Nodular morphea (NM): report of a case of concurrent NM and morphea profunda associated with limited type systemic sclerosis, and overview and definition for NM.

Morphea consists of various variants, including nodular morphea (NM), keloidal morphea (KM) and morphea profunda (MP). NM and KM are used synonymously. We present a 67-year-old Japanese female, the first case of limited type systemic sclerosis (SSc), developing both NM and MP. The NM and MP lesions on the lower legs were partly continuous, and showed histopathological features of morphea but not of keloid or scar. Pregabalin relieved severe lesional pain. Our literature review of all NM/KM cases indicated that some cases had merely keloid or scar, and, therefore, we first propose to abandon the term KM and use only the term NM for this condition to avoid confusion. We also propose making the diagnosis of NM only in cases showing histopathological features characteristic of SSc or morphea, which should facilitate the establishment of this entity. Our literature review also indicated that NM is closely associated with SSc, while the standard classification for morphea, which does not refer to NM/KM, probably due to ambiguity of this entity, suggests that morphea is rarely associated with SSc. Therefore, NM should be included as a distinct and unique subset in the classification of morphea when the disease entity of NM is established. Establishment of the disease entity of NM may change the general consensus of the rare association of morphea with SSc.