied. No musculoskeletal, respiratory, gastrointestinal, neurologic, and vascular symptoms were present. Her past medical history was irrelevant except for asthma and there was no family history of autoimmune diseases.

At the physical exam, right breast asymmetry was noted, with an infra-areolar patch with 7 by 3 cm, with nontender, brownish, hyperpigmented, and hypopigmented areas (Figure 1). There were no other relevant cutaneous lesions.

418257.fig.001
Figure 1: Right breast asymmetry, infra-areolar patch, with 7 cm, with nontender, brownish, hyperpigmented, and hypopigmented areas.

A skin biopsy was performed, showing thickening of the collagen bundles in the dermis with a perivascular lymphohistiocytic infiltrate. No eccrine glands were present (Figure 2). These histologic findings confirmed the clinical diagnosis of morphea.

418257.fig.002
Figure 2: Haematoxylin and eosin—thickening of the dermal collagen bundles with a perivascular lymphohistiocytic infiltrate.

Screening for systemic involvement and for Borrelia burgdolferi, as well as for autoimmunity was negative, including antinuclear antibodies and extractable nuclear antigens.

Breast ultrasonography showed scarce mammary tissue at the right breast. Skin and subcutaneous tissue showed no alterations, suggesting breast hypoplasia.

Although a topical corticosteroid or a topical calcineurin inhibitor could be indicated, given the absence of symptoms, functional limitations, and tenderness, and with the parents’ acknowledgement, no therapeutic intervention was performed.

After a 3-year follow-up period without progression of disease, reconstructive surgery with breast augmentation is scheduled.

3. Discussion

Plaque Morphea can involve all layers of the skin, subcutaneous tissue, and underlying bone but associated breast deformity is rare.

Breast associated morphea can mimic benign and malignant inflammatory breast disorders, being misdiagnosed in two-thirds of the cases as inflammatory breast cancer or breast infections, justifying the benefit for early tissue biopsy in patients with unexplained breast erythema to confirm a clinical diagnosis and thus guide subsequent interventions [5].

While idiopathic morphea, all subtypes included, is an infrequent disease with a reported incidence of 2.7 per 100.000 in the United States [6, 7], breast associated morphea is a relatively common effect after radiation therapy for breast cancer, with an incidence as high as 1 in every 500 patients [8, 9]. It is usually confined to the radiated site but can also rarely occur distant to the radiation site [10].

Breast morphea generally has a good prognosis and did not result in significant disfiguration and morbidity [5, 11], but hyperpigmentation and breast deformity in young girls have been described [1215].

It should be thought when evaluating an adolescent for breast deformity [1215] and kept in mind as an early diagnosis that can possibly lead to a therapeutic intervention with a different outcome.

Physicians should be aware and recognize the signs of what could result in a major burden of disease, with severe adverse psychological and social implications in a delicate period of development such as adolescence.

Conflict of Interests

The authors declare that there is no conflict of interests regarding the publication of this paper.

References

  1. S. Christen-Zaech, M. D. Hakim, F. S. Afsar, and A. S. Paller, “Pediatric morphea (localized scleroderma): review of 136 patients,” Journal of the American Academy of Dermatology, vol. 59, no. 3, pp. 385–396, 2008. View at Publisher · View at Google Scholar · View at Scopus
  2. J. J. Leitenberger, R. L. Cayce, R. W. Haley, B. Adams-Huet, P. R. Bergstresser, and H. T. Jacobe, “Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases,” Archives of Dermatology, vol. 145, no. 5, pp. 545–550, 2009. View at Publisher · View at Google Scholar · View at Scopus
  3. A. V. Marzano, S. Menni, A. Parodi et al., “Localized scleroderma in adults and children. Clinical and laboratory investigations of 239 cases,” European Journal of Dermatology, vol. 13, no. 2, pp. 171–176, 2003. View at Google Scholar · View at Scopus
  4. N. Fett and V. P. Werth, “Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis,” Journal of the American Academy of Dermatology, vol. 64, no. 2, pp. 217–228, 2011. View at Publisher · View at Google Scholar · View at Scopus
  5. C. J. Clark and D. Wechter, “Morphea of the breast—an uncommon cause of breast erythema,” The American Journal of Surgery, vol. 200, no. 1, pp. 173–176, 2010. View at Publisher · View at Google Scholar · View at Scopus
  6. L. S. Peterson, A. M. Nelson, W. P. D. Su, T. Mason, W. M. O'Fallon, and S. E. Gabriel, “The epidemiology of morphea (localized scleroderma) in Olmsted County 1960–1993,” Journal of Rheumatology, vol. 24, no. 1, pp. 73–80, 1997. View at Google Scholar · View at Scopus
  7. K. J. Murray and R. M. Laxer, “Scleroderma in children and adolescents,” Rheumatic Disease Clinics of North America, vol. 28, no. 3, pp. 603–624, 2002. View at Publisher · View at Google Scholar · View at Scopus
  8. D. A. Davis, P. R. Cohen, M. D. McNeese, and M. Duvic, “Localized scleroderma in breast cancer patients treated with supervoltage external beam radiation: radiation port scleroderma,” Journal of the American Academy of Dermatology, vol. 35, no. 6, pp. 923–927, 1996. View at Publisher · View at Google Scholar · View at Scopus
  9. N. R. Bleasel, K. M. Stapleton, C. Commens, and V. A. Ahern, “Radiation-induced localized scleroderma in breast cancer patients,” Australasian Journal of Dermatology, vol. 40, no. 2, pp. 99–102, 1999. View at Publisher · View at Google Scholar · View at Scopus
  10. J. Kushi and M. E. Csuka, “Generalized morphea after breast cancer radiation therapy,” Case Reports in Rheumatology, vol. 2011, Article ID 951948, 9 pages, 2011. View at Publisher · View at Google Scholar
  11. G. Shetty, F. Lewis, and S. Thrush, “Morphea of the breast: case reports and review of literature,” Breast Journal, vol. 13, no. 3, pp. 302–304, 2007. View at Publisher · View at Google Scholar · View at Scopus
  12. T. Eidlitz-Markus, M. Mukamel, Y. Haimi-Cohen, J. Amir, and A. Zeharia, “Breast asymmetry during adolescence: physiologic and non-physiologic causes,” Israel Medical Association Journal, vol. 12, no. 4, pp. 203–206, 2010. View at Google Scholar · View at Scopus
  13. E. S. Treiber, N. S. Goldberg, and H. Levy, “Breast deformity produced by morphea in a young girl,” Cutis, vol. 54, no. 4, pp. 267–268, 1994. View at Google Scholar · View at Scopus
  14. S. A. Slavin and S. Gupta, “Reconstruction of scleroderma of the breast,” Plastic and Reconstructive Surgery, vol. 99, no. 6, pp. 1736–1741, 1997. View at Google Scholar · View at Scopus
  15. J. M. Weinberg, M. Russo, R. J. Hirsch, and P. C. Don, “Morphoea of the breast in a young girl,” Clinical and Experimental Dermatology, vol. 26, no. 6, pp. 497–498, 2001. View at Publisher · View at Google Scholar · View at Scopus