Histiocytosis intralymphatic

O'Grady, 1994

Rare (< 50 cases have been reported to date), chronic, reactive lymphatic vascular proliferation with red or reddish-brown spots, plaques or nodules of the skin of unknown aetiology, first described in 1994 under the name "intravascular histiocytosis". It is also observed as reactive intravascular proliferation after osteosynthesis implants (see implant intolerance below).

The cases described have associations with rheumatoid arthritis, implanted artificial joints, dental metal implants and, in individual cases, malignancies. A lymphostasis caused by chronic inflammation with dilated lymph vessels in which histiocytes from the persistent inflammation accumulate is postulated. It is not clear what role metal particles play in this causal chain.

60-80 years, more frequent after joint implants

Extremities - here mostly above or near the joint implant sites; face

Mostly painless, chronically persistent, non-itching, blurred 1.0-5.0 cm, by confluence also larger, red or brownish spots, plaques or nodules. Occasionally livedo-like patterns have also been described. Some cases showed circumscribed firm, flat nodules or swellings, which reminded of a Melkersson-Rosenthal syndrome.

Nodular infiltrates that penetrate the entire dermis in a patchy pattern. These consist of proliferates of improperly configured dilated vascular structures, some with empty lumina, some filled with mononuclear histiocytes. The lymphatic vascular proliferates are surrounded by inflammatory infiltrates. The endothelia of the vessels are flat, often discontinuous. The intralymphatic mononuclear histiocytes express CD68, CD31, CD34 (see CD classification), podoplanin; Prox-1. In some cases, myeloperoxidase was expressed.

Differential diagnosis must exclude reactive angioendotheliomatosis.

The disease proves to be extremely resistant to therapy. The use of antibiotics for several weeks is a (usually unsuccessful) option. Local corticosteroids were unsuccessful. Tacrolimus local was successful in one case.

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