Carcinomatous lymphangiosis C80.x

Erysipelas carcinomatosum; Cancer en cuirasse;   

Lymphangiosis carcinomatosa refers to the spread and accumulation of malignant epithelial tumor cells within lymphatic vessels. Lymphangiosis carcinomatosa is primarily a histopathological term and can affect various organs (see case report and figure).

In the context of a cutaneous manifestation, the intracutaneous, lymphatic spread of a carcinoma results in various clinical manifestations (skin-colored nodules, lumps, plaques). In the presence of a concomitant inflammatory reaction (common in breast cancer), an erysipelas-like clinical presentation may develop (erysipelas carcinomatosum).

Extensive, mostly painless, rough, skin-colored nodules, lumps, and plaques may appear on the skin. Inflammatory plaque-like lesions may also occur, accompanied by erysipelas-like redness and swelling of the skin, as well as tongue-shaped extensions (see alsoErysipelas carcinomatosum; see also Case Report). 

Clusters of tumor cells (emboli) within the lumens of lymphatic vessels

Frequently dilated lymphatic vessels

Secondary interstitial edema

Detection of lymphatic endothelial structures, e.g., using D2-40 (podoplanin)

The difference between lymphangiosis carcinomatosa and erysipelas carcinomatosum lies primarily in their descriptions:

• Lymphangiosis carcinomatosa describes a histopathological pattern of malignant tumor cell spread
• Erysipelas carcinomatosum refers to a clinical-morphological presentation, an “erysipelas-like” clinical presentation (the most important differential diagnosis).

The two terms are thus closely related but not synonymous. 

A female patient in her mid-70s with a known lung adenocarcinoma (T4N3M0) that was being treated with pembrolizumab. Three months prior to the findings shown in the images, the patient noticed lymphedema of the right arm as well as palpable axillary and cervical lymph nodes. The skin lesions were painless but continued to spread. Extensive, map-like, reddish-livid, well-defined, firm plaques were observed. A biopsy revealed the presence of carcinoma clusters in the lymphatic spaces of the skin (Source for this case report: Kerkhoff L (2026)).       

1. Kerkhoff, L. (2026) Lymphangiosis carcinomatosa of the skin in lung cancer. Dt Ärzteblatt 123: 280