Acral erythma as a manifestation of undifferentiated connective tissue disease

Abstract

Acral erythema (AE) is a swelling and numbness of the hands and feet that is observed in viral infections such as hepatitis C, HIV, and toxic drug induced skin reactions (chemotherapy). Patients with undifferentiated connective tissue disease (UCTD) may present with various symptoms. The most common symptoms at presentation include Raynaud's phenomenon (48-59%), arthralgia (37-81%), arthritis (22-71%), mucocutaneous symptoms such as photosensitivity, malar rash, alopecia and oral ulcerations (23-52%), fever (15-23%), sicca symptoms (12-42%) and central nervous system symptoms (8.5%). There is no literature available on patient presenting with AE as a symptom of connective tissue disease who are not on any treatment. In this particular patient icterus, multiple oral ulcers, bilateral pitting edema feet and reddish discoloration of palms and soles were present. Nervous system examination revealed weakness in muscles of shoulder and pelvic girdle (power grade 3/5). Laboratory investigations showed conjugated hyperbilirubinemia, raised liver enzymes and albuminuria. HIV and hepatitis C serology was negative. Electromyography showed mixed neurogenic and myogenic pathology. Autoantibody testing revealed that the patient was positive for the following: Antiribonucleoprotein (RNP) antibody, anti-Smith antibodies, anti-double-stranded DNA antibody, antihistone antibodies, antinucleosomal antibodies, antiribosomal P antibody thereby suggesting UCTD. This case is unique for the fact that AE was the sole cutaneous manifestation of connective tissue disorder without any history of drug intake.