Cutaneous Lymphangiectasia

Cutaneous Lymphangiectasia Secondary To Lymph Node Tuberculosis

Riyaz Najeeba, Nair V Laxmi ,
Correspondence Address:Riyaz Najeeba

Abstract

Acquired cutaneous lymphangiectasia in two female patients with tuberculous lymphadenitis of the inguinal glands is described.

Keywords: Cutaneous lymphangiectasia, Tuberculous lymphadenitis

Introduction:

Cutaneous lymphangiectasia represents a rare cutaneous condition resulting from lymphatic obstruction caused by a spectrum of scarring processes. We report 2 such cases that occurred as a consequence of lymph node tuberculosis.

Case 1

A 38- year -old woman was seen with elephantiasis and multiple warty papules of the vulva of 4 years duration. The lesions occasionally ruptured and got infected. Past medical history was significant for bilateral inguinal swellings of 8 years duration. The inguinal swellings used to rupture and healed gradually with scarring in a period of 6 months. She also had irregular fever. There was no personal or family history of pulmonary tuberculosis or any history of genital ulcer.

The pubic region was oedematous and the labia majora were swollen and firm in consistency and studded with multiple papulovesicles [Figure - 1].

Inguinal region showed a few non tender, matted lymph nodes of size 2x1.5cm and scars on both sides. Systemic examination was normal.

The lymph node biopsy was consistent with tuberculous lymphadenitis and the biopsy of a cutaneous lesion showed dilated lymph spaces lined with endothelium. Other investigations were normal except for positive Mantoux text and raised ESR.

Inspite of adequate antituberculosis treatment the skin lesions persisted and hence she was sent for plastic surgical repair.

Case 2

A 65-year-old woman was seen with swelling of the external genitalia of 1 year duration and multiple vulva) papules and papulovesicles of 3 months duration. She gave history of (R) inguinal swelling 5 years ago which was incised. This persisted as a discharging sinus for a long time and gradually healed with scarring. Inguinal biopsy report was tuberculous adenitis and was put on antituberculosis drugs, which she took only for 2 months. After a year she developed similar lesions on the (L) inguinal region which also suppurated and led to discharging sinuses and gradually healed.

One year later she noticed elephantiasis of the vulva and gradual development of occasionally pruritic papulovesicles.There was no history of pulmonary tuberculosis in her or in other family members. She denied any history of genital ulcer.

Skin biopsy of the warty lesion showed hyperkeratosis and acanthosis. Multiple dilated lymph spaces were seen in the upper dermis.

Discussion

Majority of the cutaneous lymphangiomas are congenital in origin. Occasionally they arise from lymphatic obstruction due to malignancies,' radiation therapy,' cutaneous trauma' or secondary to infectious diseases like lymph node tuberculosis,[4] recurrent erysipelas, filariasis and lymphogranuloma venereum. Heuvel et al[4] described a case of vulval lymphangiectasia secondary to lymph node tuberculosis, exactly similar to our patients. Lymphangiecatses of the chest wall resulting from scarring scrofuloderma has been reported by Leonardo et al.[5] Lymphatic obstruction leads to dilatation of lymph channels in the superficial and middermis or even in the deep dermis or subcutaneous tissues. The overlying epidermis may show varying degrees of hyperkeratosis, acanthosis and papillomatosis and may appear to enclose the ectatic lymphatic channels.[6]

References

Weakly DR, Juhlin EA. Lymphangiectases and lymphangiomata. Arch Dermatol 1961; 84:574.
2.
Fisher I, Orkin M. Acquired lymphangioma (lymphangiectasis) report of a case. Arch Dermatol 1970; 101:230. [PUBMED]
3.
Zufall R. Lymphangiectasis of penis. Urology 19: 53, 1982; 19:53.
4.
Heuvel NVD, Stolz E, Notowicz A. Lymphangiectasias of the vulva in a patient with lymph node tuberculosis. Int J Dermatol 1979; 18:65-66.
5.
Leonardo MD, Jacoby PA. Acquired cutaneous lymphangiectasis secondary to scarring from scrofuloderma. J Am Acad Dermatol 1986;14: 688-689.
6.
Lever WF, Schaunburg Lever G. Histopathology of the Skin, Ed 6.Philadelphia 1983, JB Lippincott Co. PP. 631-633.

Indian Journal of Dermatology

* * * *
Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites.

Garcia-Doval I, de la Torre C, Losada A, Ocampo C, Rodriguez T, Cruces MJ.Service of Dermatology, Hospital Provincial de Pontevedra, Spain.
igarciad@meditex.es

BACKGROUND:
Lymphangiectasia results from acquired dilation of lymphatic vessels. Areas of skin affected by obstruction or destruction of lymphatic drainage are said to be prone to the development of lymphangiectasia. Cirrhosis is a cause of alterations of lymph flow.

METHODS:
Case report.

RESULTS:
We report a case of acquired, late-onset, lymphangiectasia associated with alcoholic hepatic cirrhosis. Lesions were scattered over the right, lower, anterior abdominal wall, a region that is drained by a common group of lymphatic vessels that were probably disordered.

CONCLUSION:
We think that this is the first reported case of lymphangiectasia associated with altered lymph flow in cirrhosis and ascites.

Publication Types:
Case ReportsPMID: 10568489 [PubMed - indexed for MEDLINE]

* * * *

Acquired lymphangiectasis after breast conservation treatment for breast cancer: Report of a case

Ismet Tasdelen1, Sehsuvar Gokgoz1, Ela Paksoy1, Omer Yerci2, Sibel Kahraman Cetintas3, Mutlu Demiray4, Veysi Samsa1Dermatology Online Journal 10 (1): 9 From the Departments of Surgery1, Pathology2, Radiation Oncology3, and Medical Oncology4, School of Medicine, University of Uludag, 16059, Bursa, Turkey.

sehsuvar@uludag.edu.tr

Abstract

Acquired lymphangiectasis is a dilatation of lymphatic vessels that can result as a complication of surgical intervention and radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histological features with the congenital lesion, lymphangioma circumscriptum. Diagnosis and treatment of these vesiculobullous lesions is important because they may be associated with pain, chronic drainage, and cellulitis. We describe patient who had these lesions after treatment for cancer. Although a number of treatment options are available, we have found cryosurgery and electrocautery to be particularly effective.

Introduction

Acquired lymphangiectasis (AL) represent superficial lymphatic dilatation caused by a wide range of scarring processes and occurs as a consequence of lymphatic damage by an external cause, leading to obstruction of local lymphatic drainage. It has been reported after treatment of breast, vulva, cervix, skin, and lung cancers and it is sometimes termed acquired lymphangiomas [1]. This condition has also been associated with metastatic lymph node invasion and obstruction, scleroderma, and scrofuloderma. Acquired lymphangiomas most commonly occur in adults as a late sequel of mastectomy and radiation therapy. Patients usually present with numerous translucent vesicles in a chronic lymphedematous area several years after surgery with or without radiation therapy [2]. This report describes a case of acquired lymphangiectasis of the breast in 68-year-old woman. We discuss the clinical features, diagnosis, and treatment of acquired lymphangiectasis of the breast with a literature review.

Clinical Summary

A 68-year-old woman presented to our outpatient clinic for evaluation of multiple small vesicular lesions on the left breast. Three years earlier, she had had a breast conservative treatment—lumpectomy, chemotherapy, and radiation therapy for grade II invasive lobular carcinoma of the left breast. The breast lesions started to develop three years after breast conservative treatment. The patient had not had any significant lymphedema. On physical examination, the patient had numerous isolated and translucent vesicles and bullae of the inferior left nipple (Figs.1, 2). They measure 2 to 3 mm in diameter. No lymphedema was present also. We performed biopsy from lesions that showed numerous dilated lymphatics, edema and mononuclear inflammatory cells throughout the papillary and reticular dermis (Fig. 3). With these clinical and histological findings the patient was diagnosed as cutaneous lymphangiectasis.

Some of the lesions were treated with cryosurgery and others were drained and then treated with electrocautery. Cryotherapy freezes these lesions with liquid nitrogen (-195.8°:). Cryotherapy was applied to the skin twice for 20 seconds. Daily wound care consisted of gentle cleansing with povidone iodine and application of mupirocin ointment. At 8 weeks, the wound had completely re-epitheliazed. The wounds healed without scarring and there was no evidence of recurrence in subsequent 2 months.

Discussion

Acquired lymphangiectasis is a condition in which dilated superficial lymphatics develop after damage to previously normal lymphatics. Acquired lymphangiectasis has been reported as clinically and histologically indistinguishable from congenital lymphangioma circumscriptum and is often reported in the literature as lymphangioma circumscriptum or acquired lymphangiomas [2, 3, 4, 5].

Cutaneous lymphangiectasis (CL) represents acquired vesicular dilatation of lymphatic channels secondary to an external cause with no evidence of true tumor formation. It has been associated mostly with the treatment of malignancies of breast and cervix and is often coexistent with lymphedema [1, 6, 7, 8]. There have also been reports of CL after treatment of myxoid chondrosarcoma and bronchial carcinoid [4, 9]. It has been documented after surgery alone, surgery and irradiation, and irradiation alone [8, 10]. Other reported cases without lymphedema include scarring from scrofuloderma, scleroderma, and cirrhosis [11, 12, 13].

Acquired lymphangiectasis has been reported in the literature with increased frequency in the past 20 years. This is most likely the result of increased surgical excision, surgery and radiation therapy for certain cancers. This condition has been documented to occur after surgical excision, surgery and irradiation and irradiation alone [3, 4, 10]. In our case, new lesions of lymphangiectasia ceased to develop after the conservative treatment. Although AL has been documented to occur after surgical intervention alone, we believe that x-ray therapy is a major contributor in its pathogenesis because the main site of injury of x-ray therapy is a major contributor in its pathogenesis, the site of injury from x-ray therapy is at the junction the fat and reticular dermis [8, 14]. This damage most likely causes fibrosis and lymphatic obstruction at the base of the reticular dermis and an accumulation of lymph fluid in the dermal lymphatics with resultant increased pressure [8]. This can then lead to sacular dilation of the superficial lymphatic channels with subsequent vesicle formation. The cutaneous lesions can range from clear, fluid-filled blisters to smooth, flesh-colored nodules, often appearing along an incisional scar. Coexisting lymphedema is present in most patients with acquired lymphangioma. Patients can present with localized wetness or copious drainage of clear or milky fluid from ruptured vesicles. Clinically, lymphangiectasia consists of several clusters of translucent, thick-walled, fluid-filled vesicles. The vesicles typically measure 2-10 mm in diameter. The affected area appears to be speckled by numerous translucent vesicles with normal-appearing skin among the lesions. Some lesions may become pedunculated with a hyperkeratotic verrucous surface mimicking a wart. The physical findings of our case were the patient had numerous isolated and translucent vesicles of the inferior left nipple area. They measure 2-3 mm in diameter. On microscopic examination, large, dilated lymph vessels lined by a single layer of endothelial cells characterize lymphangiectasis. They are usually found in the papillary dermis and the reticular dermis. Involvement in the deeper dermis is rare. At times, acquired lymphangiectasis grow above the level of adjacent skin. The histological findings of our case were consistent with these features, the numerous dilated lymphatics, edema and mononuclear inflammatory cells throughout the papillary and reticular dermis.

Complications of AL include leakage of the vesicles, recurrent cellulites, and pain [15, 16]. Acquired lymphangiectasis is not precancerous, although there is one reported case of a squamous cell carcinoma arising in lymphangioma circumscriptum [17]. This entity is different from the Stewart-Treves syndrome of lymphangiosarcoma developing in the setting of post-mastectomy lymphedema. Edematous and ecchymotic plaques progressing to red to purple nodules characterize lymphangiosarcoma [1].

Treatment for lymphangiectasis can be difficult, but it is important because of the risk that ruptured vesicles may provide a portal of entry for infection and subsequent cellulites. To prevent superinfection of ruptured vesicles, cleansing the affected area daily with topical antibacterial agents and applying mupirocin ointment or silver sulfadiazine cream are advisable. Many surgical treatment modalities have been advocated in the care of lymphangiectasis; these modalities include electrodesiccation, laser therapy, sclerotherapy, cryotherapy, and surgical excision. Daily compression through bandaging or hosiery in accessible areas has yielded acceptable results. In addition, laser therapy has also been shown to be an effective treatment. Various types of lasers have been used for this treatment, including the argon, tunable dye and CO&sub2; [1, 18, 19, 20]. There can be recurrences with all these treatments, and some may leave significant scarring. Our patient received treatment using the cryosurgery and some of them were drained and treated with electrocautery. Daily wound care consisted of gentle cleansing with povidone iodine and application of mupirocin ointment. At 8 weeks, the wound had completely re-epitheliazed. The wound was healed without any tissue scar and evidence of recurrence in following 2 months after re-epitheiazation.

Acquired lymphangiectasis is an important complication of radiation therapy and surgical intervention for malignancy and probably not as rare as once believed. Although this lesion has been described as histologically indistinguishable from lymphangioma circumscriptum, we believe these two conditions have true histologic differences in addition to their differing pathophysiology and clinical presentation. Acquired lymphangiectasis is an acquired dilation of lymphatics and can be associated with lymphedema after surgery and/or radiation therapy. Clinicians should be familiar with is presentation and pathogenesis since its complications can be significant. In addition, lesions are often cosmetically undesirable. The treatment is relatively simple, though recurrences are not uncommon.

References

Dermatology Online Journal

* * * *

Additional Resources

* * * *

Chylous reflux into abdominal skin simulating lymphangioma circumscriptum in a patient with primary intestinal lymphangiectasia.