Correspondence Address:
Ahmet Demir
Dermatology and Venereology, Faculty of Medicine, Akdeniz University, Antalya
Turkey

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Sir,

A 49-year-old male patient presented to our outpatient clinic with a widespread rash on the back which began 6 months ago. The patient did not have any complaints like pruritus, pain or dysesthesia. He had no history of cardiovascular, rheumatological, hematological disorders or any liver disease. There was no family history of similar symptoms. Physical examination revealed erythematous macules and patches widespread on the back between T1-L4 dermatome in which superficial telangiectasias were observed in an asymmetrical distribution. [Figure 1] and [Figure 2]. In his dermoscopic examination, no additional features were observed except for telangiectasias. His detailed laboratory workup (complete blood count, comprehensive metabolic panel, thyroid function tests, testosterone, estrogen, luteinising hormone, follicle-stimulating hormone, and complement levels) were within the normal range. Screening tests (ANA, ANCA, RF) for rheumatological diseases and the serology for hepatitis and HIV were negative. A histopathological study of multiple biopsies taken from the lesions revealed multiple capillaries with increased diameter in the dermis [Figure 3]. He was diagnosed with bilateral nevoid telangiectasia (BNT) with clinical and histopathological findings.{Figure 1}{Figure 2}{Figure 3}

Before diagnosing BNT, hereditary haemorrhagic telangiectasia and generalised essential telangiectasia were excluded by the absence of epistaxis and family history, and the distribution and morphological features of the lesions. In all diseases mentioned above, histopathology is characterised by many thin-walled dilated vessels in the superficial dermis.[1] Furthermore, telangiectasia macularis eruptiva was excluded with the histopathology in which mast cells stained with toluidine blue were absent.

Intravascular lymphoma (IVL) is a rare and aggressive subtype of extranodal non-Hodgkin lymphoma. Selective growth of lymphoma cells is observed in the lumen of capillaries and small vessels. In approximately 40% of IVL cases, the skin lesions are tender and indurated, they can range from erythematous patches to nodules, usually distributed over the trunk and extremities which can be accompanied by edema. Due to the diversity in skin findings, many diseases are included in the differential diagnosis of IVL. Superficial telangiectasias covering indurated plaques have also been described in IVL.[2] In this respect, BNT can be included in the differential diagnosis with IVL. In our case, the patient's lesions were not infiltrated, and he did not have any symptoms related to a tumor burden. In addition, IVL is more common in older age (with mean age of 70), and no intravascular lymphoma cells were observed in our histopathological work up.[2]

Unilateral nevoid telangiectasia (UNT) is superficial telangiectasias observed in unilateral dermatomal localisation, usually in the upper part of the trunk and typically in the region of the C3-T4 dermatomes. The exact pathogenesis is unknown. However, it is assumed to be associated with increased estrogen levels, as it has a higher prevalence in puberty, pregnancy, and liver disease. Furthermore, there is an increased number of estrogen receptors in the dermatomal area where telangiectasias are located. However, since serum estrogen values were not studied in non-pregnant UNT cases, the relationship between estrogen and UNT has not been fully demonstrated.[1]

BNT is an extremely rare variant of UNT with a bilateral dermatomal distribution.[1] More than 100 cases of nevoid telangiectasia have been described so far and only a few of them had BNT.[3] In 2004, Tang et al.[4] reported the first case of BNT in a pregnant woman with gastrointestinal involvement, which completely regressed after delivery. Turan et al.[5] then reported a similar case in a male patient associated with gastrointestinal involvement, without a known cause. In the case series of Jee et al., two cases out of nine had liver disease and two others were in the postmenopausal period.[1] In 2020, Huang et al. reported a case of BNT with bier spots.[3] In our case, no specific etiological factor has been identified. [Table 1] summarises the general characteristics of previously reported BNT cases together with the current case.{Table 1}

Of the total 12 BNT cases reported in the literature, seven cases were male and five were female. The age distribution was between 27 and 65 years. Only four of the cases had an underlying pathological condition. Eleven out of the 12 cases had spider shape telangiectasias. Our patient's demographic and clinical characteristics were generally similar to those in the literature. The telangiectasias of all reported cases were located between C2 and T12 dermatomes. However, in our case, the telangiectasias were observed between T1-L4 dermatomes. This is the first reported case in which telangiectasias were also observed in the lumbosacral dermatomal area.

Although the number of BNT reported in the literature is extremely limited, it should be considered in the differential diagnosis of cases with specific clinical findings as described above and possible underlying diseases should be investigated.

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