Cutaneous histiocytic lymphangitis: An unusual manifestation of rheumatoid arthritis

Abstract

Two cases are presented of unusual cutaneous lesions associated with rheumatoid arthritis in underlying joints. The lesions were evanescent, erythematous and violaceous partly macular and partly indurated plaques, with a livedo-like pattern of erythema at the edge in one case. Histological changes were identical in the two cases. The major features were dilated, dermal lymphatics containing aggregates of inflammatory cells, mainly histiocytes, with adjacent perivascular lymphoid aggregates. An appropriate name for this reaction would appear to be cutaneous histiocytic lymphangitis.

Successful Treatment with Tocilizumab in a Case of Intralymphatic Histiocytosis Associated with Rheumatoid Arthritis

“…The most frequent underlying disease of ILH is RA, but other causes, such as a breast cancer (4) and replacement arthroplasty (6), have been reported. The cause of ILH has yet to be eluci-dated, although it is speculated that lymphatic drainage of inflammatory cells may be involved in its development in RA (7).…”

Intravascular histiocytosis associated with rheumatoid arthritis

“…This rare disorder was firstly described by O'Grady et al in 1994 [9]. However, these reported cases have used different terms, involving intravascular histiocytosis, cutaneous histiocytic lymphangitis and intravascular or intralymphatic histiocytosis [2,3,8,10]. Also similar skin lesions were named angioendotheliomatosis with rheumatoid arthritis [4].…”

“…Pruim et al suggested that the cutaneous lesions might represent lymphatic drainage of inflammatory components away from inflamed synovium and not be specifically related to RA [2]. Reactive intravascular histiocytosis associated with RA has a benign nature, but these lesions are relatively resistant to topical and systemic therapies and show a prolonged clinical course [7,8].…”

“…Its primary manifestations present in the synovium of joints that has a propensity to affect the small joints of the hands and feet with extra-articular manifestations comprising skin lesions [1,2]. The prototypic skin lesion is the rheumatoid nodule [1].…”

Cheilitis granulomatosa und Melkersson-Rosenthal-Syndrom

“…Ätiologisch werden die intralymphatischen histiozytären Aggregate entweder als Resultat einer "zellulären Drainage" des entzündlichen Prozesses der Synovia über die Lymphbahnen interpretiert [3] oder im Rahmen der vaskulären Histiozytose als frühe Form einer reaktiven Angioendotheliomatose [7].…”