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accompanied by erythema and desquamation &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Similar lesions were present in the vulvar area &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The physical examination was otherwise completely normal&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">A biopsy of a vulvar lesion revealed a dense lymphohistiocytic perivascular infiltrate in the upper dermis as well as the formation of noncaseating epithelioid granulomas &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The epidermis was spared&#46; Ziehl-Neelsen and periodic acid-Schiff stains were negative&#46; The presence of <span class="elsevierStyleItalic">Demodex folliculorum</span> was not detected in the follicles&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">A complete blood count and biochemistry profile revealed no abnormalities&#46; Levels of angiotensin-converting enzyme &#40;ACE&#41; and immunoglobulins were normal&#46; A Mantoux test was negative and a chest radiograph showed no anomalies&#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">Childhood granulomatous periorificial dermatitis &#40;CGPD&#41;&#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">Topical corticosteroids were suspended&#46; Treatment with oral erythromycin was started&#44; but little improvement was noted&#46; The patient was switched to oral metronidazole &#40;250<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#41;&#46; A striking improvement was noted after 2 weeks and the lesions had resolved completely at 4 weeks&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Childhood granulomatous periorificial dermatitis &#40;CGPD&#41; is a benign&#44; self-limited skin disease that has many characteristics in common with the perioral dermatitis typically seen in middle-aged women&#46; The disease was first described in 1970 by Gianotti&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> who reported a case series of 5 children with monomorphous papules that resembled perioral dermatitis&#46; Other names have been used to describe this entity&#44; including facial Afro-Caribbean cutaneous eruption &#40;FACE&#41;&#44; sarcoid-like granulomatous dermatitis&#44; and Gianotti-type perioral dermatitis&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> The term <span class="elsevierStyleItalic">childhood granulomatous periorificial dermatitis</span>&#44; first used by Urbatsch et al&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> in 1989&#44; is currently the most widely accepted term because it most accurately encompasses the characteristics of the entity&#46; CGPD usually affects prepubertal children&#44; and the incidence of the disease may be higher in black patients&#44; although publication bias may account for the difference&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">CGPD is characterized by the appearance of lesions in the form of monomorphous skin-colored papules distributed across the periorificial regions of the face&#44; which may be accompanied by erythema and desquamation&#46; Unlike perioral dermatitis&#44; CGPD generally does not manifest with pustules&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Extrafacial involvement can include the perivulvar region&#44; the neck&#44; the upper trunk&#44; and the limbs&#46; The lesions are usually asymptomatic and tend to resolve without scarring&#46; The etiology of CGPD is unknown&#44; but it appears that topical corticosteroids&#44; especially fluorinated corticosteroids&#44; can trigger or exacerbate the process&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Histologic findings show a perivascular and perifollicular lymphohistiocytic infiltrate and the formation of epithelioid granulomas that can be either caseating or noncaseating&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a> The presence of granulomas is not a necessary criterion for diagnosis&#46; The differential diagnosis should include rosacea&#44; acne vulgaris&#44; sarcoidosis&#44; Blau syndrome&#44; lupus miliaris disseminatus faciei&#44; candidiasis&#44; angular cheilitis&#44; atopic dermatitis&#44; demodicidosis&#44; and benign cephalic histiocytosis&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> It is important to ask about constitutional symptoms&#44; joint pain&#44; and respiratory involvement&#46; A laboratory workup including angiotensin-converting enzyme levels and a chest radiograph should be ordered in order to rule out sarcoidosis&#46; In the case of our patient&#44; it was possible to rule out these possible diagnoses on the basis of the medical history and the additional test results&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Treatment options include the suspension of topical corticosteroids and the application of various topical antibiotics&#44; including erythromycin and 0&#46;75&#37; metronidazole&#44; with variable results&#46; In patients with extensive or refractory lesions&#44; systemic treatment with erythromycin or doxycycline is recommended &#40;the latter in children older than 8 years of age&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> A recent case report described the successful treatment of CGPD with oral metronidazole&#44; as in our patient&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p></span></span>"
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Case for Diagnosis
Periorificial Lesions in a Young Girl
Lesiones periorificiales en una niña
A. Mayor-Ibarguren
Corresponding author
andermayor@gmail.com

Corresponding author.
, R. Maseda-Pedrero, M. Feito-Rodríguez
Servicio de Dermatología, Hospital Universitario La Paz, 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">An 8-year-old girl with no relevant personal or family medical history presented with asymptomatic facial lesions that had appeared several months earlier and had not responded to topical corticosteroids&#46; The patient had no history of atopic dermatitis&#46; She was in good general health&#44; with no joint pain&#44; fever&#44; abdominal pain&#44; or weight loss&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">The patient presented numerous firm&#44; skin-colored&#44; monomorphous papules distributed in periorificial areas &#40;perioral&#44; perinasal&#44; periocular&#41;&#44; accompanied by erythema and desquamation &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Similar lesions were present in the vulvar area &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The physical examination was otherwise completely normal&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">A biopsy of a vulvar lesion revealed a dense lymphohistiocytic perivascular infiltrate in the upper dermis as well as the formation of noncaseating epithelioid granulomas &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The epidermis was spared&#46; Ziehl-Neelsen and periodic acid-Schiff stains were negative&#46; The presence of <span class="elsevierStyleItalic">Demodex folliculorum</span> was not detected in the follicles&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">A complete blood count and biochemistry profile revealed no abnormalities&#46; Levels of angiotensin-converting enzyme &#40;ACE&#41; and immunoglobulins were normal&#46; A Mantoux test was negative and a chest radiograph showed no anomalies&#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">Childhood granulomatous periorificial dermatitis &#40;CGPD&#41;&#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">Topical corticosteroids were suspended&#46; Treatment with oral erythromycin was started&#44; but little improvement was noted&#46; The patient was switched to oral metronidazole &#40;250<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#41;&#46; A striking improvement was noted after 2 weeks and the lesions had resolved completely at 4 weeks&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Childhood granulomatous periorificial dermatitis &#40;CGPD&#41; is a benign&#44; self-limited skin disease that has many characteristics in common with the perioral dermatitis typically seen in middle-aged women&#46; The disease was first described in 1970 by Gianotti&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> who reported a case series of 5 children with monomorphous papules that resembled perioral dermatitis&#46; Other names have been used to describe this entity&#44; including facial Afro-Caribbean cutaneous eruption &#40;FACE&#41;&#44; sarcoid-like granulomatous dermatitis&#44; and Gianotti-type perioral dermatitis&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> The term <span class="elsevierStyleItalic">childhood granulomatous periorificial dermatitis</span>&#44; first used by Urbatsch et al&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> in 1989&#44; is currently the most widely accepted term because it most accurately encompasses the characteristics of the entity&#46; CGPD usually affects prepubertal children&#44; and the incidence of the disease may be higher in black patients&#44; although publication bias may account for the difference&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">CGPD is characterized by the appearance of lesions in the form of monomorphous skin-colored papules distributed across the periorificial regions of the face&#44; which may be accompanied by erythema and desquamation&#46; Unlike perioral dermatitis&#44; CGPD generally does not manifest with pustules&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Extrafacial involvement can include the perivulvar region&#44; the neck&#44; the upper trunk&#44; and the limbs&#46; The lesions are usually asymptomatic and tend to resolve without scarring&#46; The etiology of CGPD is unknown&#44; but it appears that topical corticosteroids&#44; especially fluorinated corticosteroids&#44; can trigger or exacerbate the process&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Histologic findings show a perivascular and perifollicular lymphohistiocytic infiltrate and the formation of epithelioid granulomas that can be either caseating or noncaseating&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;3</span></a> The presence of granulomas is not a necessary criterion for diagnosis&#46; The differential diagnosis should include rosacea&#44; acne vulgaris&#44; sarcoidosis&#44; Blau syndrome&#44; lupus miliaris disseminatus faciei&#44; candidiasis&#44; angular cheilitis&#44; atopic dermatitis&#44; demodicidosis&#44; and benign cephalic histiocytosis&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> It is important to ask about constitutional symptoms&#44; joint pain&#44; and respiratory involvement&#46; A laboratory workup including angiotensin-converting enzyme levels and a chest radiograph should be ordered in order to rule out sarcoidosis&#46; In the case of our patient&#44; it was possible to rule out these possible diagnoses on the basis of the medical history and the additional test results&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Treatment options include the suspension of topical corticosteroids and the application of various topical antibiotics&#44; including erythromycin and 0&#46;75&#37; metronidazole&#44; with variable results&#46; In patients with extensive or refractory lesions&#44; systemic treatment with erythromycin or doxycycline is recommended &#40;the latter in children older than 8 years of age&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> A recent case report described the successful treatment of CGPD with oral metronidazole&#44; as in our patient&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p></span></span>"
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ISSN: 15782190
Original language: English
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Idiomas
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