Chronic lymphatic leucemia, skin manifestations C91.10

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)

1. Colburn DE et al (2002) Skin infiltration with chronic lymphocytic leukemia is consistent with a good prognosis. Hematology 7: 187-188
2. Filling DR (2003) Granuloma annulare-like infiltrates with concomitant cutaneous involvement by B-cell non-Hodgkin's lymphoma: report of a case. At J Dermatopathol 25: 57-61
3. 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
4. Kaddu S, Cerroni L, Kerl H (2003) Leukemias of the skin. In: Kerl H et al (ed.) Histopathology of the skin. Springer Verlag Berlin, Heidelberg, New York: S 917-919
5. Kazakov DV (2003) Unusual manifestation of specific cutaneous involvement by B-cell chronic lymphocytic leukemia: spontaneous regression with scar formation. Dermatology 207: 111-115
6. Lu C et al (2015) Cutaneous manifestations in a patient with chronic lymphocytic leukemia involving the head, neck and distal extremities. Exp Ther Med 9:877-879
7. Popovic L et al (2014) Concurrent chronic lymphocytic leukemia and merkel cell carcinoma in primary skin tumor and metastatic lymph node. Indian J Hematol Blood Transfus 30(Suppl 1):422-424
8. 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
9. Sandhu M et al(2015) Chronic lymphocytic lymphoma presenting with recurrent demodicidosis. Ann Allergy Asthma Immunol 114:426-42
10. Stuhr PM et al (2015) Wells syndrome associated with chronic lymphocytic leukemia. An Bras Dermatol 90:571-57