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Case Report
Vulval lymphangiomata secondary to tubercular inguinal lymphadenitis with HIV P. Madavamurthy, Nadiga Rajashekhar, Chatura, Sugareddy, Prashanth Kumar Desai
Department of Dermatology, Venereology and Leprosy, J. J. M. Medical College, Davangere, Karnataka, India Correspondence: Dr. P. Madavamurthy, Department of Dermatology, Venereology and Leprosy, J. J. M. Medical College, Davangere-577 004, Karnataka, India. E-mail:
[email protected]
Abstract Acquisition of vulval lymphangiomata is a rare occurrence; clinically characterized by the presence of circumscribed eruptions of thin walled, translucent vesicles. Here, we report a case of 18-year-old HIV-positive female with multiple smooth to warty, flesh colored papules and nodules covering labia and mons pubis with bilateral inguinal linear puckered scars with enlarged right cervical group of lymph nodes. Our case is unique because of its association with HIV. Key words: HIV positive, tubercular inguinal lymphadenopathy, vulval lymphangiomata
Lymphangioma circumscriptum is a rare congenital malformation of the lymphatic system, characterized by persistent multiple clusters of translucent vesicles containing clear lymph fluid. Acquisition of vulval lymphangiomata is a rare occurrence; clinically characterized by the presence of circumscribed eruptions of thin walled, translucent vesicles. [1] However, epithelial changes and tissue organization may occasionally give rise to firmer nodules or even warty lesions, seen in association with scarring process due to recurrent infections, [2] radiotherapy, or surgeries.[3] We report such a case of vulval lymphangiomata due to tubercular inguinal lymphadenopathy in a HIV-positive patient.
CASE REPORT An 18-year-old female patient presented to our OPD with complaints of genital lesions since one year. The patient had a history of fever and cough along with multiple swellings and ulcerations over both the inguinal regions. On examination, smooth to warty, flesh colored papules, vesicles, and nodules covering labia majora and mons pubis were present [Figure 1]. Bilateral inguinal puckered scars were present. Right cervical group of lymph nodes were enlarged, tender,
and matted. Laboratory investigations showed routine blood and urine examinations within normal limits. HIV was reactive (by capillus, tridot, and Coombs methods), and Venereal Disease and Research Laboratory test as well as HbsAg were nonreactive. Chest X-ray showed resolving TB, FNAC of right cervical lymph nodes showed TB lymphadenitis, Mantoux showed strong positivity [Figure 2]. CD4 cell count was 515 cells/cumm. Histopathology showed acanthosis, papillomatosis, and hyperkeratosis of epidermis, dilated thin walled lymphatic channels in the superficial papillary dermis [Figure 3], dense infiltrate of lymphocytes and neutrophils in the dermis, and scattered neutrophils, plasma cells, and pigmented macrophages. The patient was initially treated with isoniazid 300 mg, rifampicin 450 mg, pyrazinamide 1500 mg, and ethambutol 800 mg for two months and subsequently with two drugs, isoniazid 300 mg and rifampicin 450 mg. The lesions regressed initially with one month treatment and thereafter remained static [Figure 4]. The patient was followed up for four months.
DISCUSSION Acquired or secondary lymphangioma arise following
How to cite this article: Madavamurthy P, Rajashekhar N, Sugareddy C, Desai PK. Vulval lymphangiomata secondary to tubercular inguinal lymphadenitis with HIV. Indian J Sex Transm Dis 2008;29:37-9.
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[Downloaded free from http://www.ijstd.org on Sunday, December 28, 2008] Madavamurthy, et al.: Vulval lymphangiomata
Figure 1: Multiple clusters of translucent vesicles containing clear lymph fluid on the labia majora and mons pubis
Figure 3: Dilated thin walled lymphatic channels in the superficial papillary dermis (H&E, x100)
Figure 2: Montoux test showing strong positivity
Figure 4: Regressed lesions with 1 month treatment
damage to previously normal deep lymphatic vessels.[4] In lymphangioma circumscriptum, dilated superficial lymphatics occur due to a congenital anomaly of the deep dermal and subcutaneous lymphatics. The pathogenesis of lymphangioma circumscriptum was studied by Whimster, who concluded that large lymphatic vessels running mainly vertically through the dermis transmit pressure from underlying abnormal cisterns that do not communicate with the deeper dermal lymphatics, and therefore form a closed system.[5] Lymphangiomas can be congenital or can be acquired from local factors, such as surgery, neoplasms (including lymphoma), radiotherapy, [3] erysipelas, lymphogranuloma venereum, tuberculosis, or filariasis. [6] They are most often observed in the areas of neck, upper trunk, tongue, and proximal extremities.[7] Lymphangioma of the vulva are also described following cancer treatment, tuberculous inguinal lymphadenopathy,[8] and genital involvement with Crohn’s disease. [9] Our case is
following tubercular inguinal lymphadenopathy in association with HIV. Histopathologically also, it satisfies required findings consistent with acquired lymphangioma. Other treatment modalities include radical surgery, intralesional administration of sclerosant, for example, doxycycline[10] or picibanil[11] (OK-432), simple electricautery, and vaporization with CO2 laser.[12] Recognition and appropriate treatment of vulval lymphangioma is important, primarily because the lesions may act as portals of entry for infections and secondarily to prevent complications. In addition, persistent leakage of lymphatic fluid, the cosmetic appearance, and the social repercussions of misdiagnosis as a STD may all pose significant problems.
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vulva in a patient with lymphnode tuberculosis. Int J Dermatol 1979;18:65-6. 9. Dap RF, van der Meijaden WI. Vulvar lymphangiectasia in Crohn’s disease. Ned Tijdschr Geneeskd 2000;144:1692-5. 10. Molitch HI, Unger EC, Witte CL, vanSonnenberg E. Percutaneous sclerothrapy of lymphangiomas. Radiology 1995;194:343-7. 11. Mikhail M, Kennedy R, Cramer B, Smith T. Sclerosing of recurrent lymphangioma using OK-432. J Pediater Surg 1995;30:1159-60. 12. Fliezzri YD, Sklar SA. Lymphangioma circumscriptum review and evaluation of CO2 laser vapourization. J Dermatol Surg Oncol 1988;141:357-36.
Source of Support: Nil, Conflict of Interest: None declared.
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