Morphea or localized scleroderma is characterized by thickening and hardening of skin and underlying tissues due to excessive collagen deposition by the activity of fibroblasts. In the United States 3 in one million individuals are affected by it. It is three times more common in females than in males and in Caucasians; however, it is equally prevalent in both children and adults.
What Is Morphea On Breast?
Localized morphea presents as one to several hard and thickened plaques, which are mostly superficial, but can be deep too. They are mostly present in the trunk area and breasts are commonly affected, in which nipples are uniformly spared. Breast associated morphea is commonly found in breast cancer irradiated patients and has an incidence of 1 in every 500. Although, morphea is mostly seen at the site of irradiation, but it is not uncommon for it to occur distant to the site of radiation.
Post irradiation fibrosis is more common that breast associated morphea. Fibrosis is a deep fascial and subcutaneous fibrosis, which may or may not be associated with inflammatory infiltrate. In contrast, radiation induced morphea is localized scleroderma that mostly affects the dermal layer caused by disturbance of the immune system, collagen metabolism and vascular system due to radiation therapy. Morphea consists of an inflammatory phase in the beginning, which subsequently leads to hardening and thickening of fibrotic tissue leading to pigmentation of the breast tissue.
There is no correlation between the ages of the patient, radiation dose or dose per fraction that pose as any risk factor for developing radiation induced morphea. However, systemic scleroderma is a potential risk factor that can lead to an exaggerated post irradiation fibrosis in patients with a history of systemic scleroderma. The pathogenesis behind radiation induced morphea is the formation of neoantigen due to tissue irritation by radiation therapy, which leads to transforming growth factor beta secretion. Transforming growth factor beta is associated with the activation of fibroblasts, which in turn synthesize collagen, thus fibrosis. Radiation induced morphea can be a slow occurring condition or a delayed response to irradiation, as there have been cases of radiation induced morphea as early as 1-12 months post irradiation and as delayed as 32 years after irradiation.
Morphea of the breast can mimic inflammatory breast disorders, both benign and malignant. About 75% of these cases have been misdiagnosed with inflammatory breast cancer or breast infections. So, if no predisposing factors are present, such as irradiation to the site, then it is reasonable to get an early tissue biopsy in patients with breast erythema with an unexplained cause. This can help reach a definitive diagnosis that will guide towards subsequent treatment options.
Although, breast associated morphea has a positive outcome and prognosis without any significant morbidity or limitation, hyperpigmentation and breast deformity has been seen in young girls. This breast deformity at such a tender age could have severe psychological and social impact. So, it is important for the health care providers to be aware of the condition and recognize it as early as possible, so as to reduce the trauma associated with it and treat it in its early stages.
There is no proven treatment for radiation induced morphea once it has progressed. However, various treatment options can be used, such as topical, intralesional or systemic steroids, colchicine, methotrexate, azathioprine, cyclophosphamide, cyclosporine, extracorporeal photopheresis, plasmapheresis and D-penicillamine with varying degree of benefit. PUVA is also used that has shown alleviation in itching, tightness, hardening and pigmentation of the affected skin by reduction in transforming growth factor that has a stimulatory effect on fibroblasts. It is important to not wait for the condition to subside on its own, especially in Young patients. The best outcome is achieved when treatment is started early as, although, PUVA is very effective, it does not reverse fibrosis and atrophy once morphea has progressed. In the patients, who have breast deformity, they can undergo reconstructive surgery that has provided very good cosmetic results.
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