ABSTRACT
Objective@#To analyze clinical and pathological features of papular elastorrhexis.@*Methods@#Clinical data were collected from 22 patients with confirmed papular elastorrhexis in Hospital for Skin Diseases, Chinese Academy of Medical Sciences and Pekin Union Medical College from September 2006 to May 2018. Clinical manifestations, pathological findings and follow-up results were retrospectively analyzed.@*Results@#The average age of onset of the 22 patients was 5.7 years (range: 1 - 10 years) , and the male to female ratio was 4.5∶1. The average duration from the occurrence of disease to the confirmation of diagnosis was 1.5 years, and no definite etiology was found. The patients had no itching or pain sensation. Skin lesions were soft, slightly elevated, well-circumscribed, round, oval or polygonal-shaped, white papules with diameters of 1 - 10 mm, and wrinkles appeared on the surface of the papule when the papule was pushed towards its center. Among the 22 patients, 16 (73%) presented with scattered lesions, 13 (59%) had less than 5 papules, and lesions were located in the trunk in 21 (95%) . Histopathological examination of skin lesions in 8 patients showed no obvious increase of collagen fibers in the superficial and middle dermis, which were normally arranged with slightly widened spaces between them. Elastic fiber staining showed that elastic fibers disappeared or were dissociated focally in the superficial and middle dermis. After confirmed diagnosis, the 22 patients received no treatment. In 18 patients, skin lesions did not continue to expand after onset, and no new skin lesions occurred. Skin lesions were slightly enlarged, but remained steady thereafter in 4 patients. Sixteen patients achieved partial remission.@*Conclusions@#Papular elastorrhexis is a rare skin disorder of elastic fibers that occurs predominantly during childhood and adolescence, and its diagnosis relies on clinical manifestations combined with histopathological findings. No special treatment is needed and the prognosis is good.
ABSTRACT
Objective To analyze clinical and pathological features of papular elastorrhexis.Methods Clinical data were collected from 22 patients with confirmed papular elastorrhexis in Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Pekin Union Medical College from September 2006 to May 2018.Clinical manifestations,pathological findings and follow-up results were retrospectively analyzed.Results The average age of onset of the 22 patients was 5.7 years (range:1-10 years),and the male to female ratio was 4.5:1.The average duration from the occurrence of disease to the confirmation of diagnosis was 1.5 years,and no definite etiology was found.The patients had no itching or pain sensation.Skin lesions were soft,slightly elevated,well-circumscribed,round,oval or polygonal-shaped,white papules with diameters of 1-10 mm,and wrinkles appeared on the surface of the papule when the papule was pushed towards its center.Among the 22 patients,16 (73％) presented with scattered lesions,13 (59％)had less than 5 papules,and lesions were located in the trunk in 21 (95％).Histopathological examination of skin lesions in 8 patients showed no obvious increase of collagen fibers in the superficial and middle dermis,which were normally arranged with slightly widened spaces between them.Elastic fiber staining showed that elastic fibers disappeared or were dissociated focally in the superficial and middle dermis.After confirmed diagnosis,the 22 patients received no treatment.In 18 patients,skin lesions did not continue to expand after onset,and no new skin lesions occurred.Skin lesions were slightly enlarged,but remained steady thereafter in 4 patients.Sixteen patients achieved partial remission.Conclusions Papular elastorrhexis is a rare skin disorder of elastic fibers that occurs predominantly during childhood and adolescence,and its diagnosis relies on clinical manifestations combined with histopathological findings.No special treatment is needed and the prognosis is good.
ABSTRACT
Papular elastorrhexis is a rare entity, possibly a form of connective tissue nevi, characterized by asymptomatic white papules on the trunk and extremities first appearing during childhood or adolescence. Histopathologically, the elastic fibers are decreased and may appear in thin and fragmented forms. This rare condition has clinical and histological findings that overlap with those of other connective tissue nevi, making diagnosis a challenge for dermatologists. Herein we report the case of a 27-year-old Korean female presenting with asymptomatic 2~5 mm sized whitish papules on both extremities and showing histopathologic findings of increased layers of collagen fibers and decreased and fragmented elastic fibers in the dermis.
Subject(s)
Adolescent , Adult , Female , Humans , Collagen , Connective Tissue , Dermis , Diagnosis , Diagnosis, Differential , Elastic Tissue , Extremities , NevusABSTRACT
BACKGROUND: Papular elastorrhexis (PE), eruptive collagenoma (EC), and nevus anelasticus (NA) are described as multiple small papules with decrease, fragmentation, or lack of dermal elastic fibers. These diseases are suggested to be the same entity. The change of collagen fibers in the conditions has not been addressed to date. OBJECTIVE: We compared the clinical features of the 3 diseases and investigated changes in the collagen fibers involved. METHODS: Twenty-four cases of PE, 12 cases of EC, and 2 cases of NA found in PubMed and the Korean database were reviewed. Changes in dermal collagen fibers in 10 cases with histological figures were investigated. RESULTS: There were significant similarities between the 3 entities in terms of their clinical features. Four patients with PE and 2 with EC with fine, dense collagen fibers were women who had multiple white to hypopigmented, slightly indurated to firm, millimeter-size papules on the trunk and/or extremities that progressed gradually after developing in the patients' first to third decades. CONCLUSION: The 3 conditions are the same clinical entity in our opinion; such cases with fine, dense collagen manifest typical features.
Subject(s)
Female , Humans , Collagen , Elastic Tissue , Extremities , NevusABSTRACT
BACKGROUND: Papular elastorrhexis (PE), eruptive collagenoma (EC), and nevus anelasticus (NA) are described as multiple small papules with decrease, fragmentation, or lack of dermal elastic fibers. These diseases are suggested to be the same entity. The change of collagen fibers in the conditions has not been addressed to date. OBJECTIVE: We compared the clinical features of the 3 diseases and investigated changes in the collagen fibers involved. METHODS: Twenty-four cases of PE, 12 cases of EC, and 2 cases of NA found in PubMed and the Korean database were reviewed. Changes in dermal collagen fibers in 10 cases with histological figures were investigated. RESULTS: There were significant similarities between the 3 entities in terms of their clinical features. Four patients with PE and 2 with EC with fine, dense collagen fibers were women who had multiple white to hypopigmented, slightly indurated to firm, millimeter-size papules on the trunk and/or extremities that progressed gradually after developing in the patients' first to third decades. CONCLUSION: The 3 conditions are the same clinical entity in our opinion; such cases with fine, dense collagen manifest typical features.
Subject(s)
Female , Humans , Collagen , Elastic Tissue , Extremities , NevusABSTRACT
Papular elastorrhexis is a rare disorder, possibly a form of multiple elastic tissue nevi. The disorder typically presents as several asymptomatic, firm, nonfollicular, 1- to 5-mm-diameter, and well-demarcated white papules. It predominantly appears on the trunk, including the chest, abdomen, back and shoulders, upper extremities, and, rarely, thighs. Histologically, it is characterized by substantial fragmentation or nearly complete loss of elastic tissue in the reticular dermis. We report a 37-year-old female with popular elastorrhexis, which developed on a rare site, only dorsal aspect of the hand and foot with no involvement of the proximal extremities.
Subject(s)
Adult , Female , Humans , Abdomen , Dermis , Elastic Tissue , Extremities , Foot , Hand , Nevus , Shoulder , Thigh , Thorax , Upper ExtremityABSTRACT
Papular elastorrhexis is a rare cutaneous disorder that occurs predominantly during adolescence. The disorder is characterized by asymptomatic indurated white or flesh papules showing decreased and fragmented elastic fibers in the dermis. We herein report on a 12-year-old girl with multiple asymptomatic white, nonfollicular, firm papules scattered on the extremities and trunk. Histological examination revealed focal areas showing increased layers of collagen fibers and decreased and fragmented elastic fibers in the upper dermis.
Subject(s)
Adolescent , Child , Humans , Collagen , Dermis , Diagnosis, Differential , Elastic Tissue , Extremities , NevusABSTRACT
Papular elastorrhexis is a rare disorder in which children or adolescents present with asymptomatic, non-follicular, 1~5 mm firm white papules on the trunk and extremities. Histologically, there are focal areas of collagen homogenization with decreased and fragmented elastic fibers. Most reported cases are sporadic, but familial occurrence has been described. We report an uncommon and interesting case of papular elastorrhexis in both a brother and sister.
Subject(s)
Adolescent , Child , Humans , Collagen , Elastic Tissue , Extremities , SiblingsABSTRACT
Papular elastorrhexis (PE) is a rare connective tissue disease, histopathologically characterized as decreased and fragmented elastic fibers in the reticular dermis, with or without change to the collagen bundles. It presents as small, white, nonfollicular papules predominantly scattered over the chest, shoulders, or back, and is most often seen in females of the second decade. Although all reported cases of PE have been asymptomatic, a 21-year-old woman presented with a one-year history of multiple, small skin-colored papules on the back which were associated with an intermittent itching sensation. Histopathologic examination revealed fragmented elastic fibers with focal homogenization of collagen in the reticular dermis. Therefore, we report a rare case of papular elastorrhexis with "pruritus".
Subject(s)
Female , Humans , Young Adult , Collagen , Connective Tissue Diseases , Dermis , Elastic Tissue , Pruritus , Sensation , Shoulder , ThoraxABSTRACT
Papular elastorrhexis is a rare disease, characterized by multiple nonfollicular white papules that usually occur on the trunk. Papular elastorrhexis occurs during childhood and adolescence. Histopathologically, the elastic fibers are decreased and they may appear in thin and fragmented forms. Herein, we report a 20-year-old Korean woman with asymptomatic multiple nonfollicular white firm papules that were scattered over the anterior chest.
Subject(s)
Adolescent , Female , Humans , Young Adult , Elastic Tissue , Rare Diseases , ThoraxABSTRACT
Papular elastorrhexis is a rare disorder characterized by multiple, white, non-follicular, firm papules usually occurring on the trunk during adolescence. Histologically, there are focal areas of collagen homogenization with decreased and fragmented elastic fibers. There has been no established treatment for this disease. We report a case of papular elastorrhexis in a 19-year-old female patient improved by intralesional injections of triamcinolone acetonide (TA).