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Given the acute course and the presence of an underlying mass&#44; a deep biopsy was performed&#44; revealing the presence of foul-smelling fatty tissue with a putrefied appearance &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41;&#44; suggestive of a necrotic infection&#46; A sample was taken for microbiological analysis and calcofluor-white staining and fungal culture were ordered&#46; Direct observation revealed the presence of numerous broad&#44; branched&#44; aseptate hyphae compatible with mucormycosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; After extensive early surgical resection liposomal amphotericin B treatment was instituted and corticosteroid treatment gradually withdrawn&#46; Histology revealed intense dermal necrosis and blood vessel obstruction by numerous fungal structures &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Diagnosis was confirmed based on the results of the fungal culture&#44; from which <span class="elsevierStyleItalic">Rhizopus arrhizus</span> was isolated following ethanol-formic acid extraction and MALDI-TOF &#40;matrix-assisted laser desorption&#47;ionization &#8211; time-of-flight&#41; mass spectrometry&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">1</span></a> After lesion spread was ruled out the patient was diagnosed with primary cutaneous mucormycosis due to <span class="elsevierStyleItalic">R&#46; arrhizus</span>&#46; The early initiation of treatment resulted in a favorable response and progressive improvement of the patient&#39;s clinical picture&#46; No new lesions appeared nor were other organs affected&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Mucormycosis is an opportunistic fungal infection with a rapid&#44; fulminant course caused by fungi of the order Mucorales&#46; The most frequently isolated fungi are those of the genera <span class="elsevierStyleItalic">Rhizopus</span>&#44; <span class="elsevierStyleItalic">Mucor</span>&#44; and <span class="elsevierStyleItalic">Rhizomucor&#46;</span><a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">2</span></a> There is some debate as to whether this group of infections should be described as zygomycosis or mucormycosis&#44; but use of the latter term is supported by the results of molecular studies&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Distinct clinical forms of mucormycosis are described&#46; The most common is rhinocerebral mucormycosis&#44; followed by pulmonary&#44; gastrointestinal&#44; cutaneous&#44; and disseminated forms&#46; The cutaneous form &#40;10&#37;&#8211;19&#37; of cases&#41; is the result of direct inoculation of the spores into the dermis or direct contact of the skin with contaminated material&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a> It can also be caused by traumatic injuries &#40;70&#37;&#41;&#44; surgical interventions &#40;15&#37;&#41;&#44; burns &#40;3&#37;&#41;&#44; and&#44; in the case of nosocomial infections&#44; contact with contaminated material such as sheets&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">5</span></a> intravenous lines&#44; adhesives&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">6</span></a> or wooden tongue depressors&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">7</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Mucormycosis predominantly affects immunocompromised patients&#46; The majority of reported cases have involved patients with blood or metabolic disorders&#46; Our patient was receiving high-dose intravenous corticosteroids for the treatment of an underlying neurological condition&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The typical clinical picture consists of an erythematous&#44; edematous plaque with central ulceration that progresses rapidly and may affect subcutaneous tissue&#44; muscle&#44; and bone&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">8</span></a> Atypical clinical presentations include that of our patient&#44; in whom the aforementioned clinical picture was accompanied by eczematous lesions&#46;<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">6&#44;9</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">It is important to establish diagnosis early&#46; This requires a high level of clinical suspicion&#44; especially in cases with atypical presentations&#44; and the use of early diagnosis techniques such as direct examination of samples treated with KOH or calcofluor-white&#46; A presumptive diagnosis can be established and early treatment initiated based on identification of the characteristic hyphae of Mucorales&#46; The hyphae are wide &#40;5&#8211;15<span class="elsevierStyleHsp" style=""></span>&#956;m in diameter&#41;&#44; septate&#44; and branched at a 45&#176; angle&#46; Microbiological culture&#44; on which definitive diagnosis is based&#44; can take several days&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">10</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In both children and adults&#44; extensive surgical debridement and the administration of systemic antifungals &#40;liposomal amphotericin B&#44; 5&#8211;10<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#41; is the treatment of choice&#46; Once stabilized&#44; patients can be treated with oral posaconazole or isavuconazole&#44; either alone or in combination with systemic antifungals&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">11&#44;12</span></a> The duration of treatment has not been clearly established&#44; but usually spans several weeks&#44; until clinical resolution is achieved and the signs and symptoms of infection have disappeared&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The mortality rate associated with primary cutaneous mucormycosis ranges from 4&#37; to 10&#37;&#44; and only 3&#37; of cases progress to disseminated infection&#44; mainly in patients with risk factors &#40;in whom the mortality rate increases to 83&#37;&#8211;94&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The case described here illustrates the importance of the recognition and early diagnosis of cutaneous mucormycosis in order to quickly institute appropriate treatment&#44; which is an important determinant of the clinical course of this infectious disease&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Case and Research Letters
Primary Cutaneous Mucormycosis Due to Rhizopus arrhizus in an 8-Year-Old Girl
Mucormicosis cutánea primaria por Rhizopus arrhizus en una niña de 8 años
M.F. Albízuri-Prado
Autor para correspondencia
faprado8@gmail.com

Corresponding author.
, A. Sánchez-Orta, A. Rodríguez-Bandera, M. Feito-Rodríguez
Servicio de Dermatología, Hospital universitario La Paz, Madrid, España
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Given the acute course and the presence of an underlying mass&#44; a deep biopsy was performed&#44; revealing the presence of foul-smelling fatty tissue with a putrefied appearance &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41;&#44; suggestive of a necrotic infection&#46; A sample was taken for microbiological analysis and calcofluor-white staining and fungal culture were ordered&#46; Direct observation revealed the presence of numerous broad&#44; branched&#44; aseptate hyphae compatible with mucormycosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; After extensive early surgical resection liposomal amphotericin B treatment was instituted and corticosteroid treatment gradually withdrawn&#46; Histology revealed intense dermal necrosis and blood vessel obstruction by numerous fungal structures &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Diagnosis was confirmed based on the results of the fungal culture&#44; from which <span class="elsevierStyleItalic">Rhizopus arrhizus</span> was isolated following ethanol-formic acid extraction and MALDI-TOF &#40;matrix-assisted laser desorption&#47;ionization &#8211; time-of-flight&#41; mass spectrometry&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">1</span></a> After lesion spread was ruled out the patient was diagnosed with primary cutaneous mucormycosis due to <span class="elsevierStyleItalic">R&#46; arrhizus</span>&#46; The early initiation of treatment resulted in a favorable response and progressive improvement of the patient&#39;s clinical picture&#46; No new lesions appeared nor were other organs affected&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Mucormycosis is an opportunistic fungal infection with a rapid&#44; fulminant course caused by fungi of the order Mucorales&#46; The most frequently isolated fungi are those of the genera <span class="elsevierStyleItalic">Rhizopus</span>&#44; <span class="elsevierStyleItalic">Mucor</span>&#44; and <span class="elsevierStyleItalic">Rhizomucor&#46;</span><a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">2</span></a> There is some debate as to whether this group of infections should be described as zygomycosis or mucormycosis&#44; but use of the latter term is supported by the results of molecular studies&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Distinct clinical forms of mucormycosis are described&#46; The most common is rhinocerebral mucormycosis&#44; followed by pulmonary&#44; gastrointestinal&#44; cutaneous&#44; and disseminated forms&#46; The cutaneous form &#40;10&#37;&#8211;19&#37; of cases&#41; is the result of direct inoculation of the spores into the dermis or direct contact of the skin with contaminated material&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a> It can also be caused by traumatic injuries &#40;70&#37;&#41;&#44; surgical interventions &#40;15&#37;&#41;&#44; burns &#40;3&#37;&#41;&#44; and&#44; in the case of nosocomial infections&#44; contact with contaminated material such as sheets&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">5</span></a> intravenous lines&#44; adhesives&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">6</span></a> or wooden tongue depressors&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">7</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Mucormycosis predominantly affects immunocompromised patients&#46; The majority of reported cases have involved patients with blood or metabolic disorders&#46; Our patient was receiving high-dose intravenous corticosteroids for the treatment of an underlying neurological condition&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The typical clinical picture consists of an erythematous&#44; edematous plaque with central ulceration that progresses rapidly and may affect subcutaneous tissue&#44; muscle&#44; and bone&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">8</span></a> Atypical clinical presentations include that of our patient&#44; in whom the aforementioned clinical picture was accompanied by eczematous lesions&#46;<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">6&#44;9</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">It is important to establish diagnosis early&#46; This requires a high level of clinical suspicion&#44; especially in cases with atypical presentations&#44; and the use of early diagnosis techniques such as direct examination of samples treated with KOH or calcofluor-white&#46; A presumptive diagnosis can be established and early treatment initiated based on identification of the characteristic hyphae of Mucorales&#46; The hyphae are wide &#40;5&#8211;15<span class="elsevierStyleHsp" style=""></span>&#956;m in diameter&#41;&#44; septate&#44; and branched at a 45&#176; angle&#46; Microbiological culture&#44; on which definitive diagnosis is based&#44; can take several days&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">10</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In both children and adults&#44; extensive surgical debridement and the administration of systemic antifungals &#40;liposomal amphotericin B&#44; 5&#8211;10<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#41; is the treatment of choice&#46; Once stabilized&#44; patients can be treated with oral posaconazole or isavuconazole&#44; either alone or in combination with systemic antifungals&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">11&#44;12</span></a> The duration of treatment has not been clearly established&#44; but usually spans several weeks&#44; until clinical resolution is achieved and the signs and symptoms of infection have disappeared&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The mortality rate associated with primary cutaneous mucormycosis ranges from 4&#37; to 10&#37;&#44; and only 3&#37; of cases progress to disseminated infection&#44; mainly in patients with risk factors &#40;in whom the mortality rate increases to 83&#37;&#8211;94&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">4</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The case described here illustrates the importance of the recognition and early diagnosis of cutaneous mucormycosis in order to quickly institute appropriate treatment&#44; which is an important determinant of the clinical course of this infectious disease&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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