Chronic lymphatic leucemia, skin manifestations C91.10

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

The skin changes occur

• as monomorphic specific cutaneous or subcutaneous infiltrates in the form of disseminated papules or nodules
• or
• as variable, polymorphic, non-specific concomitant symptoms as part of the therapy measures carried out or the immune incompetence induced by the underlying disease. These include:
  • Pruritus (frequent)
  • chronic urticaria
  • pupura
  • atypical pyoderma
  • severe viral infections (e.g. zoster disease) or
  • secondary tumors(Merkel cell carcinoma, for the development of which immunosuppression is significant (Dücker P et al. 2020); 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 at higher ages:

• 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, asymptomatic plaques lying at skin level or slightly raised, with a smooth atrophic surface (no more follicular structures detectable); also dome-shaped raised, red or red-brown, soft-elastic nodules up to 10 cm in diameter, also with a smooth atrophic surface.
• Rare are diffuse infiltrates of the face, which can lead to extensive, even misshapen, distension of the cheeks and/or forehead and nose(facies leontina). Such clinical pictures are rarely seen today, even in large dermatology centers. They are therefore of historical significance.
• Mucosal manifestations: extensive infiltrates, tumors, macules, tonsillar tumors.

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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2. Dücker P et al. (2020) Merkel cell carcinoma in chronic lymphocytic leukemia. Dermatology 71:553-556.
3. Fullen DR (2003) Granuloma annulare-like infiltrates with concomitant cutaneous involvement by B-cell non-Hodgkin's lymphoma: report of a case. Am J Dermatopathol 25: 57-61
4. Hull PR et al. (2000) Mycosis fungoides and chronic lymphocytic leukaemia--composite T-cell and B-cell lymphomas presenting in the skin. Br J Dermatol 143: 439-444
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9. Robak E et al. (2003) Successful treatment of leukaemia cutis with cladribine in a patient with B-cell chronic lymphocytic leukaemia. Br J Dermatol 147: 775-780
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11. Stuhr PM et al. (2015) Wells syndrome associated with chronic lymphocytic leukemia. An Bras Dermatol 90:571-57