Unusual Manifestation Of Specific Cutaneous Involvement By B-cell Chronic Lymphocytic Leukemia: Spontaneous Regression With Scar Formation
Kazakov, D. V., Belousova, I. E., Michaelis, S., Palmedo, G., Samtsov, A. V., & Kempf, W. (2003). Unusual manifestation of specific cutaneous involvement by B-cell chronic lymphocytic leukemia: spontaneous regression with scar formation. Dermatology (Basel, Switzerland), 207(1), 111–115. https://doi.org/10.1159/000070961
View Original Source →Abstract
We report a patient with specific cutaneous involvement by B-cell chronic lymphocytic leukemia, who demonstrated unusual clinical features during the course of the disease, namely several spontaneous regressions of skin lesions with the formation of scars. In addition, histologically proven keratoacanthoma was found. During the follow-up period of approximately 1.5 years, the patient experienced several recurrences of skin lesions and their partial spontaneous regression. The scars persisted and remained unchanged. We hypothesize that vascular injury combined with edema could have accounted for dermal ischemia and the subsequent development of the scarring lesions.
Case Details
Disease Location
Skin
Personal Characteristics
79 -year-old male
Clinical Characteristics
Diagnosis of b-cll with specific cutaneous involvement march 2001, patient presented with an approx. 1-month history of asymptomatic generalized skin erupatientions physical revealed multiple erythematous patches and plaques varying in size and shape distributed over the whole integument some lesions had annular and semiannular configs, there was a solitary nodule on his right shoulder with a central depression, the patient reported this had appeared in jan 2001 and showed rapid growth there was moderate bilateral enlargement of the inguinal lymph nodes biopsies from one of the plaques, the skin tumor, the bone marrow and the right inguinal lymph nodes were performed, bone marrow aspiration was also carried out histology of the skin specimens from the plaque showed a dense patchy infiltrate arranged around the blood vessels of the upper and mid dermis less dense cellular aggregations were found around dilated small vessels in fate lobules in the subcutis the infiltrate was mainly composed of small monthsomorphous lymphocytes with scant cytoplasm and clumped chromatin few small to medium cells with denser chromatin and a narrow rim of eosinophilic cytoplasm, prolymphocytes and paraimmunoblasts were seen occasional mitotic figures were detected in foci, the cells of the infiltrate were found within the walls of small and medium-sized vessels, the endothelium appeared swollen, the dermis was edematous biopsy from the nodule lesion revealed histopathological changes consistent with keratoacanthoma a dense infiltrate was noted at the base of the neoplasm, the infiltrate consisted of small uniform lymphocytes admixed with a few paraimmunoblasts the marrow showed the interstitial type of marrow involvement with 30-70% of small lymphoid cells possessing scant cytoplasm and clumped chromatin the inguinal ln biopsy revealed effacement of normal lymph node architecture by diffuse proliferation of small lymphoid cells with cytological features identical to those detected in the skin and ln biopsies focally, pseudo follicular structures were found within the area of the neoplastic infiltrate ihc found the neoplastic cells in the skin (plaque), marrow, and ln had the same immunophenotype, they expressed cd45rb, CD20, 79a, 5, and 23 a few cells in the skin expressed CD3, the cells in the vessel walls expressed CD20 and 5, CD3+ lymphocytes were also seen the cells in the infiltrate at the base of the keratoacanthoma were positive for CD20 and 5 monoclonal b-cell populations in the skin and ln were detected by pcr fragment analysis of igh genes. The monoclonal population appeared as 2 peaks, either biallelic rearrangement or 2 different dominant clones peripheral blood revealed an elevated wbc count and lymphocytosis, lymphocytes were small and uniform CT found slight to moderate enlargement of several intra-abdominal lymph nodes and neoplastic-looking lesion involving the right suprarenal gland diagnosis was made, monthly blood work and skin exams were performed as regular observation
Remission Characteristics
5 months after the initial presentation, the skin lesions spontaneously regressed partially the majority of the patches and plaques on the trunk and lower limbs regressed completely within 2-3 weeks some lesions disappeared leaving bruise-like patches on both arms, the regression of the lesions led to the formation of peculiar scars with bizarre jagged configuration until september 2002, there were several new recurrences of the skin lesions followed by sr recurrences were characterized by less extensive erupatientions the scarring lesions persisted and remained unchanged during this time, general condition remained good but his wbc count had gradually increased with a peak of 28x10^9/l with 61% lymphocytes in oct 2002, 18 months after diagnosis, the patient showed a rapid decrease in platelet count (150x10^9/l), a further increase in wbc (24x10^9/l) and lymphocytosis (87% lymphocytes) he was referred to hematology for specific therapy, his condition was good but complained of fatigue
Treatment & Mechanisms
Proposed Remission Mechanisms
No major mechanism proposed
Clinical Treatment
None reported
Non-Clinical Treatment
None reported