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Cited by 8 publications
(13 citation statements)
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“…Our patient also manifested thick transversely folded scalp skin with scarring alopecia, indicating scalp involvement. Scarring alopecia occurring in PDP is considered a rare presentation and was once reported without histopathological evaluation [7]. However, our case and a previously reported case have brought attention to the association between scalp involvement of PDP and the development of scarring alopecia.…”
Section: Answermentioning
confidence: 57%
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“…Our patient also manifested thick transversely folded scalp skin with scarring alopecia, indicating scalp involvement. Scarring alopecia occurring in PDP is considered a rare presentation and was once reported without histopathological evaluation [7]. However, our case and a previously reported case have brought attention to the association between scalp involvement of PDP and the development of scarring alopecia.…”
Section: Answermentioning
confidence: 57%
“…Scleromyxedema manifests generalized sclerotic skin, sclerodactyly, and a circulating monoclonal gammopathy while leukemia cutis commonly presents systemic symptoms and abnormal complete blood count results. To date, conditions that have been reported with pachyderma on the face and scarring alopecia include PDP [7], multiple familial trichoepitheliomas [13], and folliculotropic mycosis fungoides [14, 15]. Table 1 summarizes and compares the characteristics of the 3 diseases.…”
Section: Answermentioning
confidence: 99%
“…Patients with PDP demonstrate increased plasma levels of several substances, including osteocalcin, endothelin-1, beta-thromboglobulin, platelet-derived growth factor, von Willebrand factor, and vascular endothelial growth factor, which lead to increased fibroblast proliferation in the bone marrow and skin biopsies, associated with diffuse dermal endothelial hyperplasia, partial occlusion of the vascular lumen, pericapillary lymphohistiocytic infiltrate and thickening and packaging of collagen fibers. 3,6 PDP has recently been mapped to band 4q33-q34. Two susceptibility genes have been identified underlying PDP: HPGD encoding 15-hydroxyprostaglandin dehydrogenase, the main enzyme of prostaglandin degradation, and SLCO2A1, involved in the transportation of prostaglandin.…”
Section: Discussionmentioning
confidence: 99%
“…Sporadic patients were also noted which could represent new mutations or segregation of a dominant mutation with incomplete penetrance and mild expression in the parents. 3 Primary familial PDP may be expressed in 3 general ways: (a) the complete form which presents with the fullblown phenotype; (b) the incomplete form which has no pachydermia; and the (c) "forme fruste" which has prominent pachydermia and minimal skeletal changes. 1,3 In the latter form, thickening of the skin, coarsening of facial features akin to leonine facies of leprosy, and cutis verticis gyrata of the scalp are all present, giving the patient an acromegaloid appearance, while periosteal changes are either absent or minimal at the time of examination.…”
Section: Discussionmentioning
confidence: 99%
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