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- Agnieszka Giza, Tomasz Stramek, Andrzej Jaworek, Wojciech Jurczak, Aleksander SkotnickiScalp skin primary cutaneous follicle center lymphoma - Case report

- Raditsa Sokolova, Svetlan Dermendzhiev, Rumyna YankovaAngioedema associated with Helicobacter pylori and type 2 diabetes

- Aboubacar H. Bambara, Amina N Ouedraogo, Nina A Ouedraogo Nde, Vincent B Ili, Jérôme Sanou, Achach Thouraya Chtioui, Tarticus KonsemDermatofibrosarcome de Darier Ferrand sous orbitaire: Une localisation rare [Dermatofibrosarcoma Darier Ferrand under orbital: A rare location]

Supplement 1 / 2016

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Contents

case repOrts � 366

369

Agnieszka Giza, Tomasz Stramek, Andrzej Jaworek, Wojciech Jurczak, Aleksander Skotnicki

Angioedema associated with Helicobacter pylori and type 2 diabetes............................................

Scalp skin primary cutaneous follicle center lymphoma - Case report............................................

Raditsa Sokolova, Svetlan Dermendzhiev, Rumyna Yankova

Dermatofibrosarcome de Darier Ferrand sous orbitaire: Une localisation rare[Dermatofibrosarcoma Darier Ferrand under orbital: A rare location]..............................................372Aboubacar H. Bambara, Amina N Ouedraogo, Nina A Ouedraogo Nde, Vincent B Ili,Jérôme Sanou, Achach Thouraya Chtioui, Tarticus Konsem

© Our Dermatol Online Suppl. 1.2016 366

Our Dermatology Online

How to cite this article: Giza A, Stramek T, Jaworek A, Dyduch G, Jurczak W, Skotnicki A. Scalp skin primary cutaneous follicle center lymphoma - Case report. Our Dermatol Online. 2016;7(Suppl. 1):366-368.Submission: 30.06.2016; Acceptance: 02.08.2016DOI: 10.7241/ourd.20163.100

Scalp skin primary cutaneous follicle center Scalp skin primary cutaneous follicle center lymphoma - Case reportlymphoma - Case reportAgnieszka Giza1, Tomasz Stramek1, Andrzej Jaworek2, Grzegorz Dyduch3, Wojciech Jurczak1, Aleksander Skotnicki1

1Department of Heamatology, Jagiellonian University College of Medicine, Krakow, Poland, 2Department of Dermatology, Jagiellonian University College of Medicine, Krakow, Poland, 3Department of Pathomorphology, Jagiellonian University College of Medicine, Krakow, Poland

Corresponding author: Dr. Agnieszka Giza, E-mail: agnieszka.giza4@wp.pl

INTRODUCTION

Primary cutaneous follicle center lymphoma is defined as the neoplastic proliferation of follicle center cells affecting the skin. It is classified as Indolent Cutaneous B-cell Lymphoma and is the most common primary B-cell lymphoma of the skin representing almost 11% of cutaneous lymphomas. There are three different growth patterns of the PCFCL: follicular, a follicular and diffuse, or a diffuse type. Neither of them is characterized by a worse or better prognosis [1,2].

Indolent cutaneous B-cell lymphomas have an excellent prognosis and 5- year survival rate for cutaneous follicle center lymphoma is more than 95%. Local recurrences are observed in about 20 % of the patients but extracutaneous involvement is rare. Complete staging investigations are necessary to distinguish between primary cutaneous follicle center lymphoma and the secondary skin involvement by systemic follicle center lymphoma because clinicopathologic features

are extremely alike [1,2]. Staging procedures comprise CT scan of the whole body and bone marrow biopsy.

Local radiotherapy is preferred treatment method in PCFCL. Anthracycline-based chemotherapy might be considered in patients with widespread cutaneous disease and in cases of extracutaneous involvement [3]. Systemic or local ant-CD20 antibody administration is alternative treatment method report in some recent papers. It might be useful for advanced disease with generalized lesions as well [4].

CASE REPORT

A 55-year-old male patient presented to the Department of Dermatology of the University Hospital in Krakow in February 2015 with small, bug bite alike, erythematous, gradually extending lesion on the parietal area of the head. The lesion was asymptomatic. No pruritus or pain was reported. Since then patient was treated with topical

ABSTRACT

Primary cutaneous follicle center lymphoma is an Indolent Cutaneous B-cell Lymphoma responsible for almost 11% of primary cutaneous hematopoietic skin neoplasm. Although PCFCL has excellent prognosis of more than 95% 5 year survival rate it might be a challenge for a clinician because of diagnostic difficulties and a need to proceed complete differential diagnosis with systemic follicle center lymphoma. We present a case of a 55-year-old male patient with small, bug bite alike, erythematous, gradually extending lesion on the parietal area of the head. There were no other sign of disease on the skin and no other sides spreading of disease which was confirmed in CT imaging and no other systemic symptoms. After the diagnosis was made course of radical skin lesions 30 Gy radiotherapy were administered. In the follow-up examination skin lesion resolved completely and patient achieved complete remission of the disease.

Key words: PCFCL; Skin scalp; Lymphoma; Non-Hodgkin; Primary cutaneous follicle center lymphoma

Case Report

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steroids (Elitasone) without any improvement. Skin biopsy were performed in June 2015 and revealed small lymphocyte infiltration of the dermis (CD3/CD20=2/3). Expression of CD20 was positive, CD10 negative and there was faint expression of bcl-2. In two separate FR1-JH and FR2-JH amplifications reveal monoclonal proliferation of B-cells. Staging procedures were performed and patient was refered to the Department of Hematology of the University Hospital in Krakow.

Physical examination at the admission to Outpatient Clinic of the Department of Hematology revealed 2 localized erythematous tumors on the parietal area of the scalp, 4 cm and 1,5 cm in diameter (Fig. 1). There were no other alteration on the skin and no other symptoms like fever, night sweats, weight lost, skin pruritus or malaise. Physical examination didn’t reveal lymph node enlargement and hepatosplenomegaly. The CT scans of chest, abdomen and pelvis did not show any abnormalities. USG of the neck revealed multiple lymph nodes of regular structure as in reactive lymph nodes. The laboratory tests such us CBC, LDH and beta 2 microglobulin level, liver and kidney were performed repeatedly and did not show any alterations beside slightly elevated monocyte count in CBC and CRP level. Result of a test for Borrelia Burgdorferi antibodies was negative.

After fifth excisional biopsy the final diagnosis of the Primary Cutaneous Follicle Center Lymphoma was made. The patient was referred to Department of Radiotherapy and the course of radiotherapy from 15 to 20 December 2015 were administered. The dose of radical skin lesions 30 Gy radiotherapy was devided into 15 fractions of radiation. In the follow-up examination both skin lesions resolved completely and no other symptoms of the disease were noticed (Fig. 2). Next follow-up is planned for September 2016.

DISCUSSION

Primary cutaneous lymphoma are rare entity of all lymphomas with an estimated annual incidence of 1:100000 [5]. They are heterogenic and most, about 65%, derived from T lymphocytes and only 25% from B lymphocytes. Primary cutaneous follicle center lymphoma is the most common cutaneous B cell lymphoma and represent almost 11% of cutaneous lymphomas. In the previous classification was defined as a variant of follicular lymphoma, but since 2008 in classification WHO represent because of different clinical course new entity.

Primary cutaneous follicle center lymphoma presents clinically with characteristic erythematous solitary or grouped plaques, tumors or papules without ulceration. Lesions are preferentially located on the scalp, forehead or on the trunk [6]. Erythematous macules and papules may accompany central tumor or plaque and form a lesion called in the past “reticulohistiocytoma of the dorsum” or “Crosti’s lymphoma” [7]. These presentations of PCFCL form mainly diffuse pattern of growth and affect preferably the trunk. Typical follicle growth pattern primary cutaneous follicle center lymphomas have predilection for the head and neck [2]. Multifocal lesions might be observed but there is no association between the skin presentation and the course of the disease or prognosis [1]. Presentations of PCFCL may resemble non-malignant lesions of granulomatous rosacea, rhinophyma or even acne [8].

Figure 1: Erythematous non-pruritic lesions on the skin of parietal area of the scalp in 55-year-old patient.

Figure 2: Complete resolution of the lesions after course of radical radiotherapy.

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PCFCL may involve entire dermis and extend to the subcutaneous tissue. Lesions usually consists of small and large cleaved cells (centrocytes) and some number of non-cleaved cells (centroblasts). Small, reactive T-cells are mixed with the tumor tissue. The epidermis is not affected [9]. The follicles of the lymphoma have a reduced or absent mantle zone [10].

The usual immunophenotype of neoplastic cells include CD20+, CD79a+, bcl-6+. Diffuse type lesions may reveal CD10 expression dissimilarly to follicular growth type [11]. Expression of bcl-2 protein in primary cutaneous follicle center lymphomas is usually absent but there might be some faint bcl-2 staining in minority of follicular B-cells [12]. There are negative staining results for CD5, CD43 and MUM-1 [13].

Primary cutaneous follicle center lymphomas reveal a monoclonal rearrangement of the JH immunoglobulin gene and somatic hypermutation of variable heavy and light chain genes, which indicates their follicle center cell origin [1,14]. Interchromosomal t(14:18) translocation characteristic for systemic follicular lymphomas is extremely uncommon in PCFCL [2].

The main and most difficult issue is to make properly diagnosis quite often by some, repeated skin biopsies which should be examined in medical center with experienced histopathologist. In differential diagnosis should be considered diffuse large B cell lymphoma leg type and primary cutaneous marginal zone lymphoma. After evaluation of clinical stage and exclusion of secondary skin involvement by systemic follicle center lymphoma local, radical skin radiotherapy seem to be the best therapeutic option carries 95% of 5 years survival rate.

REFERENCES

1. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classifi cation for cutaneous lymphomas.

Blood. 2005;105:3768-85.2. Cerroni L, Gatter K, Kerl H. Skin Lymphoma: The Illustrated

Guide, 3rd Edition. Wiley-Blackwell, Oxford 2011:129-37.3. Grange F, Bekkenk MW, Wechsler J, Meijer CJ, Cerroni L, Bernengo M,

et al. Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study. J Clin Oncol. 2001;19:3602-10.

4. Heinzerling LM, Urbanek M, Funk JO, Peker S, Bleck O, Neuber K, et al. Reduction of tumor burden and stabilization of disease by systemic therapy with anti-CD20 antibody (rituximab) in patients with primary cutaneous B-cell lymphoma. Cancer. 2000;89:1835-44.

5. Frank D. Groves, Martha S. Linet, Lois B. Travis, and Susan S. Devesa. Cancer Surveillance Series: Non-Hodgkin’s Lymphoma Incidence by Histologic Subtype in the United States From 1978 Through 1995. JNCI J Natl Cancer Inst. 2000;92:1240-51.

6. Willemze R, Meijer CJ, Sentis HJ, Scheffer E, van Vloten WA, Toonstra J, et al. Primary cutaneous large cell lymphomas of follicular center cell origin. J Am Acad Dermatol. 1987;16:518-26.

7. Berti E, Alessi E, Caputo R, Gianotti R, Delia D, Vezzoni P. Reticulohistiocytoma of the dorsum. J Am Acad Dermatol. 1988;19:259-72.

8. Barzilai A, Feuerman H, Quaglino P, David M, Feinmesser M, Halpern M,, et al. Cutaneous B-Cell neoplasms mimicking granulomatous rosacea or rhinophyma. Arch Dermatol. 2012;148:824.

9. Nathwani BN, Winberg CD, Diamond LW, Bearman RM, Kim H. Morphologic criteria for the differentiation of follicular lymphoma from fl orid reactive follicular hyperplasia: A study of 80 cases. Cancer. 1981;48:1794–806.

10 Goodlad JR, Krajewski AS, Batstone PJ, McKay P, White JM, Benton EC, et al. Primary cutaneous follicular lymphoma: a clinicopathologic and molecular study of 16 cases in support of a distinct entity. Am J Surg Pathol. 2002;26:733-41.

11. de Leval L, Harris NL, Longtine J, Ferry JA, Duncan, LM. Cutaneous B-cell lymphomas of follicular and marginal zone types: use of Bcl-6, CD10, Bcl-2, and CD21 in differential diagnosis and classifi cation. Am J Surg Pathol. 2001;25:732-41.

12. Geelen FA, Vermeer MH, Meijer CJ, Van der Putte SC, Kerkhof E, Kluin PM, et al. Bcl-2 expression in primary cutaneous large Bcell lymphoma is site-related. J Clin Oncol. 1998;16:2080-5.

13. Hoefnagel JJ, Dijkman R, Basso K, Jansen PM, Hallermann C, Willemze R, et al. Distinct types of primary cutaneous large B-cell lymphoma identifi ed by gene expression profi ling. Blood. 2005;105:3671-8.

14. Aarts WM, Willemze R, Bende RJ, Meijer CJLM, Pals ST, van Noessel CJ. VH gene analysis of primary cutaneous B-cell lymphomas: evidence for ongoing somatic hypermutation and isotype switching. Blood. 1998;92:3857-64.

Copyright by Agnieszka Giza, 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.Source of Support: Nil, Confl ict of Interest: None declared.

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Our Dermatology Online

How to cite this article: Sokolova R, Dermendzhiev S, Yankova R. Angioedema associated with Helicobacter pylori and type 2 diabetes. Our Dermatol Online. 2016;7(Suppl. 1):369-371.Submission: 27.06.2016; Acceptance: 23.07.2016DOI:10.7241/ourd.20163.101

Angioedema associated with Helicobacter pylori and Angioedema associated with Helicobacter pylori and type 2 diabetestype 2 diabetesRaditsa Sokolova1, Svetlan Dermendzhiev2, Rumyna Yankova1

1Department of Dermatology and Venereology, Medical University Plovdiv, University Hospital of Plovdiv, Plovdiv, Bulgaria, 2Department of Allergologyand Occupational Medicine, Medical University Plovdiv, University Hospital of Plovdiv, Plovdiv, Bulgaria

Corresponding author: Dr. Raditsa Sokolova, E-mail: radica.sokolova@gmail.com

INTRODUCTION

Chronic urticaria/angioedema is defined as wheals, angioedema or both with daily or almost daily symptoms lasting for more than 6 weeks [1]. Angioedema is characterized by localized, self-limiting, nonpitting swelling of the skin and/or of the mucous membranes of the upper respiratory and gastrointestinal tracts [2,3]. Most guidelines include isolated angioedema as a subtype of chronic spontaneous urticaria [4-6]. About 10% of patients with chronic spontaneous urticaria do not show wheals and exclusively develop angioedema [7]. Angioedema without wheals with duration of symptoms over six weeks is relatively rare. The most common forms of chronic and isolated angioedema are induced by medications such as angiotensin-converting enzyme inhibitors (ACE inhibitors), nonsteroidal anti-inflammatory agents and antibiotics. Hereditary angioedema with different types is also part of the chronic isolated forms of angioedema, and their diagnosis is important for proper treatment [1]. On the other

hand, isolated angioedema associated with metabolic abnormalities, and laboratory evidence of infection with Helicobacter pylori is more rarely [7,8]. Various etiologic factors, the broad range of immunological and non-immunological changes reflecting the pathogenetic mechanisms and defining forms of urticaria and angioedema often create diagnostic and differential diagnostic difficulties for dermatologists and allergists. This is especially true for the chronic forms, the more that they sometimes establish an association with a number of accompanying metabolic, infectious, malignant, systemic connective tissue diseases and others.

CASE REPORT

We present a case of 66-year-old male patient with recurrent isolated angioedema without wheals, affecting the upper lip (Fig. 1), with disease duration of one year and without symptoms of respiratory and gastrointestinal tract. The patient reported only numbness of the upper

ABSTRACT

Angioedema without wheals with duration of symptoms over six weeks is relatively rare. This applies especially for the chronic forms of angioedema associated with metabolic, infectious, malignant, and systemic connective tissue disease. Described in the literature cases are rare, especially when the etiology of isolated angioedema associated with Helicobacter pylori and type 2 diabetes. We report a case of a 66-year-old male patient with recurrent isolated angioedema without wheals, affecting the upper lip, with a disease duration of one year and no symptoms of respiratory and gastrointestinal tract. Laboratory, immunological and serological studies we found only elevated blood sugar and elevated Anti Helicobacter pylori IgG antibody. In patients with type 2 diabetes mellitus etiology of angioedema may be associated with Helicobacter pylori and poor metabolic control. To be an effective treatment, the etiology of such phenomena should be precisely clarified with the respective set of immunological and serological tests.

Key words: Angioedema; Helicobacter pylori; Type 2 diabetes

Case Report

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lip a few hours before the start of angioedema. With a history of acute episode of angioedema with wheals 7 years ago, drug induced by a single dose of ACE-inhibitor (enalapril maleate). Without family history of atopy and other allergic diseases. He suffers from type 2 diabetes and hypertension. His daily treatment included diet for type 2 diabetes and chlophasolin 0,15 mg/day for hypertension. Except angioedema localizated to upper lip (Fig. 1), findings from the physical and dermatology examination and are unremarkable.

The hematology and blood differential tests were in reference ranges. Total bilirubin, total protein, albumin, urea, ALT, AST and electrolytes in blood were normal expect blood glucosae levels was elevated 8,18 mmol/l (reference ranges 3,3-6, 0 mmol/l). Anti-thyreoglobulin (anti-TG) and anti-peroxidase (anti-TPO) antibodies, TSH and FT4 were normal. C1 inhibitor levels, C4 serum complement levels, IgA, IgG, and total IgE were in reference ranges. Anti-Toxoplasma gondii IgM and IgG titer were negative. Anti Helicobacter pylori IgG antibody titer was positive 55 IU,ml (reference ranges < 20 IU,ml), but the Helicobacter pylori stool antigen was negative. Stool tests for enteroparasites (Giardia lamblia, Amoebiasis and Blastocystis hominis) were negative. Abdominal ultrasound was normal.

We started treatment with levocetirizine dihydrochloride 10 mg/daily for 6 months and Metformin 1000 mg/daily. Attacks of angioedema disappeared. After discontinuing treatment with an levocetirizine dihydrochloride patient is in complete remission, asymptomatic and without recurrence of angioedema (Fig. 2).

DISCUSSION

Type 2 diabetes is one of the diseases often associated with the etiology of chronic urticaria. As unlockable clinical manifestations of urticaria factors indicating unsatisfactory metabolic control and received antidiabetic drugs, many of which rash and itching are cited as more frequent or rare side effects. Hypertension is a common comorbidity in patients with diabetes. The American Diabetes Association guidelines recommend the use of either ACE inhibitors or angiotensin receptor blocker (ARB) as initial therapy to achieve the blood pressure target in patients with type 2 diabetes mellitus [9,10]. One of the most common causes of drug-related angioedema is from ACE inhibitor [1]. Our patient had a medical history of drug-related angioedema from ACE inhibitor. Angioedema is also seen with ARB therapy but much less frequently than with ACE inhibitors [11]. On the

Figure 1: Angioedema without wheals, affecting the upper lip.

other hand, antidiabetic drugs-Dipeptidyl peptidase-IV inhibitor are also associated with angioedema and increase angioedema risk in patients treated with ACE inhibitors [12]. In our patient, laboratory testing showed elevated blood glucose levels. Rogala et al. associated isolated angioedema with elevated blood glucose levels and impaired glucose tolerance. The reasons for this remain unclear. It may be that impaired glucose tolerance and high glucose levels contribute to the development of recurrent angioedema [7]. In our patient, serology testing showed elevated Anti Helicobacter pylori IgG antibody titer. Described in the literature cases of isolated angioedema induced from Helicobacter pylori are rare and the treatment designed to eradicate the infection shows no significant results [8].

CONCLUSION

Isolated angioedema without wheals induced by the combination of various factors presented our case is

Figure 2: Patient without angioedema.

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a rare phenomenon. In patients with type 2 diabetes mellitus etiology of angioedema may be associated with Helicobacter pylori and poor metabolic control. To be an effective treatment, the etiology of such phenomena should be precisely clarified with the respective set of immunological and serological tests.

Consent

The examination of the patient was conducted according to the Declaration of Helsinki principles.

REFERENCES

1. Powell RJ, Leech SC, Till S, Huber PA, Nasser SM, Clark AT; British Society for Allergy and Clinical Immunology. BSACI guideline for the management of chronic urticaria and angioedema. Clin Exp Allergy. 2015;45:547-65.

2. Mansi M, Zanichelli A, Coerezza A, Suffritti C, Wu MA, Vacchini R, et al. Presentation, diagnosis and treatment of angioedema without wheals: a retrospective analysis of a cohort of 1058 patients. J Intern Med. 2015;277:585-93.

3. Kaplan AP. Angioedema. World Allergy Organ J. 2008; 1:103-13.4. Fer rer M, Bar t ra J, Giménez-Arnau A, Jauregu i I ,

Labrador-Horrillo M, Ortiz de Frutos J, et al. Management of urticaria: Not too complicated, not too simple. Clin Exp Allergy. 2015;45:731-43.

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Copyright by Raditsa Sokolova, 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.Source of Support: Nil, Confl ict of Interest: None declared.

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How to cite this article: Bambara AH, Ouedraogo AN, Ouedraogo Nde NA, Ili VB, Sanou J, Chtioui AT, Konsem T. Dermatofi brosarcoma Darier Ferrand under orbital: A rare location. Our Dermatol Online. 2017;7(Suppl. 1):372-376.Submission: 11.08.2016; Acceptance: 12.08.2016DOI: 10.7241/ourd.20163.102

Dermatofi brosarcoma Darier Ferrand under orbital: Dermatofi brosarcoma Darier Ferrand under orbital: A rare locationA rare locationAboubacar H. Bambara1, Amina N. Ouedraogo2, Nina A. Ouedraogo Nde3, Vincent B. Ili4, Jérôme Sanou5, Achach Thouraya Chtioui6, Tarticus Konsem4

1Service of Cancerology, Teaching Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso, 2Service of Dermatology, Raoul Follereau Center Ouagadougou, Burkina Faso, 3Service of Radiology, District Bogodogo Hospital Ouagadougou, Burkina Faso, 4Service of Stomatology and Maxillo-Facial Surgery, Teaching Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso, 5Service of Ophtalmology, Teaching Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso, 6Laboratoire of Anatomy and Cytopathology, Monastir, Burkina Faso

Corresponding author: Dr. Aboubacar H. Bambara, E-mail: boubabambara@hotmail.com

ABSTRACT

Dermatofibrosarcoma protuberans (DFS) is a rare malignant skin tumor, characterized by local aggressiveness, significant potential for recurrence and a low risk of distant metastasis. The location under the eyes, not yet reported in the literature may involve the functional and aesthetic prognosis. We report a case.

Keywords: Dermatofibrosarcoma protuberans; Under orbital; Recurrence; Surgery

24.Dermatofi bro_CR

Case Report

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Our Dermatology Online

How to cite this article: Bambara AH, Ouedraogo AN, Ouedraogo Nde NA, Ili VB, Sanou J, Chtioui AT, Konsem T. Dermatofi brosarcome de Darier Ferrand sous orbitaire: Une localisation rare. Our Dermatol Online. 2017;7(Suppl. 1):372-376.Submission: 11.08.2016; Acceptance: 12.08.2016DOI: 10.7241/ourd.20163.102

Dermatofi brosarcome de Darier Ferrand sous Dermatofi brosarcome de Darier Ferrand sous orbitaire: Une localisation rareorbitaire: Une localisation rareAboubacar H. Bambara1, Amina N. Ouedraogo2, Nina A. Ouedraogo Nde3, Vincent B. Ili4, Jérôme Sanou5, Achach Thouraya Chtioui6, Tarticus Konsem4

1Service of Cancerology, Teaching Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso, 2Service of Dermatology, Raoul Follereau Center Ouagadougou, Burkina Faso, 3Service of Radiology, District Bogodogo Hospital Ouagadougou, Burkina Faso, 4Service of Stomatology and Maxillo-Facial Surgery, Teaching Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso, 5Service of Ophtalmology, Teaching Hospital Yalgado Ouedraogo, Ouagadougou, Burkina Faso, 6Laboratoire of Anatomy and Cytopathology, Monastir, Burkina Faso

Corresponding author: Dr. Aboubacar H. Bambara, E-mail: boubabambara@hotmail.com

RESUME

Le dermatofibrosarcome de Darier-Ferrand (DSF) est une tumeur cutanée maligne rare, caractérisée par une agressivité locale, un important potentiel aux récidives et par un faible risque de métastase à distance. La localisation sous orbitaire, non encore rapporté dans la littérature peut mettre en jeu le pronostic fonctionnel et esthétique. Nous en rapportons un cas.

Mots clés: Dermatofibrosarcome de Darier et Ferrand; Sous orbitaire; Récidive, Chirurgie

INTRODUCTION

Le dermatofibrosarcome de Darier-Ferrand (DSF) est une tumeur mésenchymateuse cutanée maligne rare. Il représente 1% des tumeurs malignes cutanées et moins de 5 % des sarcomes des tissus mous de l’adulte. Il est caractérisé par une agressivité locale, un important potentiel aux récidives et un faible risque de métastase à distance [1]. Les localisations les plus fréquemment décrites sont le tronc et les extrémités [2]. La localisation sous orbitaire n’est pas fréquemment décrite dans la littérature. Nous en rapportons un cas.

CASE REPORT

Mme XY, âgée de 26 ans, femme au foyer, domiciliée à Ouagadougou, était reçue en consultation d’oncologie médicale pour une reprise évolutive rapide, non douloureuse d’une tumeur sous orbitaire droite après une 3ème exérèse sans examen anatomopathologique. La tuméfaction évoluerait depuis 14 ans. L’examen clinique

retrouvait une tuméfaction nodulaire, molle, polylobée, érythémateuse luisante, hypochromique au centre, parcourue de lacis veineux, développée aux dépens de la région palpébrale inférieure droite s’étendant à la joue droite, à la racine et à l’aile droite du nez, mesurant 16 cm x 15 cm x 12 cm. On notait une occlusion partielle de l’œil droit par la tumeur, un affaissement de l’aile droite du nez et une déviation de la pointe du nez du côté opposé sans obstruction nasale (Fig. 1). L’acuité visuelle était de 9/10 à l’œil droit et de 10/10 à l’œil gauche. On ne notait pas d’adénopathies sous mandibulaires, ni sus claviculaires palpables. Les autres aires ganglionnaires sont libres. L’auscultation pulmonaire était normale ainsi que le reste de l’examen physique.

L’étude histologique de la biopsie de la tumeur a noté une prolifération tumorale siégeant dans le derme et l’hypoderme, composée de cellules fusiformes, avec peu d’atypies et de mitoses, groupées en petits faisceaux flexueux, enchevêtrés dans les zones nodulaires de forte densité cellulaire réalisant un aspect en “ panier tressé ” ou aspect “ storiforme ” des Anglo-Saxons).

Case Report

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La tumeur infiltre l’hypoderme en nappes dissociant les lobules adipeux, et sous forme de coulées dans les cloisons interlobulaire (Fig. 2).

L’immunomarquage par le CD 34 réalisé à l’extérieur du pays était positif, ainsi que celui par le PDGFB-COL1A1. (Fig. 3) et négatif au PS100 (Fig. 4).

Le scanner cranio-facial retrouvait une masse de densité tissulaire mesurée à 96 x 43 x 42 mm avec un effet de masse sur le globe oculaire, qui est refoulé en dehors mais sans l’envahir. Il n’y avait pas de densité de la graisse intra orbitaire droite, ni de lyse osseuse (Fig. 5).

La radiographie du thorax et l’échographie abdomino-pelvienne étaient normales. Le bilan biologique était également normal.

Nous avons conclu au diagnostic de dermatofibrosarcome de Darier Ferrand sous orbitaire récidivant au stade

Figure 1: Vue de face de la tumeur: une tuméfaction nodulaire, molle, polylobée, érythémateuse luisante, hypochromique au centre, parcourue de lacis veineux, développée aux dépens de la région palpébrale inférieure droite.

Figure 2: Tumeur envahissant le derme profond et l’hypoderme. HES X40.

Figure 3: Positivité diffuse des cellules tumorales avec le CD34.

Figure 4: Négativité des cellules tumorales avec la PS100.

Figure 5: Coupe axiale en fenêtre parenchymateuse, effet de masse sur le globe oculaire droit refoulé en dehors sans anomalie de densité de la graisse intra orbitaire droite.

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localement avancé sans métastases, ni envahissement ganglionnaire.

Une chimiothérapie néoadjuvante à visée réductrice à l’Imatinib et une chirurgie large avec reconstruction probable étaient proposés à la patiente.

DISCUSSION

Le DSF a été décrit en 1924 par Darier et Ferrand [3]. C’est une tumeur cutanée rare mais non exceptionnelle. L’âge de survenu est variable, mais elle touche de manière préférentielle l’adulte jeune comme c’est le cas de notre patiente âgée de 26 ans [4-7].

Des localisations préférentielles au niveau du tronc, des membres supérieurs et inférieurs ont été souvent décrites [1,8,9]. La localisation à l’extrémité céphalique représente 15% des localisations, et celle sous orbitaire palpébrale inhabituelle, retrouvée chez notre patiente n’a pas encore été rapporté à notre connaissance.

Le caractère récidivant de la DSF après une exérèse incomplète, avec des marges insuffisantes ou non en monobloc présents dans la quasi-totalité des cas rapporté dans la littérature était également observé chez notre patiente qui était à sa 4e récidive [10-13].

L’aspect clinique du DSF DF à un stade avancé de nodule réalise une masse multinodulaire, irrégulière, bosselée, dure, souvent polychrome, de taille variable. L’état général est généralement conservé, il n’y a pas de signes fonctionnels, ni généraux associés, ce qui était retrouvé chez notre patiente.

Le diagnostic du DSF DF, fait recours à un faisceau d’arguments constitués par:• l’histoire évolutive de la tumeur caractérisée par des

récidives, ce qui était noté chez notre patiente.• le siège dermique strict de la prolifération ce qui

était noté à l’histologie de la biopsie de la tumeur de notre patiente.

• l’aspect histologique de la tumeur: prolifération tumorale intradermique à cellules fusiformes avec une architecture storiforme tourbillonnante également noté chez notre patiente.

• L’immunohistochimie qui objective un marquage positif à l’antigène CD34, était retrouvé chez notre patiente.

Le diagnostic différentiel du DSF DF peut être difficile, notamment sur des prélèvements biopsiques.

Il peut simuler un histiocytofibrome de forme cellulaire et profond dans les zones de forte densité cellulaire. Les zones de faible densité cellulaire peuvent être confondues avec un neurofibrome, un dermatomyofibrome.

Cependant l’immunomarquage permet de rejeter ces hypothèses. Les cellules exprimant le CD 34 n’expriment généralement pas le facteur XIIIa (contrairement aux histiocytofibromes). La négativité de la PS100 permet d’éliminer une tumeur nerveuse.

La possibilité d’une transformation sarcomateuse du DSF DF doit toujours rester à l’esprit devant les récidives multiples, et l’apparition de métastases surtout pulmonaire. Il doit être recherché à l’histologie par les critères tels: atypies cellulaires, un pattern fasciculaire, 10 mitoses pour 10 champs au grossissement 40, une diminution de l’expression du CD34, une augmentation de l’expression de Mib1 est observée à l’immunomarquage. Une transformation de plus de 5 % du tissu tumoral recherché [5].

Les risques de métastases est rare, estimé à 5 % et essentiellement pulmonaires. Les cas de métastases rapportés étaient liés à une transformation sarcomateuse de plus haut grade de malignité du DSF DF récidivant.

La chirurgie est le traitement de choix de la DSF non métastasé [12]. La chirurgie de Mohs ou une chirurgie d’exérèse en bloc avec des marges de sécurité larges de 3 à 5 cm de tissu sain, emportant une barrière saine en profondeur est le seul moyen thérapeutique ayant fait la preuve de son efficacité du DSF DF ainsi que de la prévention des récidives. Dans les formes localement avancée, ou les formes avec des métastases, on a recours à une chimiothérapie à l’Imatinib, ou une radiothérapie en traitement palliatif.

Cependant, la localisation sous palpébrale inhabituelle, la grande taille de la tumeur, l’atteinte des parties molles avoisinantes (joue, nez) et les antécédents de récidive chez notre patiente ont motivé le choix d’une chimiothérapie néoadjuvante avec l’Imatinib avant d’envisager une chirurgie de reconstruction maxillo-faciale afin de préserver la fonction de l’œil.

CONCLUSION

Tu m e u r m é s e n c h y m a t e u s e m a l i g n e , l e dermatofibrosarcome de Darier-Ferrand est rare. Le

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risque évolutif de cette tumeur est plus lié aux récidives locales qu’aux métastases justifiant de la nécessité d’une chirurgie d’exérèse large d’emblée. Un suivi régulier également nécessaire afin de dépister une transformation sarcomateuse. Sa location sous orbitaire non encore rapportée, peut mettre en jeu le pronostic fonctionnel et esthétique de l’œil et du visage.

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Copyright by Aboubacar H. Bambara, 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.Source of Support: Nil, Confl ict of Interest: None declared.

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