Diffuse Bier white spots revealing cryoglobulinaemia
Authors: D. Bessis, O. Dereure, S. RiviEre, N. Rvi, A. Le Quellec, J-J. Guilhou
Journal: British Journal of Dermatology (2002)

Sir, Bier white spots are characterized clinically by asymptomatic white anaemic macules, gradually increasing in size, number and definition, standing out against a cyanotic background. This phenomenon is regarded as an exaggerated but physiological vasoconstrictive response induced by venous stasis‐associated hypoxia or, conversely, by a failure of the venoarteriolar reflex in dermal ascending arterioles in response to venous filling.1 However, no pathological condition underlying this cutaneous pattern has been mentioned previously in the literature. We hereby report the case of diffuse Bier white spots revealing the presence of a mixed cryoglobulinaemia in an elderly patient. A 75‐year‐old woman without relevant medical background was admitted for the evaluation of cutaneous lesions previously referred to as ′vitiligo', lasting for at least 4 years and progressively worsening. These lesions were permanent, were visible mainly when in a standing position and were more obvious with cold exposure. They involved the whole skin but relatively spared the head except for nonpermanent similar elements of the nose and ears. The patient denied any drug intake and did not express any functional complaint. Cutaneous examination while standing displayed multiple permanent white, ivory‐like spots standing out from an erythrocyanotic background ranging from several millimetres to 10 cm in size; these spots were either isolated or clustered, showing a linear or circular, roughly symmetrical pattern. They predominated on the trunk (Fig. 1), the proximal parts of the limbs, the palms and soles and also involved mucous areas such as the tip of the tongue and the middle part of the inner lower lip. The erythrocyanosis faded almost completely within minutes of lying down whereas discrete white spots persisted on the lower limbs around the knees and distal areas; all the cutaneous lesions quickly recurred within minutes of resuming a standing position (Fig. 2a,b). The clinical examination was otherwise unremarkable. Investigations including full blood count, C‐reactive protein, urea and electrolytes, liver function tests, and calcium were all normal. A type II mixed cryoglobulinaemia was present with a monoclonal component of the IgM kappa type together with polyclonal IgG. Serological tests for hepatitis C along with a search for circulating RNA were negative. Further explorations failed to detect circulating lupus anticoagulant, antiphospholipid and antinuclear autoantibodies or rheumatoid factor, whereas global and specific coagulation tests including cephalin‐activated time, serum rates of proteins C and S and search for Factor V Leiden revealed no abnormality. Bone marrow aspiration and biopsy were normal. Cutaneous biopsies performed on a white spot and erythrocyanosis were normal. Digital capillaroscopy displayed a hypoaemia associated with a vasospasm on several fingers, without clear vessel dystrophies. Computerized X‐scan of the chest, abdomen and pelvis, ultrasound of the abdomen and of the heart were normal. A therapeutic attempt with oral nifedipin was ineffective and was stopped after 1 month. The outcome was marked by the occurrence of a stroke 6 months later with residual hemiplegia.