If blue sclera is detected in patients, several possible causes should be considered. The Japanese doctor Dr has one cause. Yasuhiro Kano (Department of General Internal Medicine, Tokyo Metropolitan Tama Medical Center) in a 46-year-old woman.
The patient and her story
The 46-year-old woman presented because of increasing tiredness and shortness of breath for three months.She had a history of uterine fibroids with heavy menstrual bleeding.
The findings
The physical examination revealed a pallor of the face and conjunctival margin, blue sclera and koilonychia.
Laboratory tests showed haemoglobin of 4.0 g/dl (reference range 12-16), haematocrit of 16.7% (37-47), mean corpuscular volume of 54.8 fL (80-98), serum ferritin of 0.8 ng/ml (24-307) and transferrin saturation of 2.8% (20-50%).
Diagnosis and therapy
Based on the diagnosis Iron deficiency anaemia the patient received a blood transfusion and iron supplements. Because of her uterine fibroids, she was treated with oral gonadotropin-releasing hormone antagonists. Three months later, according to Yasuhiro Kano, her symptoms and physical findings, including the blue sclera, were gone; Hemoglobin and ferritin levels had normalized.
Discussion
Blue sclera is a common finding of iron deficiency, according to Kano, but is often overlooked.In 1908, Sir William Osler first described a blue discoloration of the sclera as a symptom of anemia in young girls. Almost 80 years later, British doctors in «Lancet» reported that blue sclera is more common in patients with iron deficiency anaemia (87%) than in patients with other types of anaemia (7%). In adult patients, blue sclera was reported to have a sensitivity of 87% to 89% and a specificity of 64% to 94% for iron deficiency anaemia and iron deficiency.
According to Kano, blue sclera also occur in other diseases vor e.g. rheumatoid arthritis, myasthenia gravis and long-term steroid therapy. They are particularly common in genetic diseases, especially in osteogenesis imperfecta.
A known cause is also a long-term treatment minocycline.Almost a year ago, for example, US dermatologists led by Professor Dr. Misha Rosenbach (University of Pennsylvania, Philadelphia) reported on such a case in the journal «JAMA» .
According to the authors, minocycline-induced hyperpigmentation can manifest as bluish pigmentation of the skin, that of the sclera, nails, teeth and oral mucosa. The reported incidence varies between 2% and 15% in patients treated for acne and between 41% and 54% in patients with rheumatoid arthritis. The mechanism of hyperpigmentation is unclear; According to Rosenbach and his colleagues, histopathological examinations indicate that minocycline and/or its metabolites are likely to form insoluble complexes with iron that are deposited in the tissues. The cutaneous hyperpigmentation caused by minocycline persists even after discontinuation of the drug; the therapy is difficult.
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