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crateriform lesion&#44; whose center was filled with a predominantly orthokeratotic keratin plug&#44; with some areas of parakeratosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#41;&#46; The surrounding epithelium showed mild irregular acanthosis containing abundant ground-glass cytoplasm cells&#44; displaying minimal atypia and pleomorphism at the basal layer &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>B&#41;&#46; Intratumoral neutrophilic microabscesses were observed&#44; alongside with lymphocytic infiltrate in the underlying dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>C&#41;&#46; These findings were consistent with the diagnosis of keratoacanthoma&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">What is your diagnosis&#63;</span><p id="par0015" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">a&#41;</span><p id="par0020" class="elsevierStylePara elsevierViewall">Muir-Torre syndrome</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">b&#41;</span><p id="par0025" class="elsevierStylePara elsevierViewall">Follicular lichen planus</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">c&#41;</span><p id="par0030" class="elsevierStylePara elsevierViewall">Pityriasis rubra pilaris</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">d&#41;</span><p id="par0035" class="elsevierStylePara elsevierViewall">Generalized eruptive keratoacanthomas of Grzybowski</p></li></ul></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">Correlating the clinical and histological findings&#44; the diagnosis of generalized eruptive keratoacanthomas of Grzybowski &#40;GEKA&#41; was established&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">GEKA is a rare variant of multiple keratoacanthomas&#44; with only around 40 cases reported&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Its onset most commonly occurs between the fifth and seventh decades&#44; without sex predilection&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Unlike other types of multiple keratoacanthomas&#44; such as Ferguson-Smith or Witten and Zak&#44; all cases are sporadic&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It is characterized by a relatively sudden onset of hundreds to thousands of small &#40;1&#8722;3 millimeters&#41;&#44; skin-colored to erythematous papules with a tendency to coalesce&#46; Some show a central umbilication containing a horny keratotic plug&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> They predominate on sun-exposed areas&#44; 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Nonetheless&#44; several factors have been proposed to play a role in its pathogenesis&#44; including trauma&#44; immunological abnormalities&#44; ultraviolet radiation&#44; chemical carcinogens&#44; and viruses&#44; particularly human papillomavirus&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;6</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">GEKA is a chronic and progressive disease for which none of the described therapeutic options has been entirely satisfactory&#46; Surgical excision&#44; cryotherapy&#44; laser ablation&#44; and radiotherapy are restricted to bigger lesions&#44; being impractical for the remaining&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Topical agents&#44; such as 5-fluorouracil&#44; corticosteroids&#44; imiquimod&#44; and tretinoin&#44; also demonstrated little benefit&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Systemic therapies&#44; especially oral retinoids&#44; are the preferred approach&#46; Other reported options include methotrexate&#44; cyclophosphamide&#44; corticosteroids&#44; and erlotinib&#44; with variable results&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The present patient started acitretin 25&#8239;mg daily&#44; with a complete resolution of pruritus and regression of numerous lesions &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The dosage was then decreased over the next five months&#46; She is currently on 10&#8239;mg three times per week and&#44; so far&#44; no new lesions have emerged&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Financial support</span><p id="par0075" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Authors&#39; contributions</span><p id="par0080" class="elsevierStylePara elsevierViewall">Maria Relvas&#58; Drafting and editing of the manuscript&#59; collection&#44; analysis&#44; and interpretation of data&#59; participation in the design of the study&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Joana Calv&#227;o&#58; Collection&#44; analysis&#44; and interpretation of data&#59; participation in the design of the study&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">In&#234;s Coutinho&#58; Collection&#44; analysis&#44; and interpretation of data&#59; participation in the design of the study&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Jos&#233; Carlos Cardoso&#58; Intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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What is your diagnosis?
Case for diagnosis. A pruritic eruption of keratotic papules over the face and neck
Maria Relvas
Autor para correspondência
mariavrelvas@gmail.com

Corresponding author.
, Joana Calvão, Inês Coutinho, José Carlos Cardoso
Department of Dermatology, Coimbra University Hospital Center, Coimbra, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case report</span><p id="par0005" class="elsevierStylePara elsevierViewall">An 83-year-old female presented with a three-month history of extremely pruritic&#44; multiple&#44; skin-coloured to erythematous to brownish&#44; millimetric papules&#44; with a keratotic center&#44; sometimes coalescing into verrucous plaques &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The lesions were distributed bilaterally over the face and neck&#44; without mucosal involvement&#46; There was no deterioration of her general condition&#46; She had no relevant personal or family medical history&#46; Laboratory findings included a complete blood count and renal and liver function tests&#44; which were all normal&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Incisional cutaneous biopsy revealed a well-demarcated&#44; crateriform lesion&#44; whose center was filled with a predominantly orthokeratotic keratin plug&#44; with some areas of parakeratosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#41;&#46; The surrounding epithelium showed mild irregular acanthosis containing abundant ground-glass cytoplasm cells&#44; displaying minimal atypia and pleomorphism at the basal layer &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>B&#41;&#46; Intratumoral neutrophilic microabscesses were observed&#44; alongside with lymphocytic infiltrate in the underlying dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>C&#41;&#46; These findings were consistent with the diagnosis of keratoacanthoma&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">What is your diagnosis&#63;</span><p id="par0015" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">a&#41;</span><p id="par0020" class="elsevierStylePara elsevierViewall">Muir-Torre syndrome</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">b&#41;</span><p id="par0025" class="elsevierStylePara elsevierViewall">Follicular lichen planus</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">c&#41;</span><p id="par0030" class="elsevierStylePara elsevierViewall">Pityriasis rubra pilaris</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">d&#41;</span><p id="par0035" class="elsevierStylePara elsevierViewall">Generalized eruptive keratoacanthomas of Grzybowski</p></li></ul></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">Correlating the clinical and histological findings&#44; the diagnosis of generalized eruptive keratoacanthomas of Grzybowski &#40;GEKA&#41; was established&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">GEKA is a rare variant of multiple keratoacanthomas&#44; with only around 40 cases reported&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Its onset most commonly occurs between the fifth and seventh decades&#44; without sex predilection&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Unlike other types of multiple keratoacanthomas&#44; such as Ferguson-Smith or Witten and Zak&#44; all cases are sporadic&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It is characterized by a relatively sudden onset of hundreds to thousands of small &#40;1&#8722;3 millimeters&#41;&#44; skin-colored to erythematous papules with a tendency to coalesce&#46; Some show a central umbilication containing a horny keratotic plug&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> They predominate on sun-exposed areas&#44; namely the face and neck&#44; leading to masked facies and ectropion&#46; Furthermore&#44; sun-protected sites&#44; including mucous membranes&#44; the trunk and intertriginous areas are commonly affected&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> The lesions tend to be severely pruritic and koebnerization is frequently reported&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The histopathological findings are similar to those observed in solitary keratoacanthomas&#46; These comprise an exo-endophytic&#44; well-demarcated lesion&#44; with an invaginating keratin filled-crater&#44; surrounded by glassy eosinophilic keratinocytes&#46; Unlike squamous cell carcinoma&#44; cytologic atypia is usually minimal&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Sometimes the lesions may show features of proliferative or regressive stages of keratoacanthomas&#44; so that histological diagnosis can easily be missed&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">The exact etiology of GEKA is still unknown&#46; Nonetheless&#44; several factors have been proposed to play a role in its pathogenesis&#44; including trauma&#44; immunological abnormalities&#44; ultraviolet radiation&#44; chemical carcinogens&#44; and viruses&#44; particularly human papillomavirus&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;6</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">GEKA is a chronic and progressive disease for which none of the described therapeutic options has been entirely satisfactory&#46; Surgical excision&#44; cryotherapy&#44; laser ablation&#44; and radiotherapy are restricted to bigger lesions&#44; being impractical for the remaining&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Topical agents&#44; such as 5-fluorouracil&#44; corticosteroids&#44; imiquimod&#44; and tretinoin&#44; also demonstrated little benefit&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Systemic therapies&#44; especially oral retinoids&#44; are the preferred approach&#46; Other reported options include methotrexate&#44; cyclophosphamide&#44; corticosteroids&#44; and erlotinib&#44; with variable results&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The present patient started acitretin 25&#8239;mg daily&#44; with a complete resolution of pruritus and regression of numerous lesions &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The dosage was then decreased over the next five months&#46; She is currently on 10&#8239;mg three times per week and&#44; so far&#44; no new lesions have emerged&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Financial support</span><p id="par0075" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Authors&#39; contributions</span><p id="par0080" class="elsevierStylePara elsevierViewall">Maria Relvas&#58; Drafting and editing of the manuscript&#59; collection&#44; analysis&#44; and interpretation of data&#59; participation in the design of the study&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Joana Calv&#227;o&#58; Collection&#44; analysis&#44; and interpretation of data&#59; participation in the design of the study&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">In&#234;s Coutinho&#58; Collection&#44; analysis&#44; and interpretation of data&#59; participation in the design of the study&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Jos&#233; Carlos Cardoso&#58; Intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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