Primary cutaneous B-cell lymphoma of the leg in a chronic lymphedematous extremity

This is a case report of a woman who had chronic lymphedema on one leg and who developed a primary cutaneous large B-cell lymphoma of the leg at that site. She received radiotherapy and did not show any systemic involvement thereafter. Other neoplasms may appear in a clinical setting of chronic lymphedema, namely, lymphangiosarcoma (Stewart-Treves), melanoma, and metastatic carcinoma. There are four other reports in the English literature of cutaneous lymphoma arising in an extremity with chronic lymphedema.     

Angiosarcoma in chronic lymphedema

Angiosarcoma that develops on a limb with chronic lymphedema is called Stewart-Treves syndrome. This typically appears as a complication of a long course lymphedema located on the arm, after mastectomy and/or radiotherapy due to breast cancer. There are cases of Stewart-Treves syndrome in chronic lymphedema in the upper limb contralateral to the breast treated for cancer and in chronic lymphedema of the leg. We present two cases of this syndrome. The first corresponds to a typical syndrome of Stewart-Treves in an 83-year-old woman who was diagnosed of angiosarcoma in a chronic lymphedema territory secondary to mastectomy and radiotherapy due to breast cancer. The second case is much rarer, since it is a case of diffuse angiosarcoma of the leg in a 42-year-old man with a history of lymphedema. Due to the aggressive nature of this syndrome, knowledge and research on its treatment are necessary.     

Cutaneous epithelioid angiosarcoma: a neoplasm with potential pitfalls in diagnosis

Angiosarcoma (AS) is a rare neoplasm. Cutaneous AS is the most common form of AS. The epithelioid variant of the disease, however, is a rare entity. This subset can histologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. We present five patients with cutaneous epithelioid angiosarcoma (EAS); in none, the clinical diagnosis included a vascular lesion. Three patients had history of breast conservation surgery with/without radiation therapy. Other patients had no previous radiation, and there was no lymphedema in any of the cases. The histopathological examination of the biopsy specimens by hematoxylin and eosin method was not suggestive of a malignant vascular neoplasm initially and the differential diagnoses included carcinoma, malignant melanoma and atypical lymphoid infiltrate. Only after performing immunohistochemical studies that included vascular markers, a definitive diagnosis was possible. Some cases showed unusual histopathological features. Cutaneous EAS is a rare variant of cutaneous AS that can mimic a variety of more common, non-vascular neoplasms, creating a major pitfall in the diagnosis. A careful and thorough histopathological examination and a high index of suspicion, along with appropriate immunohistochemical evaluation, can help reach a correct diagnosis and provide optimal patient care.     

Acquired idiopathic penile lymphedema: Successful prevention of acute phase progression with low‐dose corticosteroids

Lymphedema is classified as a congenital disorder or acquired disorder. Common causes of acquired genital lymphedema include surgery, trauma, radiotherapy, neoplastic infiltration, venereal diseases and filariasis. Acquired idiopathic cases are also sometimes observed among genital lymphedema. There is no standard algorithm for the treatment of genital lymphedema. Surgical management for penile lymphedema in the chronic phase was discussed in many previous articles, however, management to prevent progression in the acute phase has been rarely mentioned. We present a case of acquired idiopathic penile lymphedema, in which histological examination was performed and acute phase progression was successfully ceased with low‐dose corticosteroids. This is the first report that proposes the usefulness of low‐dose systemic glucocorticoids for acquired idiopathic penile lymphedema showing rapid progression and histopathological lymphocytic inflammation.     

The lymphatic system and the skin. Classification, clinical aspects und histology

Dermatology offers many clinical examples of abnormalities in the production, absorption and transport of lymph. Lymphatic thromboses also occur. Many diseases are not named in accordance with their underlying pathogenesis. Malignant lymphedema must be recognized immediately. The differential diagnosis of regional lymphedema is also part of dermatology. Stemmer sign is a clue to chronic lymphedema. Lymphoedema is first divided into primary and secondary forms, depending on the cause, and then subdivided into stages depending on severity. Complications of chronic lymphedema include recurrent erysipelas, superficial lymphangiectases, verrucous lymphedema (papillomatosis lymphostatica) and angiosarcoma, which is often multifocal. Established diagnostic approaches are available to correctly diagnose lymphatic disease. The treatment of chronic lymphedema centers around reducing the edema which then influences all of the other problems. The physical therapeutic approach to reducing blockage is the best way to achieve these goals.     

Lymphedema of male external genitalia: a retrospective study of 33 cases

INTRODUCTION: The aim of this retrospective study was to describe the main characteristics and treatment of male external genitalia lymphedema. PATIENTS AND METHODS: From 1987 to 2003, all patients seen in a single hospital for lymphedema of male external genitalia were included. For each patient, the following characteristics were recorded: primary or secondary lymphedema, cause of secondary form, date of onset of lymphedema, associated lower limb lymphedema, clinical signs, and complications. In the primary forms, lower limb lymphoscintigraphy was performed. Specific surgery was proposed in all cases of symptomatic lymphedema (circumcision, scrotum and/or penile cutaneous excision). RESULTS: Thirty-three patients with lymphedema of external genitalia (17 primary, 16 secondary) were recruited. Two primary lymphedema were congenital, one isolated. Mean age +/- SD of the onset of the 15 other primary genital lymphedema was 23.4 +/- 17.5 years, always after the appearance of lower limb lymphedema. Sixteen men had secondary lymphedema (bladder, prostate, or rectum cancer, Hodgkin or non-Hodgkin lymphoma, aorto-bifemoral bypass grafting, biopsy or curretage of inguinal nodes). Secondary genitalia lymphedema was not associated with lower limb lymphedema in two cases and, in the others it occurred 66 +/- 122 months after (n=11), at the same time (n=2) or before lower limb lymphedema (n=1). Clinically, we noted genitalia heaviness (n=31), lower limb lymphedema (n=30), vaginal hydrocele (n=13), impaired miction due to prepucial swelling (n=10), leakage of lymphatic fluid (n=10). Lower limb lymphedema was complicated by at least one erysipelas (n=20), spreading to the external genitalia (n=4). In primary forms, lymphoscintigraphy showed ipsilateral hypoplasia of inguinal nodes in lower limb lymphedema (n=14) and/or external genitalia backflow (n=7). Surgical treatment was performed in 17 cases (11 primary, 6 secondary) with good results after 21 months' median follow up (1 month-10 years). Two patients died of cancer. One secondary lymphedema improved spontaneously and one disappeared after withdrawal of lower limb pneumatic compression. DISCUSSION: Lymphedema of external genitalia is responsible for discomfort and local complications. Surgical treatment is the main procedure of this disorder.     

Lymphgefäßsystem und Haut

Dermatology offers many clinical examples of abnormalities in the production, absorption and transport of lymph. Lymphatic thromboses also occur. Many diseases are not named in accordance with their underlying pathogenesis. Malignant lymphedema must be recognized immediately. The differential diagnosis of regional lymphedema is also part of dermatology. Stemmer sign is a clue to chronic lymphedema. Lymphoedema is first divided into primary and secondary forms, depending on the cause, and then subdivided into stages depending on severity. Complications of chronic lymphedema include recurrent erysipelas, superficial lymphangiectases, verrucous lymphedema (papillomatosis lymphostatica) and angiosarcoma, which is often multifocal. Established diagnostic approaches are available to correctly diagnose lymphatic disease. The treatment of chronic lymphedema centers around reducing the edema which then influences all of the other problems. The physical therapeutic approach to reducing blockage is the best way to achieve these goals.     

Lymphatic Compression by Sclerotic Patches of Morphea: An Original Mechanism of Lymphedema in a Child

Abstract:  Lymphedema in children is mostly primary, due to lymphatic hypoplasia. Secondary lymphedema is caused by lymphatic injury or obstruction. We report the case of a child that developed a lymphedema of the left upper and lower extremities, with a simultaneous onset of ipsilateral hemicorporal morphea. We concluded that lymphatic obstruction was due to sclerosis from morphea. This is a unique, rarely reported mechanism of lymphedema. Lymphoscintigraphy revealed attenuated lymphatic flow in the left upper and lower limbs. Systemic corticosteroids were associated with slow improvement in the sclerotic patches. We simultaneously noticed an improvement in the lymphedema of limbs. Repeat lymphoscintigraphy revealed dramatically improved lymphatic function. This case suggests that at least in some cases lymphedema may be caused by morphea.     

Vesiculobullous cutaneous lymphatic reflux

Although lymphedema is a fairly common condition, cutaneous vesicles and bullae due to reflux of lymphatic fluid are rare. We describe a patient with adenocarcinoma of the cervix treated by radiation in whom acute secondary lymphedema with overlying clear vesicles and bullae developed. Histopathologic examination revealed a subepidermal vesicle communicating directly with a dermal lymphatic vessel. Vesicles occur only in lymphedema of acute onset. Vesiculobullous cutaneous lymphatic reflux should be recognized as a rare complication of lymphedema and included in the differential diagnosis of cutaneous blistering disease.     

Congenital lymphedema and distichiasis

Distichiasis is defined as a double row of eyelashes. Inherited forms of distichiasis have been associated with early and late-onset congenital lymphedema. We report on a child with distichiasis and congenital lymphedema in which it is unknown whether the cause of lymphedema was due to a spontaneous mutation or acquired secondary to an intrauterine infection. Acquired forms of distichiasis are reviewed.     

Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature

Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with a high rate of local recurrence and low metastatic potential. We describe an unusual case of RH in a 45‐year‐old patient with Milroy disease, with a prominent solid component diffusely involving a chronic lymphedematous leg. This case is consistent with the postulated relationship between lymphedema and vascular neoplasms developing as a result of local immune dysfunction, and highlights the need to closely monitor patients with Milroy disease for pathologic changes. Our case highlights a unique example of RH with atypical features. There are several noteworthy unusual clinical and histologic findings including diffuse involvement of an entire limb, solid component with cytologic atypia, D2‐40 expression, and first‐time‐reported association with Milroy disease. Given the atypical histologic presentation of cytologic atypia, solid areas and atypical immunohistochemical profile with D2‐40 positivity, this case could cause diagnostic difficulty, especially in the setting of such a broad clinical differential.     

Squamous cell carcinoma of the penis and scrotum in a patient with chronic scrotal and penile lymphedema

Squamous cell carcinoma arising from tissue affected by chronic lymphedema is rare, though it is recognized that a variety of malignant tumors can arise in chronic congenital or acquired lymphedema. We describe, a case of scrotal and penile squamous cell carcinoma arising in a patient with a history of chronic scrotal and penile lymphedema of filarial origin. We here discuss the management and possible etiology of this unusual case.     

Identification and treatment of different types of lymphedema

PURPOSE: To provide the physician and registered professional nurse with an overview of the pathophysiology and the care of patients with lymphedema. TARGET AUDIENCE: This continuing education activity is intended for physicians and nurses with an interest in learning more about the different types of lymphedema and the appropriate treatment strategies. OBJECTIVES: After reading the article and taking the test, the participant should be able to: 1. Identify the types of lymphedema, their causes, and possible complications. 2. Describe the diagnostic criteria, preventive strategies, and treatment strategies for lymphedema.     

Verrucous and Elephantoid Lymphedema:

ABSTRACT: Five patients with varying severities of hyperkeratotic verrucous thickening at the skin illustrate that the condition may occasionally become elephantoid and occurs in some cases of long-standing lymphedema regardless of its cause. The morphologic term "elephantiasis" has been as sociated with the end of lymphedema, but his been used by different authors to mean different things and has, therefore, became imprecise. The noun "lymphedema" modified by the appropriate adjective is more accurate in describing these skin changes. Under the beading-"elephantiasis"–Index Medicus now states–"see lymphedema"     

Angiosarcoma of the abdominal wall after dermolipectomy in a morbidly obese man. A rare form of presentation of Stewart-Treves syndrome

Angiosarcoma is a rare malignant tumor, with a predilection for skin in the head and neck region, although it has been described in many other locations. Its association with chronic lymphedema is well known, mainly in the setting of postmastectomy lymphedema of the arm in breast cancer patients (termed Stewart-Treves syndrome). However, angiosarcoma can appear in lower limbs with chronic lymphedema and rarely in other locations such as the abdominal wall. Herein, we present a unique case of angiosarcoma developing in the abdominal wall of a morbidly obese patient after extensive dermolipectomy.     

Localized xanthomas associated with primary lymphedema

Cutaneous xanthomas arising in chronic lymphedema are rare. We present a case of verruciform xanthoma involving the left foot and toes of a 10-year-old boy who had developed a primary lymphedema (lymphedema praecox) in the left lower extremity. Laboratory studies demonstrated a normal lipid profile.     

Lipedema from the differential diagnostic and therapeutic viewpoint

Lipedema is a hereditary disease concerning exclusively women. We discuss the characteristics of diagnosis as well as the differential diagnosis between lipedema and primary lymphedema. Therapy is effective if the lipedema resembles a lymphedema.     

Early congenital lymphedema of the lower extremities combined with pachyonychia

A four-year-old girl with early congenital lymphedema of the lower limbs in combination with the nail plate involvement (pachyonychia) is described. Such a combination is not characteristic of early lymphedema, it is usually described in late edema of the lower limbs. The described case was a sporadic one in the family. It cannot be excluded that lymphedema development was induced by impaired embryogenesis of the lymph vessels resultant from sulphanilamides intake for severe bronchitis that proband's mother had suffered during pregnancy.     

Lymphedema after sentinel lymph node biopsy for cutaneous melanoma: a report of 5 cases

BACKGROUND: Sentinel lymph node (SLN) biopsy has rapidly become the procedure of choice for assessing the lymph node status of patients with 1992 American Joint Committee on Cancer stages I and II melanoma. The procedure was designed to be less invasive and, therefore, less likely to cause complications than a complete lymph node dissection. To our knowledge, this is the first report in the literature documenting extremity lymphedema following SLN biopsy. OBSERVATION: We report 5 cases of lymphedema after SLN biopsy in patients being routinely followed up after melanoma surgery at the Massachusetts General Hospital Melanoma Center, Boston. Three cases were mild, and 2 were moderate. Potential contributing causes of lymphedema were present in 4 patients and included the transient formation of hematomas and seromas, obesity, the possibility of occult metastatic melanoma, and the proximal extremity location of the primary melanoma excision. Four of the patients underwent an SLN biopsy at our institution. We used the total number of SLN procedures (N = 235) that we have performed to calculate a 1.7% baseline incidence of lymphedema after SLN biopsy. CONCLUSIONS: Sentinel lymph node biopsy can be complicated by mild and moderate degrees of lymphedema, with an incidence of at least 1.7%. Some patients may have contributing causes for lymphedema other than the SLN biopsy, but many of these causes are difficult to modify or avoid.