An 84-year-old man had a 3-year history of a progressive, painless, papulonodular eruption, that was particularly prominent on the face and extremities. Physical examination revealed firm, bluish-red nodules and plaques, located on the tip of the nose, the cheeks, ears, and distal digits. Skin lesions produced a leonine facies (Fig. 1), deformities of the fingers and toes, finger clubbing, and onyxis. An identical lesion was seen on a postoperational scar on the left cheek. The mucous membranes were spared. The patient had anterior and posterior cervical and bilateral axillary lymphadenopathy and splenomegaly.
On admission, the peripheral blood count revealed 260,000/mm3 leukocytes (lymphocytes 97%, neutrophils 2%, and monocytes 1%), a hemoglobin level of 9.5 g/dL, and platelet count of 100,000/mm3. Hypogammaglobulinemia with reduction of immunoglobulin G (IgG) and IgM was found.
Radiography of the fingers showed multiple osteolytic lesions of the phalanges and phalangette destruction of the left median finger (Fig. 2a,b). Computed tomography of the chest and abdomen revealed bilateral axillary, mediastinal, and para-aortic lymphadenopathy and spleen enlargement.
Skin biopsy specimens from the ear and finger lesions showed a massive nonepidermal leukemic infiltration in the papillary and reticular dermis, with a grenz zone consisting of small lymphocytes (Fig. 3).
Biopsy of the enlarged cervical lymph node showed a diffuse infiltration with lymphocytes. Tissue biopsy from a finger lytic lesion revealed infiltration of bone trabecular and fibrous tissue with a dense population of small- and medium-sized lymphocytes. Immunohistochemical study of cutaneous and bone lesions showed that the infiltrate in both biopsies consisted mainly of B lymphocytes (CD20+, CD45R+, CD45Ro–, OPD4–).
Peripheral blood smear had a B-cell phenotype (CD19 98%, CD20 97%, CD23 99%, CD25 40%, CD5 90%, HLA-DR 100%). Bone marrow smear and immunophenotyping surface marker analysis found a diffuse pattern of B-lymphocytic infiltration.
A diagnosis of B-cell chronic lymphocytic leukemia stage C (Binet staging system), with specific cutaneous and bone lesions, was established.
The patient received chemotherapy with chlorambucil and methylprednisolone, which resulted in improvement of the hematologic profile. Two years later, the cutaneous lesions showed partial remission.