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She attended the clinic because of an asymptomatic hyperpigmented lesion on the back that had been present since she was 9 years old&#46; Examination revealed several macular lesions with a homogeneous brown color and well-defined but irregular borders&#44; grouped in a segmental pattern on normal skin on the right flank and arm&#44; and the right side of the back and abdomen&#46; These lesions did not cross the midline of the trunk &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41;&#46; No caf&#233;-au-lait spots or neurofibromas were seen on unaffected skin&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The patient did not recall any rash or prior trauma of the affected area although the lesions did increase in size in the first years after onset&#46; She did not have any extracutaneous abnormalities or a family history of neurofibromatosis or similar skin lesions&#46; A biopsy was taken from the affected area of the back&#46; Histopathology showed an increase in cytoplasmic pigmentation of the cells of the basal epidermal layer &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; In view of the clinical and histologic characteristics&#44; a diagnosis of PCZH was made&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Rower et al&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> reported 5 patients with pigmentary abnormalities with certain characteristics in common that constituted the diagnostic criteria for PCZH&#46; These characteristics were uniform and cribriform brown macular hyperpigmentation with a zosteriform distribution&#59; a histologic pattern consistent with a mild increase in melanin pigment in cells of the basal layer and complete absence of nevus cells&#59; no history of rash&#44; injury&#44; or inflammation that could suggest postinflammatory hyperpigmentation&#59; onset after birth with gradual increase in extension&#59; and absence of other cutaneous or internal abnormalities&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">In 2012&#44; Cho et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> published demographic&#44; clinical&#44; and histopathologic data from 30 Korean patients with PCZH&#46; The authors observed a slight predominance of men&#44; with an age of onset of PCZH between birth and 54 years &#40;77&#37; between 1 and 15 years&#41;&#46; Only one individual reported a family history of similar lesions and none had associated extracutaneous congenital abnormalities&#46; In general&#44; the lesions were asymptomatic&#44; cribriform&#44; with a Blaschkoid distribution&#44; and located preferentially on the trunk and limbs with a slight tendency towards right-sided predominance&#46; The affected skin&#44; in comparison with adjacent skin&#44; showed increased pigmentation &#40;more melanin granules&#41; of the basal layer&#46; Nevus cells were not present and there were no significant differences in the number of melanocytes&#46; Likewise&#44; there were no differences in other skin disorders except for cases with pigmentary incontinency&#46; No effective treatment has been reported for PCZH&#46; Terms such as <span class="elsevierStyleItalic">reticulate hyperpigmentation of Iijima</span><a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and <span class="elsevierStyleItalic">reticulate zosteriform hyperpigmentation</span><a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> have been used to describe conditions with similar clinical and histopathologic findings&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Differential diagnosis of PCZH includes skin conditions that present with segmental hyperpigmented lesions such as linear and whorled nevoid hypermelanosis&#44;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;5&#44;8</span></a> caf&#233;-au-lait spots&#44;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> segmental pigmentation disorders with onset in the first months of life&#44;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> and others such as Becker nevus &#40;variant without hypertrichosis&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;8&#44;10</span></a> Linear and whorled nevoid hypermelanosis is a diffuse asymmetric hyperpigmentation in lines or swirls along the Blaschko lines&#46; The lesions appear at birth or within the first few weeks of life and then spread or darken during the first 2 years&#46; These lesions are sometimes associated with extracutaneous disorders&#44; particularly of neurologic&#44; cardiac&#44; and musculoskeletal nature&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;7&#44;8</span></a> Despite the diffuse pattern&#44; associated congenital abnormalities&#44; and the different age of onset compared to PCZH&#44; the clinical manifestations may sometimes overlap&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> and so some authors consider these conditions as part of spectrum of the same disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> Caf&#233;-au-lait spots are usually round or oval&#44; although they can follow a segmental distribution in segmental neurofibromatosis type 1 and have irregular borders in the McCune-Albright and Jaffe-Campanacci syndromes&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> Segmental pigmentation abnormalities appear in the first months of life as segmental hypopigmentation or hyperpigmentation&#44; mainly on the trunk&#46; They follow a block-like pattern with sharp confinement at the midline&#44; particularly anteriorly&#44; and can be associated with extracutaneous abnormalities&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In conclusion&#44; the findings in our case are similar to those reported by Cho et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Bearing in mind the absence of associated conditions and the lack of therapeutic options&#44; it is likely that PCZH is in fact more common than the limited number of reports in the literature would have us believe&#46;</p></span>"
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Case and Research Letters
Progressive Cribriform and Zosteriform Hyperpigmentation
Hiperpigmentación zosteriforme y cribiforme progresiva
B. Monteagudoa,
Autor para correspondencia
, Á. León-Mateosb, F. Campo-Cerecedoc, M. Cabanillasa
a Servicio de Dermatología, Hospital Arquitecto Marcide, Área Sanitaria de Ferrol, SERGAS, Ferrol, A Coruña, Spain
b Servicio de Dermatología, Hospital POVISA, Vigo, Pontevedra, Spain
c Servicio de Anatomía Patológica, Hospital Arquitecto Marcide, Área Sanitaria de Ferrol, SERGAS, Ferrol, A Coruña, Spain
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She attended the clinic because of an asymptomatic hyperpigmented lesion on the back that had been present since she was 9 years old&#46; Examination revealed several macular lesions with a homogeneous brown color and well-defined but irregular borders&#44; grouped in a segmental pattern on normal skin on the right flank and arm&#44; and the right side of the back and abdomen&#46; These lesions did not cross the midline of the trunk &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41;&#46; No caf&#233;-au-lait spots or neurofibromas were seen on unaffected skin&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The patient did not recall any rash or prior trauma of the affected area although the lesions did increase in size in the first years after onset&#46; She did not have any extracutaneous abnormalities or a family history of neurofibromatosis or similar skin lesions&#46; A biopsy was taken from the affected area of the back&#46; Histopathology showed an increase in cytoplasmic pigmentation of the cells of the basal epidermal layer &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; In view of the clinical and histologic characteristics&#44; a diagnosis of PCZH was made&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Rower et al&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> reported 5 patients with pigmentary abnormalities with certain characteristics in common that constituted the diagnostic criteria for PCZH&#46; These characteristics were uniform and cribriform brown macular hyperpigmentation with a zosteriform distribution&#59; a histologic pattern consistent with a mild increase in melanin pigment in cells of the basal layer and complete absence of nevus cells&#59; no history of rash&#44; injury&#44; or inflammation that could suggest postinflammatory hyperpigmentation&#59; onset after birth with gradual increase in extension&#59; and absence of other cutaneous or internal abnormalities&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">In 2012&#44; Cho et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> published demographic&#44; clinical&#44; and histopathologic data from 30 Korean patients with PCZH&#46; The authors observed a slight predominance of men&#44; with an age of onset of PCZH between birth and 54 years &#40;77&#37; between 1 and 15 years&#41;&#46; Only one individual reported a family history of similar lesions and none had associated extracutaneous congenital abnormalities&#46; In general&#44; the lesions were asymptomatic&#44; cribriform&#44; with a Blaschkoid distribution&#44; and located preferentially on the trunk and limbs with a slight tendency towards right-sided predominance&#46; The affected skin&#44; in comparison with adjacent skin&#44; showed increased pigmentation &#40;more melanin granules&#41; of the basal layer&#46; Nevus cells were not present and there were no significant differences in the number of melanocytes&#46; Likewise&#44; there were no differences in other skin disorders except for cases with pigmentary incontinency&#46; No effective treatment has been reported for PCZH&#46; Terms such as <span class="elsevierStyleItalic">reticulate hyperpigmentation of Iijima</span><a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and <span class="elsevierStyleItalic">reticulate zosteriform hyperpigmentation</span><a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> have been used to describe conditions with similar clinical and histopathologic findings&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Differential diagnosis of PCZH includes skin conditions that present with segmental hyperpigmented lesions such as linear and whorled nevoid hypermelanosis&#44;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;5&#44;8</span></a> caf&#233;-au-lait spots&#44;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> segmental pigmentation disorders with onset in the first months of life&#44;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> and others such as Becker nevus &#40;variant without hypertrichosis&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;8&#44;10</span></a> Linear and whorled nevoid hypermelanosis is a diffuse asymmetric hyperpigmentation in lines or swirls along the Blaschko lines&#46; The lesions appear at birth or within the first few weeks of life and then spread or darken during the first 2 years&#46; These lesions are sometimes associated with extracutaneous disorders&#44; particularly of neurologic&#44; cardiac&#44; and musculoskeletal nature&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;7&#44;8</span></a> Despite the diffuse pattern&#44; associated congenital abnormalities&#44; and the different age of onset compared to PCZH&#44; the clinical manifestations may sometimes overlap&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> and so some authors consider these conditions as part of spectrum of the same disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> Caf&#233;-au-lait spots are usually round or oval&#44; although they can follow a segmental distribution in segmental neurofibromatosis type 1 and have irregular borders in the McCune-Albright and Jaffe-Campanacci syndromes&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> Segmental pigmentation abnormalities appear in the first months of life as segmental hypopigmentation or hyperpigmentation&#44; mainly on the trunk&#46; They follow a block-like pattern with sharp confinement at the midline&#44; particularly anteriorly&#44; and can be associated with extracutaneous abnormalities&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In conclusion&#44; the findings in our case are similar to those reported by Cho et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Bearing in mind the absence of associated conditions and the lack of therapeutic options&#44; it is likely that PCZH is in fact more common than the limited number of reports in the literature would have us believe&#46;</p></span>"
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