INTRODUCTION

Bier spots are asymptomatic small, whitish irregularly shaped macules on a background of diffuse erythema, which is tought to be an exaggerated but normal physiological vasoconstrictive response of small vessels to venous congestion [1–7]. It usually affects young adults, more commonly women than men [1,4] and appears to be seen worldwide, in that no racial or ethnic predominance has been reported [4]. Here, we report a 32-year-old male patient with typical clinical presentations of Bier spots.

CASE REPORT

A 32-year-old male patient came to our outpatient clinic with a history of six months of white spots appearing on distal parts of his limbs. His past medical and family history were unremarkable. Systemic examination of the patient was insignificant. Upon dermatological examination on the distal portions of the extremities, we observed multiple whitish macules, several milimeters in size, intermingling with erythrocyanosis of the affected part (Figs 1 – 4). Both flexor and extansor surfaces were affected and the lesions were more pronounced on upper extremities. Lesions became indistinguishable on diascopy (Fig. 5 while dermoscopy provided closer view of eythematous and adjacent blanched white areas (Fig. 6). In addition, when the patient raised his upper extremities above his head, lesions disappeared almost immediately (Fig. 7). Laboratory studies including complete blood count and differential, erythrocyte sedimentation rate, serum chemistry profile, thyroid panel, coagulation profile, urinalysis, C-reactive protein levels, complement levels, immunoglobulin levels and cryoglobulin levels were within normal limits. Anti-nuclear antibodies, antiphospholipid antibodies, rheumatoid factor, proteins C and S were negative. Bilateral upper and lower extremity arterial and venous doppler ultrasonography did not reveal any evidence of pathology. Based on history, clinical, laboratory and ultrasonography findings we made a diagnosis of spontaneous Bier spots and the patient taken under follow-up.

DISCUSSION

First described in 1898 by Bier [8], Bier spots are asymptomatic pale macules on a background of erythema [1,4,7,9–11]. The clinical presentation of Bier spots is unique with typical inducible and reproducible pale macules, several milimeters in size, scattered over dorsal surfaces of extremities in a speckled/mottled appearance. Typically, lesions are visible when the affected limb is put in a dependent position and disappear when the limb is raised. [5–7,9,10,12] Although Bier spots have been regarded to be a vascular anomaly with vasoconstriction in the paler areas, diascopy, which is an useful and simple technique to evaluate vasodilatation, may be also effective to confirm the diagnosis of Bier spots. Like nevus anemicus, when diascopy is performed, the lesions become indistinguishable from the surrounding skin, since the irregular borders of lesions blanches [4,7].

What are the causes and the reason why Bier spots develop have not been fully elucidated yet. However, a wide range of conditions have been reported to be associated with Bier spots, including pregnancy [13], scleroderma renal crisis [14], cryoglobulinaemia [15], aortic hypoplasia [16], coarctation of aorta [17], lower extremity lymphedema [9], Peutz-Jeghers syndrome, alopecia areata and lichen palnus [7,9]. Moreover, a rare syndrome, Marshall–White syndrome, was described in 1965, which includes Bier spots in conjunction with insomnia and tachycardia [18]. It has been suggested that the pathogenetic mechanism underlying Bier spots is exaggerated vasoconstrictive response within arterioles to tissue hypoxia [2,3,6,9] and causative factors leading to tissue hypoxia have been proposed to be hyperviscosity [15], venous [13] and lymphatic [9] hypertension. However, we think that it is unclear why some individuals develop Bier spots. It has to be clarified whether those aforementioned associations are coincidental or represent a cause and effect relationship or Bier spots are simply develop as a clinical manifestation of exaggerated physiologic vasoconstrictive response.

Here, we report a case of Bier spots in a 32-year-old male patient. We did not find any evidence of underlying conditions, which may cause Bier spots, thus we suggest that our patient represents a case of spontaneous Bier spots. However, as far as we know, Bier spots more commonly appear on the extansor surfaces than the flexor surfaces of the extremities [1,7,9,12] and involvement of palmoplantar area is occasional [4,5,15]. Therefore, since both flexor and extansor surfaces, also palmoplantar areas were involved in our patient, we think that our case is an example of noteworthy presentation of Bier spots. Moreover, to the best of our knowledge, we report herein the first case in the literature describing both the diascopic and dermoscopic examination of Bier spots.