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Case and Research Letters
Multiple Clustered Dermatofibromas: An Atypical Presentation of a Common Disease
Lo común a veces puede ser atípico: dermatofibromas múltiples agrupados
M. Castellanos-Gonzáleza,
Autor para correspondencia
, J.L. Rodríguez-Peraltob, C. Zarcoa, P. Ortíz-Romeroa
a Departamento de Dermatología y Venereología, Hospital Universitario 12 de Octubre, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital 12 de Octubre, Madrid, Spain
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        "titulo" => "Lo com&#250;n a veces puede ser at&#237;pico&#58; dermatofibromas m&#250;ltiples agrupados"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Shiny&#44; erythematous papules on the medial surface of the right thigh&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Dermatofibroma is the most common fibrohistiocytic tumor of the skin and one of the benign neoplasias that generates most visits to the dermatologist&#46; It usually presents as an isolated lesion and rarely occurs as multiple lesions&#46; The term multiple dermatofibroma is defined either as the appearance of at least 15 dermatofibromas within a few months<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> or&#44; more recently&#44; as 5 to 8 dermatofibromas in 4 months&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In 1984&#44; Dupr&#233; et al<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> described another form of presentation that is far less common&#58; multiple clustered dermatofibromas&#46; We report a new case of this rare condition&#46; A 25-year-old woman was seen in our department for small&#44; erythematous papular lesions&#44; with a dark center and paler borders&#44; and asymptomatic&#44; residual hyperpigmented macules in smaller numbers&#44; all located on the medial surface of the right thigh&#46; The lesions had appeared at 1 year of age&#44; increased in number and size over 2 to 3 months&#44; and subsequently remained stable except for partial remission of isolated lesions &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient reported no general or local history of interest&#46; She brought a magnetic resonance imaging scan that had been performed previously to rule out lymphangioma and that showed no atrophy of the adipose tissue or muscle&#44; and no other relevant alterations&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Biopsy of a lesion revealed epidermal hyperplasia and a proliferation of spindle cells arranged in fascicles or whorls interspersed in bundles of thickened collagen in the underlying reticular dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Immunohistochemistry showed an intense&#44; diffuse expression of vimentin and factor XIIIa&#46; Smooth muscle actin&#44; muscle-specific actin&#44; and CD34 were negative&#46; After histopathological confirmation of the diagnosis of dermatofibroma&#44; it was decided to follow up the patient but to apply no treatment&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">We have found 13 cases of multiple clustered dermatofibromas described in the literature&#44; the majority of which were reported in the review by Gershtenson&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> There is no clinical&#44; histological&#44; or phenotypic differentiation between the individual lesions of multiple clustered dermatofibroma and isolated dermatofibroma lesions&#46; No local triggering factors have been described and&#44; unlike multiple dermatofibromas&#44; no association has been found with states of immunosuppression or other comorbid conditions&#46; Only on 1 occasion did the lesions arise over a thrombosed superficial vein in a patient who had undergone kidney transplantation 1 month earlier&#46; In most of the articles published the lesions had appeared on lower limbs&#44; as was the case in our patient&#46; Apart from our case and 1 case of congenital lesions&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> the dermatofibromas developed in patients up to 30 years of age&#46; A benign course was reported for all patients and no malignant change or secondary metastases have been described to date&#46; Berbis<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> reports a case followed for 20 years without complications&#46; We believe it is important to bear this in mind for the management and follow-up of this condition&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">In our patient&#44; the symptoms&#44; distribution of the lesions&#44; and histopathology were identical to those previously described in the literature&#44; although the onset of the condition at an early age is noteworthy&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In conclusion&#44; we highlight the atypical presentation of multiple clustered dermatofibromas&#44; a subgroup of a very common condition as is dermatofibroma&#44; and its benign course in all the cases described to date&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Castellanos-Gonz&#225;lez M&#44; et al&#46; Lo com&#250;n a veces puede ser at&#237;pico&#58; dermatofibromas m&#250;ltiples agrupados&#46; Actas Dermosifiliogr&#46;2011&#59;102&#58;467-479&#46;</p>"
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