Abstract
A 74-year-old woman developed arthralgia and deformation of the fingers and the toes, reddish-purple discoloration of the face, Raynaud′s phenomenon, and positive antinuclear antibodies (ANA). She had been followed up in another clinic suspecting of connective tissue diseases (CTDs). She visited our hospital, desiring further examinations. Examination of the skin showed pernio-like plaques on the face and the hands. Skin biopsy specimens were taken from the plaques of the face and the hand. Hematoxylin-eosin staining specimens from both lesions showed non-caseating granulomas in the dermis. The chest roentgenogram and the computer tomography revealed a bilateral hilar and mediastinal lymphadenopathy. An ophthalmological examination disclosed a nodular lesion in the chamber angle. The tuberculin test was negative. The patient was diagnosed as lupus pernio. Serological examination revealed positive ANA with positive anticentromere antibodies suggesting the subclinical existence of CTDs, especially limited Systemic Sclerosis (lSSc). However, our case does not satisfy the criteria for lSSc.
