Histiocytosis intralymphatic

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 06.03.2024

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Synonym(s)

Histocytosis intravascular; intralymphatic histiocytosis; Intralymphatic Histiocytosis; intravascular histiocytosis

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HistoryThis section has been translated automatically.

O'Grady, 1994

DefinitionThis section has been translated automatically.

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).

EtiopathogenesisThis section has been translated automatically.

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.

ManifestationThis section has been translated automatically.

60-80 years, more frequent after joint implants

LocalizationThis section has been translated automatically.

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

Clinical featuresThis section has been translated automatically.

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.

HistologyThis section has been translated automatically.

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.

TherapyThis section has been translated automatically.

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.

LiteratureThis section has been translated automatically.

  1. Bidier M, Hamsch C et al (2015) Two cases of intralymphatic histiocytosis after hip arthroplasty implantation. JDDG 13: 700-702
  2. Chiu YE, Maloney JE et al (2010) Erythematous patch overlying a swollen knee--quiz case. Intralymphatic histiocytosis. Arch Dermatol 146:1037-1042
  3. Corti MA, Rongioletti Fet al (2014) Cutaneous reactive angiomatosis with combined histological pattern mimicking a cellulitis. Dermatology 227:226-230
  4. de Unamuno Bustos B, García Rabasco A et al (2013) Erythematous indurated plaque on the right upper limb. Intralymphatic histiocytosis (IH) associated with orthopedic metal implant. Int J Dermatol 52: 547-549
  5. O'Grady JT et al (1994) Intravascualr histiocytosis. Histopathology 24: 265-268
  6. Rossari S, Scatena C et al (2011) Intralymphatic histiocytosis: cutaneous nodules and metal implants. J Cutan Pathol 38: 534-535
  7. Saggar S, Lee B, Krivo J et al (2011) Intralymphatic histiocytosis associated with orthopedic implants. J Drugs Dermatol 10: 1208-1209
  8. Tsujiwaki M, Hata H et al.(2014) Warty intralymphatic histiocytosis successfully treated with topical tacrolimus. J Eur Acad Dermatol Venereol doi: 10.1111/jdv.12570.
  9. Wang Y, Yang H et al (2012) Upper facial swelling: an uncommon manifestation of intralymphatic histiocytosis. Eur J Dermatol 22:814-815.
  10. Washio K, Nakata K et al (2011) Pressure bandage as an effective treatment for intralymphatic histiocytosis associated with rheumatoid arthritis. Dermatology 223:20-24

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Last updated on: 06.03.2024