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Cutaneous B-Cell Neoplasms Mimicking Granulomatous Rosacea Or Rhinophyma

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Cutaneous B-Cell Neoplasms Mimicking Granulomatous Rosacea Or Rhinophyma OBSERVATION ONLINE FIRST Cutaneous B-Cell Neoplasms Mimicking Granulomatous Rosacea or Rhinophyma Aviv Barzilai, MD, MSc; Hana Feuerman, MD; Pietro Quaglino, MD; Michael David, MD; Meora Feinmesser, MD; Marisa Halpern, MD; Edit Feldberg, MD; Carlo Tomasini, MD; Hilla Tabibian-Keissar, PhD; Ninette Amarilgio, PhD; Emmilia Hodak, MD Background: Unlike T-cell neoplasms, B-cell lympho- cutaneous follicular center B-cell lymphoma in 4 cases, proliferative disorders have a limited clinical spectrum primary cutaneous marginal zone lymphoma in 6, and of skin involvement. Cutaneous B-cell neoplasms mim- skin involvement of chronic lymphocytic leukemia in 2. icking rosacea or rhinophyma are rare. All patients had an indolent course as expected for their disease. Observations: We described 12 patients with B-cell lymphoproliferative neoplasms presenting with a facial Conclusions: Cutaneous involvement of B-cell neo- eruption clinically mimicking rosacea or rhinophyma. plasms may mimic granulomatous rosacea or rhino- Eleven patients were women; ages ranged from 36 to 81 phyma. This unusual clinical presentation is more com- years. The clinical presentation included small papules mon in women and appears in the setting of preexisting on the nose and cheeks and around the eyes mimicking rosacea or as a new eruption. Proliferative B-cell disor- granulomatous rosacea; nodules on the nose, cheeks, ders should be added to the differential diagnosis of sym- chin, or forehead mimicking phymatous rosacea; or a metric papular or papulonodular eruptions of the face. combination of both. Three patients had preexisting erythematotelangiectatic rosacea and 1 had rhino- Arch Dermatol. 2012;148(7):824-831. phyma. Based on a clinicopathologic correlation and Published online April 16, 2012. B-cell clonality analysis, the diagnosis was primary doi:10.1001/archdermatol.2011.3575 -CELL NEOPLASMS CAN IN- who were referred to our tertiary dermatology volve the skin as a primary clinics from January 1, 1996, through Decem- cutaneous lymphoma or as ber 31, 2010, for a persistent facial rash. The a secondary process, includ- differential diagnoses included rosacea and ing specific infiltrates of rhinophyma. We retrieved clinical history, clinical findings, laboratory results, and nodal or extranodal lymphoma or leuke- follow-up data from the medical files and B1 mia. When involving the skin, both B- reviewed the biopsy specimens. Final diagno- cell neoplasms, lymphoma and leuke- sis was made according to the criteria of the mia, have a distinct clinical appearance, World Health Organization or the European presenting as isolated, grouped, or mul- Organization for Research and Treatment of tiple erythematous to violaceous pap- Cancer.1 The study was approved by the local ules, plaques, or nodules, usually in an ethics committees. asymmetric distribution. B-cell lympho- proliferative diseases simulating rosacea are RESULTS extremely rare.2-8 In this report, we de- scribe 12 patients who presented with ro- sacea or rhinophymalike lesions and dis- The patients’ clinical data and final diag- cuss various aspects of this rare clinical noses are described in Table 1. The manifestation of low-grade B-cell lym- study group included 11 women and 1 phoma/leukemia involving the face. man with a mean age of 57 (range, 36-81) years. The time elapsed from the initial presentation to the final diagnosis METHODS varied from a few months to 10 years (mean time, 23 months). The distin- Author Affiliations are listed at We performed a retrospective case analysis of guishing features of the clinical presenta- the end of this article. 12 patients with cutaneous B-cell neoplasms tion included nonpustular granuloma- ARCH DERMATOL/ VOL 148 (NO. 7), JULY 2012 WWW.ARCHDERMATOL.COM 824 ©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Table 1. Clinical Data and Final Diagnosis in Patients With B-Cell Neoplasms Mimicking Rosacea or Rhinophyma Patient No./ Duration Before Final Sex/Age, y Diagnosis Clinical Presentation Initial Diagnosis Early Therapy Diagnosis 1/F/51 3 y Papulonodular eruption on the Rosacea and rhinophyma Minocycline hydrochloride, PCFCL cheeks; erythematous infiltrated topical metronidazole plaque on the nose 2/F/48 4 mo Erythematous infiltrated plaque on the Rhinophyma None PCFCL nose on top of rosacea 3/F/36 3 y Facial erythematous and skin-colored Rosacea Erythromycin, minocycline PCMZL papules with erythema and telangiectasia 4/F/67 6 mo Nodular eruption on nose, cheeks, Rosacea and rhinophyma Topical clindamycin CLL, leukemia and chin phosphate cutis 5/F/81 3 y Erythematous infiltrated plaque on the Rhinophyma None PCMZL nose 6/F/60 4 mo Erythema on the distal part of the Sebaceous hyperplasia, None PCMZL nose with 3 small nodules basaliomas; granulomatous rosacea 7/F/52 6 mo Small erythematous papules on Granulomatous rosacea None PCMZL cheeks but also on neck, nape, and chest 8/F/57 6 mo Small skin-colored to erythematous Granulomatous rosacea, None PCFCL papules on cheek, sides of the basaliomas, sebaceous nose, eyebrows, helices, and upper hyperplasia back 9/F/36 6 mo Erythematous papules around the Granulomatous rosacea, Liquid nitrogen PCMZL mouth, including the chin and on sarcoidosis, and the nose molluscum 10/M/74 10 y Erythematous infiltrated nodular Rhinophyma Azithromycin, topical CLL, leukemia lesion on the nose metronidazole cutis 11/F/70 1 y Erythematous plaques on the nose Granulomatous rosacea, Topical metronidazole PCMZL and cheeks sarcoidosis 12/F/49 6 mo Erythematous infiltrated plaque on the Granulomatous rosacea Topical metronidazole PCFCL left ala nasi Abbreviations: CLL, chronic lymphocytic leukemia; PCFCL, primary cutaneous follicular center cell lymphoma; PCMZL, primary cutaneous marginal zone lymphoma. Figure 1. Erythematous (granulomatous rosacea-like) papules on the right cheek in a patient with primary cutaneous follicular center B-cell lymphoma Figure 2. Rhinophymalike lesion in a patient with primary cutaneous (patient 1). marginal zone lymphoma (patient 11). tous rosacea-like lesions and rhinophyma/phymatous plaques (Table 1, Figure 1,andFigure 2). Four The histological and immunophenotypical features patients (patients 1-3 and 10) had a history of preexist- of the cases and the results of molecular studies are ing rosacea manifesting as erythematotelangiectatic described in Table 2. Four cases revealed superficial rosacea (in 3 patients) and rhinophyma (in 1 patient) and deep nodular aggregates of centrocytes and centro- (Figure 3). Two of these patients developed rhinophy- blasts, features of follicular-center cell lymphoma malike lymphoma on the nose. Half the patients were (Figure 4). Six cases showed superficial and deep treated for rosacea with topical or systemic antibiotics nodular aggregates of small irregular B lymphocytes, or both, without any benefit (Table 1). some with a monocytoid or plasmacytoid appearance ARCH DERMATOL/ VOL 148 (NO. 7), JULY 2012 WWW.ARCHDERMATOL.COM 825 ©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 and residual fragmented germinal centers, features of mar- ginal cell lymphoma (Figure 5). Two cases had diffuse dermal infiltrates of mostly small regular B cells (Figure 6), consistent with skin involvement by chronic lymphocytic leukemia (CLL). Furthermore, in patient 4, results of an IgH rearrangement study showed an iden- tical clone in the blood and skin (Figure 6), establishing the diagnosis of leukemia cutis. Demodex folliculorum was not present in any of the biopsy specimens. Based on a full workup including clinicopathologic cor- relation and analysis of B-cell clonality, the diagnosis re- tained was primary cutaneous follicular center B-cell lym- phoma in 4 of 12 cases (33%), primary cutaneous marginal zone lymphoma in 6 (50%), and skin involvement of CLL in 2 (17%) (Table 2). All the patients had an indolent course as expected for their disease. Initial therapy, which included radiotherapy and/or excisions for 8 patients with Figure 3. Papular/granulomatous rosacea-like lesions on the background of primary cutaneous B-cell lymphoma (PCBCL), led to com- preexisting erythematotelangiectatic rosacea. Small erythematous papules that were determined to be primary cutaneous marginal zone lymphoma plete remission. However, in patients 3 and 8, new le- were found on erythema and telangiectasia (patient 3). This patient had sions developed at sites not previously treated. Intral- papules on both cheeks and also on the submental areas. esional or potent topical corticosteroids applied to these Table 2. Histological, Immunophenotypical, and Molecular Characteristics of Cases of B-Cell Neoplasms Mimicking Rosacea and Rhinophyma Histological Features Patient IgH Gene Final No. Architecture Cells Immunophenotype Rearrangement Diagnosis 1 Superficial and deep nodular Small and large (centrocytes, Positive for CD20 and CD10 in germinal center–like ND PCFCL centroblasts) structures; negative for bcl-2 2 Superficial and deep, nodular Small and large, regular and Positive for CD20, bcl-6, and CD10 in follicular Monoclonal PCFCL and diffuse irregular center cells; CD23 in nonfollicular center cells; and CD3 small cells between aggregates 3 Superficial and deep, nodular Small irregular and monocytoid Positive for CD20 and bcl-2 in T cells and marginal Monoclonal PCMZL and diffuse, follicular in marginal areas and areas, bcl-6 and CD10 in germinal centers, and colonization, prominent
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