A Case Of Acquired Lymphangiectasia Of The Vulva And Literature Review

BackgroundAcquired lymphangiectasia of the vulva(ALV)is a rare entity that results from secondary dilation of lymphatic vessels.It is neither derived from neoplasm nor due to congenital superficial lymphatic malformation,which is usually secondary to tumor,Crohn disease,tuberculosis,recurrent cellulitis.The etiology and pathogenesis of the disease needs to be further investigated.It is difficult to diagnose clinically and thus,highlights the importance of biopsy to distinguish the disorder from the similar lesions such as condyloma acuminata and angiokeratomas.ALV is a benign condition with a good prognosis and lower risk of malignant progression.The nomenclature of the disease hasn't been definitely determined all over the world.ALV and acquired lymphangioma circumscriptum can both be seen in the literature.ObjectivesWe sought to illustrate clinical findings of a case of acquired lymphangiectasia of the vulva and retrospectively review the characteristics of the patients in the published literature,to facilitate understanding of the ALV for clinical doctors,and to reduce the possibility of inaccurate diagnosis,providing a support for further investigation.MethodsWe described the clinical characteristic of one case of ALV from our institution,and performed a comprehensive search to identify all published cases of ALV using CNKI,WANFANG MED ONLINE,VIP Web and PubMed databases.Clinical data of each case including general condition,clinical manifestation,history of past illness,presentation of histopathology and dermoscopy,treatments and prognosis were collected according to the rigorous inclusive criteria and exclusive criteria.Some clinical data(i.e.mean and standard deviation values)were calculated and analyzed by using SPSS21.0.ResultsWe reported a 55-year-old Chinese woman presented with 1-year history of multiple flesh and red vesicles on the swollen vulvas and the condition became more serious for 4 months.Systemic examination showed no abnormality.The patient was diagnosed with ALV after we re-checked the biopsy slide and the lesions were resolved following the CO2 laser ablation.The patient responded well with the treatment and showed no recurrence during 2-month follow-up.However,recurrence of small vesicles with pruritus bothered the patients again at a 9-month follow-up.124 cases of ALV patients were included and retrospectively reviewed in the recent 50 years from literature and among them,(1)age at onset ranged from 5 to78 years and mean age was 47.5±16.9 years.Women between 30-70 were more commonly affected than others.(2)The course of disease is variable between 0.2 and 32.3 years and less-than-5-year course accounts for 68.6%.(3)ALV may develop after malignant and benign tumors,recurrent infections(such as tuberculosis,cellulitis,erysipelas),Crohn disease,pregnancy,lymphedema of lower extremity,trauma,rare syndromes.(3)Typically,clinical manifestations include clusters of vesicles on the bilateral labia majora and flesh or dark-red papulovesicles,papules,and nodules with potential sensation of pain,pruritus,burning.Patients sometimes complained about no discomfort or periodically weeping discharge.(5)Histopathology examination reveals dilated thin-walled lymphatic channels with one layer of flat endothelial cells accompanied with D2-40/CD31 positive and CD34 negative pattern of immunohistochemistry staining.(6)Under a dermoscope it showed the view of white,red and dark lacunas,as well as double-colored lacunas("hypopyon-like sign")surrounded by white lines;occasionally,with dotted and several irregular vessels.(7)ALV was frequently misdiagnosed with condyloma acuminatum,angiokeratomas and molluscum contagiosum.(8)Currently,there is no universal standard therapy for ALV.Observation,surgery,laser,photodynamic therapy,cryotherapy,radiotherapy,sclerotherapy can be attempted as the treatments of ALV.Short-term results can be satisfactory,but in the long run,there remains the possibility of recurrence.(9)ALV is usually with good prognosis and lower risk of malignant development.It might transform into squamous cell carcinoma(SCC)and lymphangiosarcoma.Conclusions1.ALV is a rare entity with low incidence rate that affects commonly middle-aged and elderly women between 30 and 70 years old.2.The etiology is unclear,malignant and benign tumors,recurrent infections such as tuberculosis,cellulitis,erysipelas,Crohn disease,pregnancy,lymphedema of lower extremity,trauma,rare syndromes may be implicated with ALV.3.Remarkable clinical manifestations reveal clusters of vesicles or flesh or dark-red papulovesicles,papules,and nodules on the bilateral labia majora.4.Histopathology examination shows multiple dilated lymphatic channels with D2-40/CD31 positive and CD34 negative pattern of immunohistochemistry staining.5.Currently,there is no universal standard treatment guideline.Observation,surgery,laser and etc.can be prescribed as treatments of ALV.Therapeutic outcome can be satisfactory during short-term follow-up,but in the long run,there remains the possibility of recurrence.6.ALV is usually with good prognosis and lower risk of malignant transformation.