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complete blood count&#44; and erythrocyte sedimentation rate were within normal limits&#46; Serology for human immunodeficiency virus&#44; hepatitis A&#44; B&#44; and C viruses&#44; Epstein-Barr virus&#44; cytomegalovirus&#44; toxoplasma&#44; and parvovirus B19 was negative&#46; The Mantoux test was negative&#46; Sputum and urine cultures for mycobacteria were negative&#46; Culture and the polymerase chain reaction for mycobacteria in the skin biopsy were negative&#46; Thoracic and abdominal computed tomography revealed pathological axillary&#44; mediastinal&#44; paratracheal&#44; para-aortic&#44; hilar&#44; subcarinal&#44; cardiophrenic&#44; retroperitoneal&#44; and mesenteric lymph nodes&#46; Fine needle aspiration biopsy of an axillary lymph node showed no signs of malignancy&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What is Your Diagnosis&#63;</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Subcutaneous sarcoidosis associated with systemic sarcoidosis&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was started with prednisone&#44; 1<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#44; by mouth in a tapering regimen&#44; and the patient was administered 3 monthly intralesional injections with triamcinolone acetonide diluted 1-in-3 in normal saline&#46; This produced a slight improvement&#44; with no increase in the number of lesions at subsequent follow-up visits&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Sarcoidosis is a chronic granulomatous disease of unknown origin&#44; characterized by the formation of noncaseating granulomas in different organs and tissues&#46; Around 25&#37; of patients develop skin lesions&#44; which can be specific or nonspecific depending on the presence or absence of granulomas on histology&#46; The specific lesions include macules&#44; papules&#44; plaques&#44; lupus pernio&#44; subcutaneous sarcoidosis&#44; and cicatricial sarcoidosis&#44; while the nonspecific lesions are erythema nodosum&#44; plaques of alopecia&#44; calcifications&#44; and pruritic lesions&#44; among others&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Subcutaneous sarcoidosis&#44; also known as Darier-Roussy sarcoid&#44; is the least common specific form &#40;12&#37;&#41; and only affects the hypodermis&#46; It is most common in white women aged between 40 and 60 years&#46; Clinically it presents as multiple firm&#44; slightly painful&#44; mobile&#44; round&#44; skin-colored nodules that typically arise on the limbs bilaterally but asymmetrically&#46; They have also been reported on the trunk&#44; face&#44; buttocks&#44; and head and neck&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Ahmed and Harshad<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> reported a strong association between subcutaneous sarcoidosis and moderate systemic involvement by sarcoidosis&#46; Lesions of subcutaneous sarcoidosis are observed in up to 2&#37; of cases of systemic sarcoidosis&#46; Although the lesions may be the first sign of the systemic sarcoidosis&#44; it is more common for them to develop during the course of the disease&#59; they are rarely an isolated finding&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The diagnosis is based on the clinical manifestations and histology&#44; which reveals a predominantly lobular panniculitis with noncaseating epithelioid granulomas&#44; with no peripheral lymphocytic corona&#44; but with a large number of multinucleated giant cells&#46; Schaumann &#40;conchoidal&#41; bodies and asteroid bodies may be seen&#59; although these lesions are characteristic&#44; they are not pathognomic of sarcoidosis&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The differential diagnosis should include erythema nodosum&#44; tuberculosis&#44; and other granulomatous diseases&#44; such as fungal or atypical mycobacterial infections&#44; birefringent foreign bodies&#44; and autoimmune diseases such as rheumatoid arthritis or lupus erythematosus&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Some authors have reported spontaneous resolution of the nodules&#46; However&#44; when associated systemic disease is present&#44; the oral corticosteroids&#44; hydroxychloroquine&#44; tetracyclines&#44; methotrexate&#44; dapsone&#44; clofazimine&#44; and nonsteroidal anti-inflammatory drugs have been found to be effective&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Our case is interesting because the appearance and easy accessibility of lesions in the frontal region enabled us to make a diagnosis of systemic sarcoidosis associated with subcutaneous sarcoidosis &#40;Darier-Roussy sarcoid&#41;&#46; 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Cases for Diagnosis
Nodules on the Forehead Associated With Generalized Lymphadenopathy
Nódulos frontales asociados a adenopatías generalizadas
A. Lobato-Berezoa,
Autor para correspondencia
allobe@hotmail.es

Corresponding author.
, F. Burgos-Lázarob, M.Á. Gallego-Valdésa
a Departamento de Dermatología y Venereología, Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain
b Departamento de Anatomía Patológica, Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">The patient was a Moroccan man aged 27 years&#46; He had no known drug allergies and no past history of interest&#46; He was being investigated in internal medicine for a widespread lymphadenopathy that had developed several months earlier&#46; Two months before consultation in dermatology&#44; he developed slightly tender lesions on the forehead that had progressively increased in size&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Three nodular lesions measuring between 10 and 20<span class="elsevierStyleHsp" style=""></span>mm in diameter were observed in the left frontal region&#46; There were no changes in the overlying skin &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy of a lesion revealed epithelioid granulomas in the hypodermis with a low-grade lymphocytic response and foreign body material&#44; with no central necrosis&#46; Birefringence for foreign bodies was negative &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">General biochemistry&#44; C-reactive protein&#44; angiotensin converting enzyme&#44; complete blood count&#44; and erythrocyte sedimentation rate were within normal limits&#46; Serology for human immunodeficiency virus&#44; hepatitis A&#44; B&#44; and C viruses&#44; Epstein-Barr virus&#44; cytomegalovirus&#44; toxoplasma&#44; and parvovirus B19 was negative&#46; The Mantoux test was negative&#46; Sputum and urine cultures for mycobacteria were negative&#46; Culture and the polymerase chain reaction for mycobacteria in the skin biopsy were negative&#46; Thoracic and abdominal computed tomography revealed pathological axillary&#44; mediastinal&#44; paratracheal&#44; para-aortic&#44; hilar&#44; subcarinal&#44; cardiophrenic&#44; retroperitoneal&#44; and mesenteric lymph nodes&#46; Fine needle aspiration biopsy of an axillary lymph node showed no signs of malignancy&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What is Your Diagnosis&#63;</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Subcutaneous sarcoidosis associated with systemic sarcoidosis&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was started with prednisone&#44; 1<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#44; by mouth in a tapering regimen&#44; and the patient was administered 3 monthly intralesional injections with triamcinolone acetonide diluted 1-in-3 in normal saline&#46; This produced a slight improvement&#44; with no increase in the number of lesions at subsequent follow-up visits&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Sarcoidosis is a chronic granulomatous disease of unknown origin&#44; characterized by the formation of noncaseating granulomas in different organs and tissues&#46; Around 25&#37; of patients develop skin lesions&#44; which can be specific or nonspecific depending on the presence or absence of granulomas on histology&#46; The specific lesions include macules&#44; papules&#44; plaques&#44; lupus pernio&#44; subcutaneous sarcoidosis&#44; and cicatricial sarcoidosis&#44; while the nonspecific lesions are erythema nodosum&#44; plaques of alopecia&#44; calcifications&#44; and pruritic lesions&#44; among others&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Subcutaneous sarcoidosis&#44; also known as Darier-Roussy sarcoid&#44; is the least common specific form &#40;12&#37;&#41; and only affects the hypodermis&#46; It is most common in white women aged between 40 and 60 years&#46; Clinically it presents as multiple firm&#44; slightly painful&#44; mobile&#44; round&#44; skin-colored nodules that typically arise on the limbs bilaterally but asymmetrically&#46; They have also been reported on the trunk&#44; face&#44; buttocks&#44; and head and neck&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Ahmed and Harshad<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> reported a strong association between subcutaneous sarcoidosis and moderate systemic involvement by sarcoidosis&#46; Lesions of subcutaneous sarcoidosis are observed in up to 2&#37; of cases of systemic sarcoidosis&#46; Although the lesions may be the first sign of the systemic sarcoidosis&#44; it is more common for them to develop during the course of the disease&#59; they are rarely an isolated finding&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The diagnosis is based on the clinical manifestations and histology&#44; which reveals a predominantly lobular panniculitis with noncaseating epithelioid granulomas&#44; with no peripheral lymphocytic corona&#44; but with a large number of multinucleated giant cells&#46; Schaumann &#40;conchoidal&#41; bodies and asteroid bodies may be seen&#59; although these lesions are characteristic&#44; they are not pathognomic of sarcoidosis&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The differential diagnosis should include erythema nodosum&#44; tuberculosis&#44; and other granulomatous diseases&#44; such as fungal or atypical mycobacterial infections&#44; birefringent foreign bodies&#44; and autoimmune diseases such as rheumatoid arthritis or lupus erythematosus&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Some authors have reported spontaneous resolution of the nodules&#46; However&#44; when associated systemic disease is present&#44; the oral corticosteroids&#44; hydroxychloroquine&#44; tetracyclines&#44; methotrexate&#44; dapsone&#44; clofazimine&#44; and nonsteroidal anti-inflammatory drugs have been found to be effective&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Our case is interesting because the appearance and easy accessibility of lesions in the frontal region enabled us to make a diagnosis of systemic sarcoidosis associated with subcutaneous sarcoidosis &#40;Darier-Roussy sarcoid&#41;&#46; It should be noted that sarcoid nodules are very rare on the forehead&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Información del artículo
ISSN: 15782190
Idioma original: Inglés
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