Subclinical chronic lymphocytic leukemia with atypical cutaneous presentation

Abstract

Chronic lymphocytic leukemia (CLL) involving skin is a rare but well-documented occurrence, mainly reported in advanced disease. In contrast, CLL presenting with skin lesions is exceedingly rare, only few reports existing to date. We report a 70-year-old man who presented with two non-pruritic, papular lesions on the lower abdomen and proximal thigh. Biopsies showed dense lymphohistiocytic infiltrates involving the reticular dermis and subcutis without epidermotropism consisting mostly of small, CD20 and PAX-5-positive B-cells expressing CD5, CD23, CD43 and BCL2. Numerous large B-cells were present in a T-cell, histiocyte-rich background. A staging bone marrow biopsy showed a clonal B-cell proliferation with typical CLL flow cytometry immunophenotyping but neither lymphadenopathy nor absolute lymphocytosis was present. Numerous B and T-cell cutaneous lymphoproliferative disorders can be associated with increased numbers of histiocytes occasionally masquerading as benign disorders. This was the case with our patient's lesions, originally interpreted as cutaneous Rosai-Dorfman disease. A high index of suspicion from both the pathologist and the dermatologist is essential in identifying these rare but probably underrecognized occurrences of early systemic lymphoproliferative disorders presenting as cutaneous lesions with an unexpected cellular composition.

Bilateral cauliflower ear as the presenting sign of B‐cell chronic lymphocytic leukemia

“…In B‐CLL, cutaneous manifestations generally occur late in the course of disease and are variably seen (in 3‐45%) in patients ( ) . A specific cutaneous infiltrate of B‐CLL can appear as erythematous papules, plaques, nodules or large tumors.…”

Leukaemia cutis in chronic lymphocytic leukaemia following varicella zoster virus reactivation

“…Earlier work suggested that leukaemia cutis in patients with CLL conferred a poor prognosis, often followed by blast crisis and death . While this certainly is the case in Richter's syndrome (large cell transformation), it appears that patients with CLL infiltrates have a better prognosis than was originally thought . In the largest report of cutaneous infiltrates in CLL, the 5‐year survival in 42 patients was 66% .…”

“…9 While this certainly is the case in Richter's syndrome (large cell transformation), it appears that patients with CLL infiltrates have a better prognosis than was originally thought. 7,10 In the largest report of cutaneous infiltrates in CLL, the 5-year survival in 42 patients was 66%. 7 The same study suggested that the mean duration of disease before skin manifestations was 39 months, and in seven patients, skin lesions were the first sign of disease.…”

Ear lobe involvement in chronic lymphocytic leukemia

“…Although indolent, it is refractory to treatment with only allogeneic stem cell transplantation a curative option for selected patients. CLL involves the skin usually in its advanced stages [1]. We present an uncommon cutaneous presentation of CLL and review the literature on all similar cases.…”