SIR, We report the case of a 58-yr-old female who complained of increasing tightness and tingling pain in the hands and face for several weeks. The patient had been well until 3 months previously when she developed flu-like symptoms followed by fatigue, fever and puffiness of the hands. She also noted that blanching with subsequent cyanosis and redness of fingers became evident on exposure to cold. In addition, dry eyes and mouth were also recognized.
One week later, she developed bilateral leg oedema and low back pain along with arthralgia of the knees and ankles. She also felt that the skin over her neck and shoulders became tighter and thicker. The possibility of progressive systemic sclerosis (PSS) was raised and she was admitted for further evaluation.
The family history revealed that her second son had Reiter's syndrome and her third son had ankylosing spondylitis. Both sons were positive for human leucocyte antigen (HLA) B27 molecule.
The important laboratory findings were as follows: erythrocyte sedimentation rate (ESR) 17 mm/h, antinuclear antibodies (ANA) titre 1:1280 (speckled and nucleolar pattern), complement C3 51.5 mg/dl (normal 90–180 mg/dl), complement C4 <5.42 mg/dl (normal 10–40 mg/dl), HLA-B27 positive. Tests for haemoglobin, leucocyte count, C-reactive protein, anti-extractable nuclear antigen (ENA) antibodies, anticardiolipin antibodies, lupus anticoagulants, rheumatoid factor and cytoplasmic and perinuclear types of antineutrophil cytoplasmic antibodies (ANCA) were all normal or negative. IgM and IgG antibodies to parvovirus B19 and Epstein–Barr viral capsid antigen were all positive.
Imaging studies showed mild right sacroiliitis and C3–6 herniated intervertebral discs. Lesional skin biopsy of the wrist was performed for histology and characterization of B19 virus by DNA in situ hybridization was positive. The microscopic appearance of the sclerotic skin lesion is shown in Fig. 1.
