Acroqueratoelastoidosis de Costa / Acrokeratoelastoidosis of Costa

La acroqueratoelastoidosis de Costa es una genodermatosisde herencia autosónimica dominante con penetrancia incompleta. Es un trastorno de las fibras elásticas exclusivamente cutáneo y a nivel acral. Se caracteriza por la presencia de pápulas eritematosas, amarillentas o del color de la piel normal en la cara marginal de los dedos de las manos o de los pies, que se agrupan, forman placas con aspecto de empedrado y se extienden de forma simétrica al dorso, las palmas y las plantas. El hallazgo histológico patognomónico es la elastorrexis. Se presenta el caso de un niño de 7 años con una acroqueratoelastoidosis que comprometía las manos y los pies, asociada a prurito.

Acrokeratoelastoidosis of Costa is a genodermatosis of autosomal dominant inheritance with incomplete penetrance.It is a cutaneous disorder of the elastic fibers at the acral level.The clinical feature is the presence of erythematous, yellowish or normal skin-colored papules on the marginal aspect of the fingers and/or toes, which agminate to form cobblestone-like plaques and extend symmetrically to the dorsum and palms and soles. The pathognomonic histologic finding is elastorrhexis. We present a 7-year-old boy with acrokeratoelastoidosis involving hands and feet, associated with pruritus.

Acroqueratoelastoidosis: reporte de un caso clínico / Acrokeratoelastoidosis: reportof a clinical case

Resumen La acroqueratoelastoidosis es una rara genodermatosis de herencia autosómica dominante, familiar o esporádica, siendo esta última la forma más frecuentemente reportada en la literatura. Fue descripta por el dermatólogo de origen brasileño Oswaldo Costa en el año 1953. Así las acroqueratodermias marginales son un subgrupo de queratodermiaspalmoplantares caracterizadas por la presencia de pápulas y placas queratósicas, con una disposición lineal, que asientan sobre el margen de transición entre la piel dorsal y palmar o plantar.Suelen iniciarse en la infancia, en la adolescencia o en la vida adulta temprana y tienen un curso crónico. Su diagnóstico diferencial con el resto de las acroqueratodermias es un gran desafío, siendo el hallazgo histológico de elastorrexis lo primordial para su correcto diagnóstico. Presentamos el caso de una mujer de 22 años con un cuadro compatible clínica e histopatológicamente con Acroqueratoelastoidosis.

Abstract Acrokeratolastoidosis is a rare genodermatosis of dominant autosomal, familial or sporadic inheritance, the latter being the most frequently reported form in the literature. It was described by the Brazilian dermatologist Oswaldo Costa in 1953. It is characterized by the presence of multiple hyperkeratotic papules, usually asymptomatic, located in the marginal area of the hands, feet or both. It usually begins in childhood, adolescence or early adult life and has a chronic course. Its differential diagnosis with the rest of the acrokeratosis is a great challenge, being the histological finding of elastorrexis the primary for its correct diagnosis. We present the case of a 22-year-old woman with a clinical and histopathology compatible with Acroqueratoelastoidosis.

Clinicopathological analysis of 22 cases of papular elastorrhexis / 中华皮肤科杂志

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.

Clinicopathological analysis of 22 cases of papular elastorrhexis / 中华皮肤科杂志

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.

A Case of Papular Elastorrhexis: Differential Diagnosis with Eruptive Collagenoma / 대한피부과학회지

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.

The Importance of Collagen Tissue in Papular Elastorrhexis, Eruptive Collagenoma, and Nevus Anelasticus

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.

The Importance of Collagen Tissue in Papular Elastorrhexis, Eruptive Collagenoma, and Nevus Anelasticus

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.

Papular Elastorrhexis in Down's Syndrome / 대한피부과학회지

No abstract available.

Papular Elastorrhexis Confined to Dorsal Aspect of the Hand and Foot / 대한피부과학회지

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.

Papular Elastorrhexis: A Case and Differential Diagnosis

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.

Papular Elastorrhexis

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".

Papular Elastorrhexis in Brother and Sister / 대한피부과학회지

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.

A Case of Papular Elastorrhexis

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.

A Case of Papular Elastorrhexis Improved by Intralesional Injections of Triamcinolone Acetonide / 대한피부과학회지

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).

A Case of Acrokeratoelastoidosis / 대한피부과학회지

Acrokeratoelastoidosis (AKE) is a rare skin disorder initially described by Costa, which is inherited by autosomal dominant, but also may be sporadic. Clinically, it consists of small, firm papules with occasional keratosis or umbilication, characteristically along the margins of hands and feet. Histopathologically, it shows hyperkeratosis, acanthosis in the epidermis and fragmentation and rarefaction of elastic fibers-elastorrhexis-in the dermis. A 32-year-old man presented with multiple papules along the border of the hands and feet, and from histopathology, AKE was diagnosed. Herein we report a case of acrokeratoelastoidosis and review the clinical and histopathologic features, etiology, differential diagnosis and treatment.