Clinical and histopathologic features of acquired perforating collagenosis: A case report

@#A 51-year-old hypertensive and diabetic male patient, admitted due to acute kidney injury on top of chronic kidney disease, was referred to the dermatology service. He presented with a 3-month history of multiple discrete pruritic papules with keratotic plugs over the extensor aspects of the bilateral upper and lower extremities, initially managed as a case of scabies infestation but medications given provided no relief. Histopathologic analysis of a skin biopsy revealed findings of acquired perforating collagenosis. Resolution of lesions and pruritus were noted after starting on a topical corticosteroid ointment on the lesions, as well as hemodialysis to address the renal failure. Subsequent consults showed no recurrences and only a minor sequela of post-inflammatory hyperpigmentation.

Acquired reactive perforating collagenosis, a rare entity at uncommon site

Acquired reactive perforating collagenosis (ARPC), rare disorder characterized by transepidermal elimination (TEE) of collagen fibers, is seen in adult diabetics. Genetic predisposition, familial aggregation, trauma, bites and scratching are implicated. Diabetics develop microvascular diseases leading to intense pruritus causing repeated micro trauma leading to necrosis of connective tissue of dermis, causing TEE. Isolated papules, plaques and nodules with central keratotic plugs, are mostly seen on extensor surfaces of limbs but trunk and face may be involved. Histopathology shows extrusion of abnormal collagen fibers through epidermis. Multiple treatment modalities show variable response. A 52 year old diabetic female had multiple, itchy, well defined, erythematous papules and plaques with central adherent crusting on lower back since 1 month. Histopathology showed cup shaped epidermal depression filled with plug of altered collagen, acanthotic epidermis with hyperkeratosis and parakeratosis. Underlying epidermis was thin with fine slits through which vertically oriented basophilic collagen fibers were extruded.

Progress in cerebral venous collagenosis / 中华神经科杂志

Moody and colleagues discovered the presence of venous collagenosis (VC) in periventricular of human brains by pathological study obtained at autopsy in 1995, which was described as a noninflammatory collagenous thickening of venous walls resulting in severe periventricular venous stenosis or occlusion. Due to the lack of specific markers and staining methods, there are few studies of cerebral venous disease, and the pathological features and pathogenesis are still unclear. However, studies have reported that VC is associated with cerebral small vessel disease (CSVD) and Alzheimer′s disease (AD), suggesting that VC may play an important role in exploring and elucidating pathogenesis. The article aims to provide a review of researches on VC pathological features, possible pathogenesis and correlation with CSVD and AD.

Research progress of acquired reactive perforating collagenosis / 中国医师杂志

Reactive perforating collagenosis is a rare skin disease characterized by the expulsion of denatured collagen fibers through the epidermis. The specific pathogenesis of the disease is not clear, and according to the etiology it can be divided into hereditary and acquired. Hereditary is relatively rare, usually seen in infants, and acquired is usually seen in adults, often associated with other systemic diseases, such as diabetes, chronic renal failure, cirrhosis, pulmonary fibrosis, tuberculosis, pulmonary aspergillosis, thyroid disease, scabies, hepatitis, acquired immunodeficiency syndrome (AIDS), malignant tumors and so on. There is no standard treatment for the disease.

Giant variant of acquired perforating dermatosis, clinically masquerading as a sarcoma: A report of a rare case

Acquired perforating dermatosis is a group of disease characterized by transepidermal elimination of altered dermal constituents of unknown pathogenesis. The giant variant was first described in 2006, as an emerging entity with seven reported cases to date. Here is an 83-year-old male presented with a 4-year history of gradually enlarging soft tisssue mass with ulcerartions at the left knee joint. Imaging revealed an extra-articular, single, heterogeneous, multinodular mass, suspicious for a soft tissue sarcoma. Wide local excision of the mass showed fleshy, hemorrhagic nodules communicating with epidermal ulcers. Microscopy showed cystic spaces straddling dermis and subcutis, containing eosinophilic, amorphous, granular material extruding through epidermal craters, surrounded by exuberant myofibroblastic proliferation. Trichrome and van-Gieson stains confirmed that the extruded material is collagen and the histology was compatible with the giant variant of acquired perforating collagenosis. Awareness of histological appearance prevents misdiagnosis and overtreatment of this entity, masquerading as a sarcoma clinically.

Neumonia crónica organizada en un adolescente: presentación de un caso / Chronic organized pneumonia in an adolescent: presentation of a case

We present a case of organized chronic pneumonia (OCP), a rare interstitial disease, which usually affects people over 50 years of age and is very unusual in pediatrics, so we thought it was of interest to communicate it. Is a 14-year-old male in whom in the study of a febrile condition, a pneumonia of LII was detected. The images persist after 2 months of being treated and being asymptomatic. Infectious causes and rheumatic diseases were ruled out due to the history of discoid lupus. Chest CT, bronchoalveolar lavage and lung biopsy showing Masson's bodies were performed and OCP was diagnosed. He received prednisone 1 mg / kg day and Clarithromycin for 2 months with rapid improvement. Relapses with new radiological images typical of OCP twice, when treating on alternate days. It is indicated prednisone 30 mgr / day for 3 months and gradual reduction. At 18 months, being with prednisone 5 mgr day, has a relapse of rapid progression that was treated with pulses of methylprednisolone. The collagen study shows positive lupus anticoagulant and ANA and Systemic Lupus is diagnosed. The bad evolution would be explained because it was a secondary OCP. Mycophenolate associated with prednisone was indicates, which has been used in the OCP and in Lupus. This clinical case shows the importance of radiological follow-up of patients with OCP and the search for secondary OCP causes due to the poor response to corticosteroids.

Se presenta un caso de neumonía crónica organizada (NCO), enfermedad intersticial rara, que afecta habitualmente a mayores de 50 años y muy inusual en pediatría, por lo que creímos de interés comunicarlo. Se trata de un varón de 14 años en el que en el estudio de un cuadro febril se detecta una neumonía de LII cuyas imágenes persisten luego de 2 meses de haber sido tratado y estando asintomático. Se descartaron causas infecciosas y enfermedades reumatológicas por el antecedente de lupus discoide. Se realizó TC de tórax, lavado broncoalveolar y biopsia pulmonar que mostró cuerpos de Masson, con lo que se diagnosticó NCO. Recibió prednisona 1 mgr/ kg día y Claritromicina por 2 meses con una rápida mejoría. Recae con nuevas imágenes radiológicas típicas de NCO dos veces, al pasar de tratamiento diario a días alternos. Se indica 30 mgr/día por 3 meses de prednisona y reducción gradual posterior. A los 18 meses de tratamiento, estando con prednisona 5 mgr día, tiene una recaída de rápida progresión que se trató con pulsos de metilprednisolona. El estudio de colagenosis muestra anticoagulante lúpico y ANA positivos y se diagnóstica Lupus Sistémico. La mala evolución se explicaría porque se trató de una NCO secundaria, ante lo cual indicó micofenolato asociado a prednisona, que se ha usado en la NCO y en el Lupus. Este caso clínico muestra la importancia del seguimiento radiológico de los pacientes con NCO y de la búsqueda de causas secundarias de NCO ante la mala respuesta a corticoides

Clinical and histopathologic features of acquired perforating collagenosis: A case report

@#A 51-year-old hypertensive and diabetic male patient, admitted due to acute kidney injury on top of chronic kidney disease, was referred to the dermatology service. He presented with a 3-month history of multiple discrete pruritic papules with keratotic plugs over the extensor aspects of the bilateral upper and lower extremities, initially managed as a case of scabies infestation but medications given provided no relief. Histopathologic analysis of a skin biopsy revealed findings of acquired perforating collagenosis. Resolution of lesions and pruritus were noted after starting on a topical corticosteroid ointment on the lesions, as well as hemodialysis to address the renal failure. Subsequent consults showed no recurrences and only a minor sequela of post-inflammatory hyperpigmentation.

Acquired Reactive Perforating Collagenosis as a Preceding Manifestation of Hodgkin's Lymphoma / 대한피부과학회지

A 40-year-old man presented with pruritic, multiple, variable-sized, erythematous umbilicated papules on the trunk and both extremities for 4 months. He was diagnosed with Hodgkin's lymphoma (stage IIA) after histopathologic examination of a neck mass that developed a month ago. A punch biopsy was performed on his right lower leg. Histological examination showed transepidermal elimination of the degenerated collagen. Masson's trichrome staining was performed to distinguish collagen fibers from the muscular tissue; using Masson's stain, the collagen appeared as a bluish color crossing from the dermis to the epidermis. The diagnosis of acquired reactive perforating collagenosis was made. The skin lesions showed much improvement after 6 cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine chemotherapy. Acquired perforating disorders are a group of cutaneous disorders that occur in adults with chronic kidney disease or diabetes mellitus. Cases of acquired perforating disorders associated with Hodgkin's lymphoma have been rarely reported in the English literature. To our knowledge, this is the first case of acquired reactive perforating collagenosis in a Korean patient with Hodgkin's lymphoma.

Tuberculosis o colagenosis: un dilema diagnóstico / Tuberculosis or collagenosis: a diagnostic dylemma

Introducción: Colagenosis y tuberculosis comparten síntomas y signos, pero además, el trastorno autoinmune y los tratamientos inmunosupresores que reciben los pacientes con colagenosis, los hacen más vulnerables a esta infección, lo que puede constituir un dilema diagnóstico. Objetivo: Contribuir al conocimiento de la relación entre tuberculosis y colagenosis. Presentación de casos: Se presentan tres adolescentes con tuberculosis, atendidos en el Centro de Referencia Nacional para la Tuberculosis Infantil. Dos enfermos tenían diagnóstico previo de colagenosis (artritis idiopática juvenil y polimiositis) con tratamiento esteroideo en exacerbaciones o continuo desde hacía un año, respectivamente. El tercero presentó un síndrome febril prolongado con pleuresía y pericarditis, con sospecha de lupus eritematoso diseminado. Se diagnosticó tuberculosis por test de mantoux hiperérgico. El tratamiento fue prolongado con esteroides, drogas antituberculosas y pericardiotomía al inicio del proceso, con evolución tórpida y fallo de tratamiento. Todo el tiempo se trató de descartar una enfermedad del colágeno. Se confirmó por cultivo la tuberculosis en los tres pacientes y la evolución final fue satisfactoria. Se exponen las características de cada enfermo y se analiza la relación entre ambas entidades. Conclusiones: Se presentan tres casos que ejemplifican la relación entre tuberculosis y colagenosis(AU)

Introduction: Collagenosis and tuberculosis share similar symptoms and manifestations; and in addition, the autoimmune disorder and inmunosuppressive treatments that patients with collagenosis receive make them more vulnerable to this infection which can constitute a diagnostic dylemma. Objective: To contribute to a better knowledge on the relation among tuberculosis and collagenosis. Cases presentation: Three adolescents suffering collagenosis are presented. They were attended in the National Reference Center for Children Tuberculosis. Two of the patients had previous diagnostic of collagenosis (juvenile idiopatic arthritis and polymyositis) with steroids treatment in exacerbations or continuous since a year ago. The third patient presented a prolonged febrile syndrome with pleurisy and pericarditis, with suspicions of disseminated lupus erythematosus. Tuberculosis was diagnosed by the test of hyperergic Mantoux. The treatment was prolonged with steroids, antiturberculosis drugs and pericardiotomy at the beginning of the process, with bad evolution and failure of the treatment. All the time it was intended to rule out collagen disease. Tuberculosis was confirmed by culturing in the three patients and final evolution was satisfactorily. Characteristics of each patient were exposed and it was analyzed the relation among both diseases. Conclusions: Three cases that exemplify the relation among tuberculosis and collagenosis(AU)

Colagenosis perforante reactiva: A propósito de dos casos / Reactive perforating colagenosis: Report of two cases

Los desórdenes perforantes actualmente incluyen a la enfermedad de Kyrle, la elastosis perforante adquirida serpiginosa, la colagenosis perforante reactiva adquirida y la foliculitis perforante. Antes se consideraban entidades diferentes, pero debido a los hallazgos similares en la clínica e histopatología, se engloban dentro de este término unificador. Se manifiestan como pápulas y/o nódulos que presentan un tapón queratósico central. Su causa es desconocida, sin embargo, se las ha relacionado con distintas patologías sistémicas como la diabetes mellitus y la enfermedad renal crónica, entre otras. Se presentan dos casos de dermatosis perforante adquirida tipo colagenosis perforante reactiva, en un hombre de 69 años con antecedentes de diabetes mellitus tipo II y cirrosis hepática secundaria a infección por virus de hepatitis C y una paciente de 62 años con antecedente de diabetes mellitus tipo II.

Perforating disorders currently include Kyrle's disease, serpiginous perforating elastosis acquired, the acquired reactive perforating colagenosis and perforating folliculitis. Previously they were considered different entities, but due to similar findings in clinical and histopathology are now included within this umbrella term. The clinical findings are papules and / or nodules with a central keratotic plug and pruritus is the predominant symptom. It causes are still unknown; however they have been associated with various systemic diseases such as diabetes mellitus and chronic renal disease, among others. We report two cases of reactive perforating collagenosis one in a 69-year-old man with a history of type II diabetes mellitus and liver cirrhosis secondary to infection with hepatitis C and the other one in a 62-year-old woman with type II diabetes mellitus.

Acquired Reactive Perforating Collagenosis Treated by Doxycycline: A Case Report / 대한피부과학회지

No abstract available.

Acquired Reactive Perforating Collagenosis after Coronary Artery Bypass Graft Surgery / 대한피부과학회지

Perforating disorders are a group of skin disorders that present the characteristic clinical finding of transepidermal elimination. Acquired reactive perforating collagenosis is an uncommon condition characterized by umbilicated hyperkeratotic papules, and in almost all cases, it coexists with other disease conditions, such as diabetes mellitus, renal failure, and malignancies. The pathologic mechanism of this disease remains largely unknown. Here, we describe the case of a 42-year-old man who developed acquired reactive perforating collagenosis after coronary artery bypass graft surgery.

Colagenosis perforante reactiva adquirida: a propósito de un caso en un adolescente / Acquired reactive perforating collagenosis: about a case in a teenager

La colagenosis perforante reactiva es un trastorno de la piel poco frecuente, caracterizado por la eliminación transepidérmica de colágeno alterado a través de la epidermis. Existen dos tipos de la enfermedad: la forma hereditaria, que se manifiesta en la infancia, y la esporádica adquirida, que aparece en la edad adulta. Se presenta con pápulas pruriginosas que aumentan de tamaño. Estas pueden remitir espontáneamente, dejando áreas de hipo-hiperpigmentación o cicatrices. También es posible que ocurra el fenómeno de Koebner.Se comunica el caso de un paciente pediátrico con una colagenosis perforante reactiva. Esta publicación reviste importancia debido a que esta patología es extremadamente infrecuente.

Reactive perforating collagenosis is a rare skin disorder, characterized by the transepidermal elimination of altered collagen through the epidermis. There are two different types: the hereditary form that manifests in childhood, and the sporadic acquired one that appears in adulthood. It presents with pruritic papules that increase in size. They may remit spontaneously, leaving areas of hypo/hyperpigmentation or scars. It is also possible that the Koebner phenomenon occurs. We report one case of a pediatric patient with reactive perforating collagenosis. This publication has importance because this condition is extremely rare.

Reactive perforating collagenosis.

Reactive Perforating Collagenosis (RPC) is a rare disease of elimination of altered collagen through the epidermis. The disease exists in childhood form with autosomal recessive mode of inheritance and an adult form acquired in association with diseases such as Diabetes Mellitus (DM), Chronic Renal Failure (CRF), hypothyroidism, lymphoma, hyperparathyroidism, neurodermatitis, AIDS, pulmonary fibrosis, scabies and herpes zoster infection.

A Clinicopathologic Study of Thirty Cases of Acquired Perforating Dermatosis in Korea

BACKGROUND: Acquired perforating dermatosis (APD) is histopathologically characterized by transepidermal elimination of materials from the upper dermis. APD can be divided into four diseases: Kyrle's disease, perforating folliculitis, elastosis perforans serpiginosa, and reactive perforating collagenosis. APD is usually associated with systemic diseases, especially diabetes mellitus or chronic renal failure. So far, there have only been a few Korean studies of APD, which have a limited number of patients. OBJECTIVE: The aim of this study is to evaluate the clinical and histopathologic characteristics of 30 cases of APD and to examine the association with systemic diseases. METHODS: We retrospectively reviewed the medical records and biopsy specimens of 30 patients who were diagnosed with APD. RESULTS: The mean age was 55.5 years, and the average duration of the lesion was 7.8 months. The lower extremities (73.3%) were the most frequently occurring sites of the lesion. Twenty-five patients (83.3%) had pruritus, and Koebner's phenomenon was present in 11 patients. Patients of 63.3% had at least one systemic disease. Diabetes mellitus (n=17, 56.7%) and chronic renal failure (n=10, 33.3%) were the most commonly associated conditions. Most patients received topical steroids (93.3%) and antihistamines (80.0%). The most common histopathologic type was reactive perforating collagenosis (n=23, 73.3%). CONCLUSION: In this study, most patients had a systemic association to the diseases. Therefore, we suggest that further evaluation is necessary for patients who present with APD. This includes reviewing patient's comprehensive past medical history, clinical exam, and additional diagnostic testing to check for the possibility of associated systemic diseases.

Perforating disorders of the skin.

Background: Perforating disorders of the skin, is an often overlooked entity characterized by transepidermal elimination of material from the upper dermis and are classifi ed histopathologically according to the type of epidermal disruption and the nature of the eliminated material. They include Kyrle’s disease, perforating folliculitis, reactive perforating collagenosis, and elastosis perforans serpiginosa. Aim: The aim of this study was to delineate the clinical and histopathological features of perforating disorders of the skin. Materials and Methods: In our study, we reviewed last 2 years skin biopsies received by us. Hematoxylin and eosin sections were re-examined and histochemical stainings (elastic van Gieson and Masson trichrome stains) were also used for histopathological evaluation. Results: We reviewed fi ve cases of perforating disorders of skin which included two cases of Kyrle’s disease, two cases of reactive perforating collagenosis and a single case of perforating folliculitis. Two patients had family history of perforating dermatosis in their siblings and three had associated systemic disease. Conclusion: Perforating disorders of the skin should be considered when ulcer with keratotic plugs is found.

A Giant Variant of Acquired Perforating Collagenosis in Chronic Renal Failure / 대한피부과학회지

Reactive perforating collagenosis (RPC) is one of a range of essential perforating disorders. Two types have been recognized: the childhood or inherited form, and the adult or acquired form. Acquired RPC is usually associated with systemic disease, especially diabetes mellitus and/or renal failure. Lesions of acquired perforating collagenosis are usually smaller than 1 cm, but lesions of the giant variant of acquired perforating collagenosis are larger than 2 cm. We present a case of the giant variant of acquired perforating collagenosis, which has not previously been documented in Korea.

A Case of Acquired Reactive Perforating Collagenosis Triggered by an Insect Bite / 대한피부과학회지

Reactive perforating collagenosis (RPC) is one of a range of essential perforating disorders. Two types have been recognized: the childhood or inherited form, and the adult or acquired form. Acquired RPC is usually associated with systemic diseases, especially diabetes mellitus, and/or renal failure. We present a case of a 47-year-old female with umbilicated skin lesions triggered by an insect bite. Histology from the lesion revealed transepidermal elimination of collagen.

Acquired Reactive Perforating Collagenosis in a Chronic HCV Hepatitis Patient Who was Treated with Interferon Alpha / 대한피부과학회지

Reactive Perforating Collagenosis (RPC) is a kind of perforating dermatosis, which shows transepidermal elimination of altered collagens. Acquired form is developed in an adult without family history, is favorably developed in scratched region due to pruritic sense. Acquired RPC has been reported to be associated with various systemic disorders, such as diabetes mellitus, liver disease, chronic renal failure, lymphoma, AIDS, hypothyroidism, hyperparathyroidism, and neurodermatitis, accompanied by itching & scratching. We report a case of acquired RPC in a patient with HCV hepatitis who was treated with interferon alpha. Skin lesions showed multiple erythematous centrally umbilicated, crusted papules of variable sizes on the extensor surfaces of both shins.

Treatment of Recalcitrant Acquired Reactive Perforating Collagenosis with Low-dose Allopurinol / 대한피부과학회지

Reactive perforating collagenosis is one of a range of essential perforating disorders and is characterized by the transepithelial elimination of altered collagen. Two types have been recognized: the inherited, or childhood form and the acquired, or adult form. Acquired reactive perforating collagenosis is associated with systemic diseases, especially diabetes mellitus and/or renal failure. We report that a low-dose allopurinol showed dramatic effects on acquired reactive perforating collagenosis, which was associated with hypothyroidism and chronic renal failure caused by diabetic nephropathy.