A rare condition of scleroderma, known as keloidal or nodular scleroderma, characterized by the presence of rigid and well-defined nodules and plaques that look like overgrown scars (keloids), was reported in a case study published in the journal Case Reports in Dermatology.
Cases of nodular scleroderma were reported in patients with systemic sclerosis or localized scleroderma. It usually occurs in middle-aged women six months after the presentation of systemic sclerosis. But there is evidence that nodular scleroderma can also occur before the onset of systemic sclerosis.
The cause of nodular scleroderma is still unclear, with several proposed factors potentially playing a role, such as alteration in collagen matrix formation in the skin or pathogens as bacteria or virus.
In the case report “Scleroderma with Nodular Scleroderma,” the authors presented the case of a 50-year-old woman who was diagnosed one year earlier with systemic sclerosis and interstitial lung disease.
The patient had been prescribed several treatments to control her symptoms, including prednisolone, cyclophosphamide and aspirin. The immunosuppressant drugs she was taking were unable to prevent the occurrence of the skin lesions.
Ten months after her diagnosis, the patient started to show small, asymptomatic swellings on the neck, belly, and back. These skin lesions later increased in number and size, and some of them merged, forming plaques. These events could not be correlated with previous skin lesions due to surgical procedures or scars.
Additional physical examinations determined the patient had regions of skin on her face and trunk that were rigid and insensitive; extremities with reduced blood flow; and thickening and tightness of the skin on the fingers.
A biopsy of the skin lesions showed they were composed of thick rigid collagen fibers in the middle layers of the skin. This result was compatible with a scleroderma diagnosis.
So far, there is no specific or effective treatment for nodular scleroderma, making its treatment challenging. In this case report, the authors followed the patient’s progression for four months with no additional medication. Overall, the lesions did not show any progression.
“Although this condition is rare, it has been reported sporadically, and it should be considered in scleroderma patients clinically presenting with firm nodules or plaques,” the authors concluded.