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Vulvar Lymphangioma Circumscriptum Secondary to Crohn Disease

      A 56-year-old woman presented to the dermatologist for worsening vulvar papules and edema for 3 years. She had Crohn disease in remission status post colectomy with ileostomy and vaginal fistula repair. Prior nondiagnostic biopsies performed by the gynecology service showed squamous hyperplasia, lichenoid features, acute and chronic inflammation, and dermal fibrosis. The labia were markedly edematous, with clear to hemorrhagic papules and vesicles coalescing to a papillomatous plaque (Figure 1). These findings were suggestive of acquired lymphangioma circumscriptum (LC) secondary to Crohn disease. This diagnosis was confirmed by a skin biopsy, which showed cystically dilated vascular lymphatic spaces in the dermis (Figure 2). Clobetasol 0.05% cream daily, initiated by the gynecologist, resulted in significant improvement during several weeks (Supplemental Figure, available online at http://www.mayoclinicproceedings.org).
      Figure thumbnail gr1
      Figure 1Grouped pink and gray verrucous vesicles and papules on an erythematous base involving the bilateral vulva with marked edema.
      Figure thumbnail gr2
      Figure 2A vulvar biopsy specimen shows cystically dilated lymphatic spaces in the dermis with coagulated lymph and scattered red blood cells (hematoxylin-eosin stain, magnification ×100).
      Lymphangioma circumscriptum, a benign lymphatic malformation, is most commonly seen in children on the trunk and extremities.
      • Flanagan B.P.
      • Helwig E.B.
      Cutaneous lymphangioma.
      ,
      • Patel G.A.
      • Schwartz R.A.
      Cutaneous lymphangioma circumscriptum: frog spawn on the skin.
      It is less commonly acquired in Crohn disease or from radiation therapy for pelvic malignant disease.
      • Chang M.B.
      • Newman C.C.
      • Davis M.D.P.
      • Lehman J.S.
      Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: clinicopathologic study of 11 patients from a single institution and 67 from the literature.
      Acquired LC typically arises on the vulva as pink papillomatous or vesicular papules and plaques in the fifth decade.
      • Chang M.B.
      • Newman C.C.
      • Davis M.D.P.
      • Lehman J.S.
      Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: clinicopathologic study of 11 patients from a single institution and 67 from the literature.
      ,
      • Papalas J.A.
      • Robboy S.J.
      • Burchette J.L.
      • Foo W.C.
      • Selim M.A.
      Acquired vulvar lymphangioma circumscriptum: a comparison of 12 cases with Crohn’s associated lesions or radiation therapy induced tumors.
      Pruritus, oozing, and lymphedema are not uncommon.
      • Papalas J.A.
      • Robboy S.J.
      • Burchette J.L.
      • Foo W.C.
      • Selim M.A.
      Acquired vulvar lymphangioma circumscriptum: a comparison of 12 cases with Crohn’s associated lesions or radiation therapy induced tumors.
      Histopathologic evaluation, demonstrating dilated lymphatic channels in the papillary dermis with overlying epidermal hyperplasia, confirms the diagnosis.
      • Flanagan B.P.
      • Helwig E.B.
      Cutaneous lymphangioma.
      ,
      • Patel G.A.
      • Schwartz R.A.
      Cutaneous lymphangioma circumscriptum: frog spawn on the skin.
      Treatment options include laser therapy, cryotherapy, sclerotherapy, surgical excision, and labiectomy. The lesions tend to recur. Rarely, lymphangiosarcoma arising in long-standing LC has been described.
      • Papalas J.A.
      • Robboy S.J.
      • Burchette J.L.
      • Foo W.C.
      • Selim M.A.
      Acquired vulvar lymphangioma circumscriptum: a comparison of 12 cases with Crohn’s associated lesions or radiation therapy induced tumors.
      ,
      • King D.T.
      • Duffy D.M.
      • Hirose F.M.
      • Gurevitch A.W.
      Lymphangiosarcoma arising from lymphangioma circumscriptum.
      This case is unique because treatment with a superpotent topical steroid led to clinical improvement, although a multimodal treatment approach will likely be required. Recognizing vulvar LC early is important to establish the correct diagnosis and management.

      Supplemental Online Material

      Figure thumbnail figs1
      Supplemental FigureBefore treatment (A) compared with 3 weeks after daily topical clobetasol cream (B). Regression of erythema and skin lesions is seen.

      References

        • Flanagan B.P.
        • Helwig E.B.
        Cutaneous lymphangioma.
        Arch Dermatol. 1977; 113: 24-30
        • Patel G.A.
        • Schwartz R.A.
        Cutaneous lymphangioma circumscriptum: frog spawn on the skin.
        Int J Dermatol. 2009; 48: 1290-1295
        • Chang M.B.
        • Newman C.C.
        • Davis M.D.P.
        • Lehman J.S.
        Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: clinicopathologic study of 11 patients from a single institution and 67 from the literature.
        Int J Dermatol. 2016; 55: e482-e487
        • Papalas J.A.
        • Robboy S.J.
        • Burchette J.L.
        • Foo W.C.
        • Selim M.A.
        Acquired vulvar lymphangioma circumscriptum: a comparison of 12 cases with Crohn’s associated lesions or radiation therapy induced tumors.
        J Cutan Pathol. 2010; 37: 958-965
        • King D.T.
        • Duffy D.M.
        • Hirose F.M.
        • Gurevitch A.W.
        Lymphangiosarcoma arising from lymphangioma circumscriptum.
        Arch Dermatol. 1979; 115: 969-972