Cordoniform morphea: a clinicopathologic study of two cases presenting with the rope sign

Morphea clinically presenting as cordoniform lesions has not been described previously in the literature.

Our goal was to describe the clinicopathologic features of morphea presenting with cord-like cutaneous lesions.

The clinical notes of 420 patients with a diagnosis of morphea seen during the previous 10 years were reviewed to identify any cases that had cordoniform lesions at presentation.

Two adult patients (one male and one female) were identified. Both patients presented with chronic, slightly burning, bilateral, erythematous, linear or curvilinear elevated cutaneous indurations on the lateral chest wall strikingly reminiscent interstitial granulomatous dermatitis with arthritis. Histopathologically, typical changes of deep morphea with a band-like involvement only of the lower part of the reticular dermis and the superficial hypodermis and a remarkable perineural arrangement of the lymphoplasmocytic infiltrate were observed. The presence of Borrelia in skin biopsy samples of both patients was shown by immunohistochemistry and focus floating microscopy. In one patient, the presence of Borrelia afzelii DNA in the cutaneous biopsy was shown by polymerase chain reaction.

Cordoniform morphea is an exceedingly unusual and previously undescribed clinicopathologic presentation of morphea where Borrelia infection may play a causal role.