Managing lymphoedema

Lymphoedema is perceived by clinicians in the UK as a rare condition for which there is no effective treatment. I would like to argue that lymphoedema is in fact a common condition which is misunderstood and often poorly managed. To explain further the basic physiology of oedema has to be considered.     

Hand dermatitis and lymphoedema

Hand dermatitis is common, with lymphoedema of the hand and forearm a rare complication. The mechanism of lymphoedema in such cases is poorly understood, hence management can be challenging. To investigate the underlying mechanism of lymphoedema associated with hand dermatitis and outline recommendations for management, we identified all patients with lymphoedema associated with hand dermatitis referred to our lymphoedema service, a tertiary referral centre. Treatment outcome was assessed by telephone interview and through correspondence with primary physicians and therapists. In total, nine patients, six with bilateral and three with unilateral lymphoedema associated with hand dermatitis, attended our service over a 4-year period. Most had long-standing bilateral pompholyx eczema. Three patients reported no signs of infection prior to the onset of swelling. All patients had recurrent episodes of infection after the onset of swelling. Lymphoscintigraphy, when used, revealed a failure of small initial lymphatics of the hand to absorb and drain lymph to regional nodes. Prophylactic antibiotics together with aggressive management of the dermatitis, often with systemic therapy, were required to reduce swelling. Possible mechanisms for lymphoedema associated with hand dermatitis include obliterative lymphangitis from infection, an inflammatory effect of the dermatitis on local lymphatics or a constitutive weakness of lymph drainage exposed to chronic inflammation, or any combination of the three. Treatment is only successful once both infection and inflammation from the dermatitis are controlled.     

Chronic lymphoedema and angiosarcoma

Angiosarcoma has frequently been described arising within chronic lymphoedema of the upper limb following mastectomy and radiotherapy for carcinoma of the breast. We report a case of angiosarcoma arising in a lymphoedematous leg that had been subjected to radiotherapy 20 years previously for Hodgkin's disease. The diagnosis was expedited once the patient noticed the development of bleeding nodules. Prognosis of angiosarcoma is poor with treatment options being wide-excision surgery, palliative radiotherapy or chemotherapy. Unusual bruised areas or bleeding nodules developing within chronic lymphoedematous limbs should be biopsied to exclude the diagnosis.     

Porokeratosis in association with lymphoedema

We describe four patients in whom porokeratosis coexisted with lympoedema of the legs. A possible pathogenetic link between the two disorders is discussed, as well as the therapeutic implications and the novel physical sign of lymphoedema bulging through the porokeratotic lesions.     

Lichenoid and granulomatous dermatitis

BACKGROUND: The prototypic lichenoid eruptions, lichen planus (LP), lichenoid drug eruptions, secondary syphilis, and collagen vascular disease, are defined histologically by a band-like lymphocytic infiltrate in close apposition to the epidermis. We describe a novel form of lichenoid dermatitis with a granulomatous component. DESIGN: Skin biopsies from 40 patients demonstrating a band-like lymphocytic infiltrate with concomitant granulomatous inflammation were encountered over 4 years. Clinicians were contacted to elucidate underlying triggers and medical illnesses. RESULTS: A lichenoid dermatitis, a linear eruption, vasculitis, annular erythema, and erythroderma were among the clinical presentations. A drug-based etiology was implicated in 14 cases: the drugs included antibiotics, lipid-lowering agents, anti-inflammatory drugs, antihistamines, hydroxychloroquine sulfate, and angiotensin-converting enzyme inhibitors. Over one-third of patients with drug-related eruptions had other medical illnesses associated with cutaneous granulomatous inflammation, namely rheumatoid arthritis (RA), Crohn's disease, hepatitis C, diabetes mellitus, and thyroiditis. A microbial trigger was implicated in 12 patients in the context of infective id reactions to herpes zoster, Epstein-Barr virus (EBV), or streptococci, or active infections by Mycobacterium tuberculosis, M. leprae, fungi, and spirochetes. The remainder had hepatobiliary disease and RA without obvious exogenous triggers, cutaneous T-cell lymphoma (CTCL), and idiopathic lichenoid eruptions (i.e. LP, lichen nitidus, and lichen striatus). One patient with LP had underlying multicentric reticulohistiocytosis. The histiocytic infiltrate assumed one or more of five light microscopic patterns: (i) superficially disposed loose histiocytic aggregates; (ii) cohesive granulomata within zones of band-like lymphocytic infiltration with or without deeper dermal extension; (iii) a diffuse interstitial pattern; (iv) scattered singly disposed giant cells; and (v) granulomatous vasculitis. Additional features included lymphocytic eccrine hidradenitis in those patients with drug reactions, hepatobiliary disease, and antecedent viral illnesses, tissue eosinophilia and erythrocyte extravasation in drug hypersensitivity, granulomatous vasculitis in patients with microbial triggers, drug hypersensitivity or RA, and lymphoid atypia in lesions of CTCL or drug hypersensitivity. CONCLUSIONS: The cutaneous lichenoid and granulomatous reaction may reflect hepatobiliary disease, endocrinopathy, RA, Crohn's disease, infection, or a drug reaction. One-fifth of cases represent idiopathic lichenoid disorders. Lymphoproliferative disease or pseudolymphomatous drug reactions must be considered in those cases showing lymphoid atypia.     

Lymphocoele and localized lymphoedema of the penis.

Six patients with lymphocoele or sclerosing lymphangitis of the penis attended the Department of Venereology, Royal Infirmary, Edinburgh, during a 9-month period. Clinical details of these patients are given and the aetiology of the condition is discussed.     

Primary upper‐limb lymphoedema

Summary Background  Lymphoedema is a general term used to designate pathological, regional accumulation of protein‐rich fluid. It can be either primary or secondary, and mainly occurs after cancer treatment. Objectives  To analyse the clinical and lymphoscintigraphic characteristics of primary upper‐limb lymphoedema (ULL). Methods  All of the patients with ULL were recruited at a single Department of Lymphology between January 2007 and December 2011. Results  In total, 60 patients (33 female, 27 male) were enrolled. For the 54 noncongenital lymphoedemas, the mean age at onset was 38·5 (range 3–82) years. Lymphoedema was unilateral in 51 patients (85%). It always affected the hand, and less often the forearm (55%) or upper arm (23%). Eleven patients (18%) developed cellulitis after onset of lymphoedema, and 21 patients (35%) had associated lower‐limb lymphoedema (LLL). Forty‐six patients (with 49 lymphoedematous limbs) underwent lymphoscintigraphy: axillary lymph node uptake was diminished in 18 (37%), absent in 24 (49%) and normal in seven limbs (14%). Among the 43 patients with unilateral lymphoedema and lymphoscintigraphy, 28 had epitrochlear node visualization, suggesting a rerouting through the deep lymphatic system, with 15 only on the lymphoedematous limb and 22 on the contralateral nonlymphoedematous limb. The median follow‐up period was 103 months, and 57/60 patients (95%) considered their lymphoedema to be stable. Conclusions  Primary ULL appears later in life than LLL, without predominance in either sex. Infectious complications are rare and patients considered the lymphoedema volume stable throughout life.     

Primary cutaneous plasmacytoma on chronic lymphoedema

An unusual case of cutaneous plasmacytoma with multiple lesions in a 77-year-old man affected by chronic lymphoedema of his lower limbs is reported. Histopathology and immunohistochemical staining led to the diagnosis of cutaneous plasmacytoma. Staging studies were not able to identify extracutaneous involvement. The lesions were successfully treated with melphalan and prednisone. No signs of progression of the disease were seen at a 2- year follow-up.