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Bullous Erythrodermic Mycosis Fungoides: A Case Report Naciri Ilhame 1* , Ebongo Christelle 1 , Askour Majda 1 , Rimani Mouna 2 , Meziane Mariam 1 , Senouci Karima 1 and Hassam Badredine 1 1 Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco 2 Department of General Surgery A, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco * Corresponding author: Naciri Ilhame, Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco, Tel: +212-645-30-70-37; E-mail: [email protected] Received date: February 02, 2017; Accepted date: March 29, 2017; Published date: April 07, 2017 Copyright: © 2017 Ilhame N, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Bullous mycosis fungoides is a rare entity of cutaneous T-cell lymphomas; however, it can be particularly aggressive with an unfavorable prognosis. Mechanism of formation of bullous lesions in bullous lymphoma remains poorly understood, it can be explained by excessive epidermotropism or tumor infiltration’s toxicity. We report the case of a bullous erythrodermic mycosis fungoides. Keywords: Cutaneous lymphoma; Mycosis fungoides; Erythroderma; Blisters; Rare Introduction Bullous mycosis fungoides (MF) is a rare entity with only about 20 cases reported in literature [1]. However, it is particularly aggressive with poor prognosis [2]. We report a case of bullous erythrodermic MF. Clinical Case A 52-years-old woman with no significant pathological past history presenting for 3 years multiple erythematous-squamous and pruritic plaques of the trunk, aggravated 3 months ago by the progressive installation of diffuse bullous and erosive lesions. is evolving without fever nor alteration of general condition. Clinical examination showed a dry erythroderma, with post-bullous erosions of the trunk and limbs (Figure 1), Nikolsky sign was negative. Bilateral firm and painless inguinal lymphadenopathies were also found, the largest one was of 2 cm. Skin biopsy showed an epidermal cleavage without visible acantolysis, associated to an epidermotropism, a dense and a pilotropism (Figure 2). e infiltrate was made of atypical lymphocytes, mainly expressing anti-CD4 (Figure 3), and anti-CD3 antibodies (Figure 4), and lacking anti-CD8 (Figure 5), anti-CD20, and anti-CD30 antibodies. Direct immunofluorescence was negative. Hemogramme, hepatic and renal assessment, blood levels of beta2microglobulin and LDH were normal. ere was less than 5% of circulating sezary cells and screening for atypical lymphocytes in the peripheral lymph nodes and the bone marrow was negative. oraco- abdomino-pelvic tomodensitometry was normal. e diagnosis of stage III (T4N1B0M0) bullous erythrodemic MF was retained and the patient treated by 25 mg/week methotrexate, with a slight improvement, marked by the disappearance of erosive lesions aſter three months of treatment. But a relapse occurred aſter 8 months, then we switched for the association of PUVA-therapy (at the rate of 3 sessions per week) and acitretin 1 mg/kg/day. e outcome was favorable, with a 6-month decline. Figure 1: Dry Erythroderma with diffuse post bullous erosions on the trunk and limbs. Figure 2: Cutaneous histology (HES, G x 100), dermal lymphoid infiltrate with epidermotropism and pilotropism. Ilhame et al., J Clin Trials 2017, 7:2 DOI: 10.4172/2167-0870.1000305 Case Report Open Access J Clin Trials, an open access journal ISSN:2167-0870 Volume 7 • Issue 2 • 1000305 J o u r n a l o f C li n i c a l T r i a l s ISSN: 2167-0870 Journal of Clinical Trials
Transcript of n a l o f Cl incal u r ri o als Journal of Clinical Trials€¦ · (Figure 1), Nikolsky sign was...
Bullous Erythrodermic Mycosis Fungoides: A Case ReportNaciri Ilhame1*, Ebongo Christelle1, Askour Majda1, Rimani Mouna2, Meziane Mariam1, Senouci Karima1 and Hassam Badredine1
1Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco2Department of General Surgery A, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco*Corresponding author: Naciri Ilhame, Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco, Tel:+212-645-30-70-37; E-mail: [email protected]
Received date: February 02, 2017; Accepted date: March 29, 2017; Published date: April 07, 2017
Copyright: © 2017 Ilhame N, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricteduse, distribution, and reproduction in any medium, provided the original author and source are credited.
Abstract
Bullous mycosis fungoides is a rare entity of cutaneous T-cell lymphomas; however, it can be particularlyaggressive with an unfavorable prognosis. Mechanism of formation of bullous lesions in bullous lymphoma remainspoorly understood, it can be explained by excessive epidermotropism or tumor infiltration’s toxicity. We report thecase of a bullous erythrodermic mycosis fungoides.
Keywords: Cutaneous lymphoma; Mycosis fungoides;Erythroderma; Blisters; Rare
IntroductionBullous mycosis fungoides (MF) is a rare entity with only about 20
cases reported in literature [1]. However, it is particularly aggressivewith poor prognosis [2]. We report a case of bullous erythrodermicMF.
Clinical CaseA 52-years-old woman with no significant pathological past history
presenting for 3 years multiple erythematous-squamous and pruriticplaques of the trunk, aggravated 3 months ago by the progressiveinstallation of diffuse bullous and erosive lesions. This evolving withoutfever nor alteration of general condition. Clinical examination showeda dry erythroderma, with post-bullous erosions of the trunk and limbs(Figure 1), Nikolsky sign was negative. Bilateral firm and painlessinguinal lymphadenopathies were also found, the largest one was of 2cm. Skin biopsy showed an epidermal cleavage without visibleacantolysis, associated to an epidermotropism, a dense and apilotropism (Figure 2). The infiltrate was made of atypicallymphocytes, mainly expressing anti-CD4 (Figure 3), and anti-CD3antibodies (Figure 4), and lacking anti-CD8 (Figure 5), anti-CD20, andanti-CD30 antibodies. Direct immunofluorescence was negative.Hemogramme, hepatic and renal assessment, blood levels ofbeta2microglobulin and LDH were normal. There was less than 5% ofcirculating sezary cells and screening for atypical lymphocytes in theperipheral lymph nodes and the bone marrow was negative. Thoraco-abdomino-pelvic tomodensitometry was normal. The diagnosis ofstage III (T4N1B0M0) bullous erythrodemic MF was retained and thepatient treated by 25 mg/week methotrexate, with a slightimprovement, marked by the disappearance of erosive lesions afterthree months of treatment. But a relapse occurred after 8 months, thenwe switched for the association of PUVA-therapy (at the rate of 3sessions per week) and acitretin 1 mg/kg/day. The outcome wasfavorable, with a 6-month decline.
Figure 1: Dry Erythroderma with diffuse post bullous erosions onthe trunk and limbs.
Figure 2: Cutaneous histology (HES, G x 100), dermal lymphoidinfiltrate with epidermotropism and pilotropism.
Ilhame et al., J Clin Trials 2017, 7:2 DOI: 10.4172/2167-0870.1000305
Case Report Open Access
J Clin Trials, an open access journalISSN:2167-0870
Volume 7 • Issue 2 • 1000305
Jour
nal of Clinical Trials
ISSN: 2167-0870
Journal of Clinical Trials
Figure 3: Immunohistochemistry (G x 100), dermal andepidermotropic lymphocytes labeled with anti-CD4 antibodies,with an under epidermis detachment.
Figure 4: Immunohistochemistry (G x 100), dermal andepidermotropic lymphocytes labeled with anti-CD3 antibodies.
Figure 5: Immunohistochemistry (G x 200), dermal andepidermotropic lymphocytes predominantly CD8.
CommentsMF is the most frequent subtype of cutaneous T-cell lymphomas,
however bullous lesions remain extremely rare. The first case of bullousMF was described by Kaposi in 1887 as pemphigus-like mycosisfungoides [3]. Bullous MF usually occurs in the elderly, without sexpredominance. Bullous lesions usually appear several months or yearsafter the classic MF plaques, but in some cases they may be inaugural,making the diagnosis very difficult [4]. Bowman et al. proposed criteriafor the diagnosis of bullous MF, including the presence of clinicalblisters, pathognomonic histological signs of MF, immunofluorescencenegativity and exclusion of other bullous dermatoses [2]. The exactmechanism of blisters formation in bullous MF remains poorlyunderstood. Several hypotheses have been suggested: the direct orindirect degeneration of dermic collagen fibers induced by neoplasticcells [5]. Tumor’s infiltration toxicity, the release of certainlymphokines, and their interference with the normal cohesion ofKeratinocytes [6], and a direct mechanical effect of the accumulationof lymphocytes between epidermic cells of the basal layer in the eventof excessive epidermotropism, leading to loss of coherence betweenadjacent cells [7]. The presence of a massive tumoral infiltrate in ourpatient suggests that the third mechanism may play important role inour case.
ConclusionAlthough mycosis fungoides bullosa is extremely rare, however, it
can be particularly aggressive and associated with a poor prognosis. Ithas to be regarded as an important clinical subtype of cutaneous T-celllymphoma.
References1. Kneitz H, Bröcker EB, Becker JC (2010) Mycosis fungoides bullosa: a case
report and review of the literature. J Med Case Rep 4: 78.2. Bowman PH, Hogan DJ, Sanusi ID (2001) Mycosis fungoides bullosa:
report of a case and review of the literature. J Am Acad Dermatol 45:934-939.
3. Kaposi M (1887) “Mycosis fungoides” und ihre beziehungen zu anderenahnlichen erkrankungsformen. Wien Med Wochenschr 19: 22-37.
4. McBride SR, Dahl MG, Slater DN, Sviland L (1998) Vesicular mycosisfungoides. Br J Dermatol 138: 141-144.
5. Ueda C, Makino T, Asano Y, Watanabe H, Hanakawa H, et al. (2011) Anultrastructural examination of a blistering lesion of mycosis fungoidesbullosa. Br J Dermatol 165: 213-214.
6. Kartsonis J, Brettschneider F, Weissmann A, Rosen L (1990) Mycosisfungoides bullosa. Am J Dermatopathol 12: 76-80.
7. Konrad K (1979) Mycosis fungoides bullosa. LymphoproliferativeDiseases of the Skin (Christophers E, Goos M, eds). New York, NY:Springer- Verlag, 157-162.
Citation: Ilhame N, Christelle E, Majda A, Mouna R, Mariam M, et al. (2017) Bullous Erythrodermic Mycosis Fungoides: A Case Report. J ClinTrials 7: 305. doi:10.4172/2167-0870.1000305
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Volume 7 • Issue 2 • 1000305
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