Institut de Pathologie et de Génétique, Gosselies, Belgium.
To the Editor:
Cutaneous lymphangiectasias are vascular ectasias involving lymphatic vessels of the skin.1,2 These alterations of lymphatics arise typically in areas affected by obstruction or destruction of the lymphatic vascular network and may occasionally resemble lymphangioma circumscriptum (LC).1,2 They often occur after radical surgery and/or radiation therapy for malignant tumors.1 Other causes such as infections, drugs, Crohn disease, pregnancy, or scleroderma have also been reported.1,3 Herein, we describe 2 patients who developed such vascular abnormalities as a complication of severe lichen sclerosus (LS), a finding rarely reported to our knowledge.
The first patient is a 22-year-old white male with no previous medical history. He was admitted in the Department of Urology because of chronic urinary obstruction. Clinical examination demonstrated several whitish corrugated papules located on the foreskin, frenum, and urethral meatus. In addition, a stricture of the distal urethra was demonstrated by cystography. According to the patient, these genital lesions spontaneously appeared a few months earlier and progressively increased in size, leading to urinary problems. Urethrostomy and biopsy of the prepuce were performed. Microscopic examination disclosed markedly dilated lymphatic vessels beneath the epidermis, like LC (Fig. 1). These ectatic lymphatics were filled with proteinaceous fluid and did not contain red blood cells. The overlying epidermis was irregularly acanthotic and hyperkeratotic (Fig. 1A). Besides lymphangiectasias, the papillary dermis displayed a continuous, band-like lymphocytic infiltrate that occasionally involved the basement membrane and the basal cell layer of the epidermis (Fig. 1B). Vacuolar alteration of basal keratinocytes and mild spongiosis were also found. The endothelial cells of such lymphatics expressed CD31 and CD34 antigens and podoplanin, a marker of lymphatic endothelium (Fig. 1C). Interestingly, lymphatics and blood vessels within the deep dermis were normal.
The second patient is a 64-year-old white female with a long history of LS involving both the oral cavity and the vulva. Recently, she noticed granular changes within the clitoris area. A small biopsy was performed to rule out a neoplastic transformation. Microscopic examination revealed advanced LS with epidermal atrophy, hyperkeratosis, and extensive dermal hyalinization. Superficial lymphangiectasias like those seen in patient 1 were also found (Fig. 1D). Similarly, the endothelium of these ectatic vessels was immunoreactive for CD31 and CD34 antigens and for podoplanin.
LS is a chronic inflammatory disorder that mostly affects genital skin.4-7 Involvement of extragenital areas is less frequent and usually coexists with genital lesions.4-7 LS usually appears as whitish and/or erythematous plaques or papules, located on genital skin.4-7 In men, this scleroatrophic process may involve the glans penis, prepuce, and distal urethra, which eventually leads to phimosis or urethral stricture.7 In women, LS lesions are usually found in labial, perineal, and anal areas.4-7 Occasionally, these sclerotic changes may extend to the thighs, mons pubis, or back.4-7 Patients with long-standing LS have an increased risk for the development of squamous cell carcinoma.4-7
Histological features of LS are relatively characteristic, especially in established lesions.4-7 In early LS, however, morphological changes are more subtle and may cause diagnostic problems because of the resemblance with other inflammatory dermatoses such as lichen planus or spongiotic dermatitis (eczema).4-6 In end-stage LS, the epidermis is variably atrophic and hyperkeratotic.4-7 The upper dermis is characteristically homogenized or hyalinized and contains a scarce lymphocytic infiltrate.4-7 Dermal blood vessels, especially those located in dermal papillae, beneath the epidermis, may be dilated to some extent.6 However, large vascular ectasias like those seen in angiokeratoma are distinctly uncommon.8
According to our literature review, lymphangiectasias resembling LC have been rarely reported in LS.7 Notably, papillomatous changes of the penile skin have been described in a 71-year-old patient with lymphedema of legs and scrotum due to LS of the penis and scrotum.9 Similar changes have also been reported in very few patients with scleroderma, another sclerotic disorder of the skin possibly related to LS.3,7 Apparently, no other study reports the occurrence of such lymphatic abnormalities in LS, which suggests either a neglected finding or a rare event.
Such lymphangiectasias should be distinguished from bullous LS, a peculiar variant of LS characterized by a prominent subepidermal edema, and blistering disorders occurring in the setting of LS.7,10
In the first patient of this study, histological features indicative of LS are relatively subtle and could be easily overlooked, if attention is paid to ectatic vessels only. Therefore, it is possible that some cases of acquired angiokeratoma or LC involving genital areas may actually represent vascular ectasias developed in poorly apparent or localized LS. Interestingly, a nondescript chronic inflammatory infiltrate is often reported in these vascular lesions.2 Whether such inflammatory component is part of an underlying but poorly apparent LS remains however to be demonstrated.
In summary, the present study illustrates that LS may seriously impair the lymphatic vascular network in some patients. Because morphologic features suggestive of this sclerotic condition may be relatively subtle, histological changes that are indicative of LS should be carefully sought in case of acquired LC, especially if the lesion is located in genital area.
Jean-Louis Dargent, MD
Institut de Pathologie et de Génétique, Gosselies, Belgium
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6. Regauer S, Liegl B, Reich O. Early vulvar lichen sclerosus: a histopathological challenge. Histopathology. 2005;47:340-347.
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8. Luzar B, Neil SM, Calonje E. Angiokeratoma-like changes in extragenital and genital lichen sclerosus. J Cutan Pathol. 2009;36:540-542.
9. Wille S, Niesel T, Breul J, et al. Elephantiasis of the legs with lichen sclerosus et atrophicus of the penis and scrotum. J Urol. 1997;157:2262.
10. Leonard N, Biswas A, Hickey S, et al. Eosinophilic spongiosis in a case of lichen sclerosus: a marker for superimposed bullous pemphigoid? Histopathology. 2008;52:885-887.
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