Intravascular or intralymphatic histiocytosis associated with rheumatoid arthritis: a report of 4 cases

Intravascular histiocytosis associated with rheumatoid arthritis

“…Furthermore, Rieger et al suggested that intravascular histiocytosis might be the early stage of reactive angioendotheliomatosis [10]. Rare cases of intravascular histiocytosis presenting with a clinical and histologic picture similar to that of reactive angioendotheliomatosis but showing intravascular cells with a histiocytic immunophenotype have been described [3,11]. To date, we found twelve other published cases in the English literature that fitted the term "intravascular or intralymphatic histiocytosis associated with RA" (Table 1).…”

“…Recently, chronic erythematous lesions in the vicinity of involved joints in several patients with RA have been reported as an "intravascular histiocytosis" because of their specific histopathological findings [8]. Histopathologically, intravascular histiocytosis is characterized by the proliferation of histiocytes in the dilated blood or lymphatic vessels [3,9]. This rare disorder was firstly described by O'Grady et al in 1994 [9].…”

“…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].…”

“…Histopathologically, chronic erythematous lesions showed features of histiocytic aggregation in dilated dermal vessels [3,8,10].…”

“…The prototypic skin lesion is the rheumatoid nodule [1]. Other skin dermatoses are uncommon and defined histopathologically rather than clinically [1,3]. These dermatoses include vasculitis, rheumatoid papules, rheumatoid neutrophilic dermatosis, superficial ulcerating rheumatoid necrobiosis, neutrophilic lobular panniculitis, erythema nodosum-like lesions, digital purpuric papules, diffuse interstitial and palisading granulomatous dermatitis and pyoderma gangrenosum [1,4].…”