Histiocytosis intralymphatic

O'Grady, 1994

Rare (< 80 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 etiology, first described in 1994 under the name "intravascular histiocytosis". It is also observed as reactive intravascular proliferation after osteosynthesis implants (see implant intolerance below) (see also Sima K et al. 2025).

The cases described show associations with rheumatoid arthritis, implanted artificial joints, dental metal implants and, in individual cases, malignancies. A lymph congestion caused by chronic inflammation with dilated lymph vessels in which histiocytes from the persistent inflammation accumulate is postulated. The role of metal particles in underlying implants or malignancies, chronic ostitis in this causal chain is still unclear. However, anamnestic evidence of such associations can often be found.

60-80 years, more frequent after joint implants

Extremities - here mostly over or near the joint implant sites; face (less frequently)

Mostly painless, chronically persistent, non-itching, blurred 1.0-5.0 cm, also larger, red or brownish spots, plaques or nodules due to confluence. Livedo-like or "lymphangitic-striped" patterns have also been described in isolated cases (see figure). Some cases showed circumscribed firm, flat nodules or swellings reminiscent of 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.

Lymphangitis

Benign and malignant angioendothelimatosis

angiosarcoma

Eosinophilic cellulitis

In case of facial involvement: Melkersson-Rosenthal syndrome

The disease proves to be extremely resistant to treatment.

The use of antibiotics for several weeks is a (usually unsuccessful) option.

Local corticosteroids are usually unsuccessful.

Localtacrolimus was successful in one case.

1) Garcia-Mouronte E et al. (2022): An 87-year-old man noticed asymptomatic erythema and erythematous plaques on his left shoulder for one year. He stated that he had previously been diagnosed with severe osteoarthritis at this site. The use of regular medication, heat exposure or the presence of other conditions were denied.

Dermatologic examination revealed 2 mm wide serpiginous erythematous plaques with a vascular anatomic distribution on the front of his left shoulder. Neither cervical nor axillary adenopathies were detected. After a punch biopsy, a diagnosis of intralymphatic histiocytosis was made.

2) Blackwell TJ et al (2021): A 71-year-old woman noticed a bright red, warm and slightly painful patch on her left lower lip for about 6 months. Her medical history included 50 pack-years of smoking, psoriasis and a hospital admission a year ago due to Legionnaires' disease. She was treated with antibiotics for signs of erysipelas without improvement. A CT scan of the face and paranasal sinuses was positive.

A red to purple, slightly swollen, slightly tender plaque was found, which initially covered the left lower lip and now extended to the chin and the front of the neck. The intraoral examination revealed no changes. Blood tests revealed a hemoglobin value of 16.6 and a hematocrit value of 49.7, a CEA value of 6.4 (reference value: 0-4.7) as well as negative ANA and ANCA values. A biopsy revealed intralymphatic histiocytosis.

1. Bidier M, Hamsch C et al. (2015) Two cases of intralymphatic histiocytosis after implantation of a hip endoprosthesis. JDDG 13: 700-702

2. Blackwell TJ et al (2021) Intralymphatic Histiocytosis: An Unusual Presentation. Case Rep Dermatol 13:1-6.

3. Chiu YE, Maloney JE et al (2010) Erythematous patch overlying a swollen knee--quiz case. Intralymphatic histiocytosis. Arch Dermatol 146:1037-1042
4. Corti MA, Rongioletti Fet al. (2014) Cutaneous reactive angiomatosis with combined histological pattern mimicking a cellulitis. Dermatology 227:226-230
5. 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
6. Garcia-Mouronte E et al (2022) Intralymphatic histiocytosis. Dermatol Pract Concept 12:e2022067.
7. O'Grady JT et al. (1994) Intravascualr histiocytosis. Histopathology 24: 265-268
8. Rossari S, Scatena C et al. (2011) Intralymphatic histiocytosis: cutaneous nodules and metal implants. J Cutan Pathol 38: 534-535.
9. Saggar S, Lee B, Krivo J et al. (2011) Intralymphatic histiocytosis associated with orthopedic implants. J Drugs Dermatol 10: 1208-1209.
10. Sima K et al. (2025) Rapidly progressive, asymptomatic red patches on the right upper arm. J Dtsch Dermatol Ges 23:115-117.
11. 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.
12. Wang Y, Yang H et al. (2012) Upper facial swelling: an uncommon manifestation of intralymphatic histiocytosis. Eur J Dermatol 22:814-815.
13. Washio K, Nakata K et al. (2011) Pressure bandage as an effective treatment for intralymphatic histiocytosis associated with rheumatoid arthritis. Dermatology 223:20-24