Biomaterial Database

Lymphedema secondary to limited cutaneous systemic sclerosis. 2018

Sónia Moreira, and Jorge Crespo, and Lèlita Santos

Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2 years asymmetric lymphedema in lower extremities. Compromise in lymphatic drainage was confirmed by lymphoscintigraphy. She also presented Raynaud's phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc. Treatment with methotrexate, prednisolone and lymphatic drainage resulted in lymphedema improvement. To our knowledge, this is the first case of grade 2 lymphedema in the setting of anticentromere-positive limited cutaneous SSc. We highlight the importance of considering rheumatic diseases in the differential diagnosis of lymphedema.

UI	MeSH Term	Description	Entries
D008209	Lymphedema	Edema due to obstruction of lymph vessels or disorders of the lymph nodes.	Milroy's Disease,Congenital Familial Lymphedema,Congenital Hereditary Lymphedema,Early Onset Lymphedema,Hereditary Lymphedema,Hereditary Lymphedema 1,Hereditary Lymphedema Type I,Lymphedema, Early-Onset,Lymphedema, Hereditary, Ia,Milroy Disease,Nonne-Milroy Disease,Nonne-Milroy Lymphedema,Nonne-Milroy-Meige Disease,Primary Congenital Lymphedema,Congenital Hereditary Lymphedemas,Congenital Lymphedema, Primary,Congenital Lymphedemas, Primary,Early Onset Lymphedemas,Early-Onset Lymphedema,Early-Onset Lymphedemas,Hereditary Lymphedema 1s,Hereditary Lymphedema, Congenital,Hereditary Lymphedemas,Hereditary Lymphedemas, Congenital,Lymphedema, Congenital Hereditary,Lymphedema, Early Onset,Lymphedema, Hereditary,Lymphedema, Nonne-Milroy,Lymphedema, Primary Congenital,Lymphedemas,Lymphedemas, Congenital Hereditary,Lymphedemas, Early Onset,Lymphedemas, Early-Onset,Lymphedemas, Hereditary,Lymphedemas, Primary Congenital,Milroys Disease,Nonne Milroy Disease,Nonne Milroy Lymphedema,Nonne Milroy Meige Disease,Primary Congenital Lymphedemas
D008727	Methotrexate	An antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of TETRAHYDROFOLATE DEHYDROGENASE and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.	Amethopterin,Methotrexate Hydrate,Methotrexate Sodium,Methotrexate, (D)-Isomer,Methotrexate, (DL)-Isomer,Methotrexate, Dicesium Salt,Methotrexate, Disodium Salt,Methotrexate, Sodium Salt,Mexate,Dicesium Salt Methotrexate,Hydrate, Methotrexate,Sodium, Methotrexate
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D011239	Prednisolone	A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states.	Di-Adreson-F,Predate,Predonine,Di Adreson F,DiAdresonF
D011928	Raynaud Disease	An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or stress.	Cold Fingers, Hereditary,Raynaud Phenomenon,Raynaud's Disease,Raynauds Disease
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D016896	Treatment Outcome	Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.	Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes
D045745	Scleroderma, Limited	The least progressive form of SYSTEMIC SCLERODERMA with skin thickening restricted to the face, neck and areas distal to the elbows and/or knees, sparing the trunk. The CREST SYNDROME is a form of limited scleroderma.	Limited Scleroderma,Limited Systemic Scleroderma,Limited Systemic Sclerodermas,Scleroderma, Limited Systemic,Sclerodermas, Limited Systemic,Systemic Scleroderma, Limited,Systemic Sclerodermas, Limited
D061305	Lymphoscintigraphy	Radionuclide imaging of the LYMPHATIC SYSTEM.	Lymphoscintigraphies