Chronic lymphatic leucemia, skin manifestationsC91.10

Leukaemic B-cell lymphoma of low malignancy (indolent lymphocytic lymphoma), in which there is clonal proliferation of immunocompetent lymphocytes in lymph nodes, spleen, bone marrow and other organs including the skin.

The skin lesions occur

• appearing as monomorphic specific cutaneous or subcutaneous infiltrates in the form of disseminated papules or nodules
• or
• as variable, polymorphic, non-specific concomitant symptoms in the context of the therapy measures carried out or the immunocompetence induced by the underlying disease. These include: pruritus frequently, chronic urticaria, pupura, atypical pyoderma, severe viral infections (e.g. zoster diseases) or secondary tumours (Merkel cell carcinoma, basal cell carcinoma).

Average incidence: 4/100,000 inhabitants/year (these data do not refer to the incidence of specific skin infiltrates but to that of the underlying disease.

Increasing incidence with age:

• 5th decade of life: 5/100,000 inhabitants/year
• 8th decade of life: 30/100.000 inhabitants/year

Mainly head, face; mostly symmetrical infestation.

• Specific skin infiltrates of B-cell lymphoma (increasingly rare in recent decades due to therapeutic measures):
  • Red, symptom-free plaques lying at skin level or slightly raised, red, symptom-free plaques with smooth atrophic (no more detectable follicular structures) surface; also calotte-shaped raised, up to 10 cm in diameter, red or reddish-brown, soft-elastic nodes also with smooth atrophic surface.
  • Rare are diffuse, infiltrates of the face, which can lead to extensive and also shapeless distensions of the cheeks and/or forehead and nose ( Facies leontina). Such clinical pictures are hardly seen today even in large dermatological centres. Therefore, they have rather historical significance.
  • Appearance of mucous membranes: extensive infiltrates, tumours, macular and tonsil tumours.

Dense, diffuse or nodular dermal infiltrates which can extend into the subcutis (partly perivascular, partly periadnexal localization). Epidermal changes such as exocytosis, spongiosis or ulceration are rarely detectable. Cytomorphologically, small lymphocytes with a hyperchromatic nucleus and narrow cytoplasm predominate. Eosinophil and neutrophil granulocytes, plasma cells and mast cells are more rarely present. Skin infiltrates sometimes show signs of large cell transformation with diffuse accumulations of large monomorphic, centroblast- or immunoblast-like cells (Richter syndrome).

Immunohistologically CD20-positive or CD43-positive neoplastic B-lymphocytes are found. Proliferation marker (MIB-1) is positive in 5-80%.

Cytogenetic: deletion 13q14 (about 55% of patients); trisomy 12q (15% of patients)

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