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.

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.

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.

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.

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.

Three Cases of Acquired Reactive Perforating Collagenosis Improved by Narrowband Ultraviolet B Phototherapy / 대한피부과학회지

Acquired reactive perforating collagenosis (ARPC) is difficult to treat. We herein report 3 cases of ARPC, which have been improved by narrowband UVB phototherapy. They had been treated with topical corticosteroid and oral antihistamine, which had no therapeutic effects on their conditions. They were treated with narrowband UVB phototherapy twice a week and four-week treatments resulted in relief of pruritus in three patients and eight-week treatments lead to significant reduction of skin lesions in two patients. Based on our experience, it is suggested that narrowband UVB phototherapy is a good therapeutic modality for recalcitrant skin lesions and severe pruritus of ARPC.

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.

Three Cases of Perforating Disorder / 대한피부과학회지

The perforating disorders comprise a group of unrelated pathologic abnormalities sharing the common characteristic of transepidermal elimination. This phenomenon is characterized by the elimination or extrusion of altered dermal substances and, in some cases, by such material behaving as foreign material. Traditionally, four diseases have been included in this group: Kyrle's disease, perforating folliculitis, reactive perforating collagenosis, elastosis perforans serpiginosum. We describe three cases of perforating disorder which showed different clinical and histopathological figures.

A Case of Atypical Acquired Reactive Perforating Collagenosis / 대한피부과학회지

Reactive perforating collagenosis(RPC) is a kind of perforating dermatosis characterized by transepidermal elimination of altered dermal collagen. RPC is classified into two forms; childhood or inherited form, and adulthood or acquired form. Acquired RPC is reported to occur in association with the severe complicated diabetes mellitus, chronic renal failure and other diseases. We describe a 43-year-old Korean woman with atypical acquired RPC associated with uncontrolled diabetes mellitus and severe pruritus. The histopathologic findings of the lesions showed neither transepidermal channel nor cup-shaped epidermal depression. Multiple degenerated collagen bundles arranged vertically and were eliminated through epidermis to the surface individually. Transmission electron microscopic findings showed the same as typical RPC. Skin lesions improved after the insulin subcutaneous injections, UVB phototherapy and antihistamine administration.

A Case of Reactive Perforating Collagenosis Associated with Tricuspid Regurgitation / 대한피부과학회지

Reactive perforating collagenosis(RPC) is a kind of perforating disorder characterized by the transepidermal elimination of altered dermal collagen. Two types have been recognized; the childhood or inherited form and the adult or acquired form. Acquired RPC is frequently associated with diabetes, chronic renal failure with nephropathy and liver disease. We describe a case of RPC in a 41-year-old woman of about 2 months' duration. She has had a problem of tricuspid regurgitation for 3 years. Skin lesions showed scattered umbilicated papules with central keratotic plugs, 0.3-0.8cm sized, located on the proximal extremities.

Reactive Perforating Collagenosis Arising at the Site of Healed Herpes Zoster / 대한피부과학회지

Isotopic response describes the occurrence of a new skin disorder at the site of another one, already healed and unrelated. The preceding diseases are mostly herpes zosters. Reactive perforating collagenosis is rare dermatosis that has characteristic histopathologic features; transepidermal elimination of degenerated dermal collagen fibers. There has been only one report of an reactive perforating collagenosis occurring on the healed site of herpes zoster. We present a case of acquired reactive perforating collagenosis that was developed on the site previously affected by herpes zoster in a 67-year-old woman, which is thought to be an isotopic response.

A Case of Reactive Perforating Collagenosis Associated With Diabetes Mellitus / 대한피부과학회지

Reactive perforating collagenosis(RPC) is a kind of perforating dermatosis characterized by transepithelial elimination of an altered dermal collagen. RPC is classified into two forms; childhood or inherited form, and adult or acquired form. Acquired RPC is reported to occur in association with the severe complicated diabetes mellitus, chronic renal failure and other diseases. We describe a patient with acquired RPC associated with controlled, non-complicated diabetes mellitus and severe pruritus. Skin lesions showed rapid improvement after the intralesional injection of triamcinolone.

Acquired Reactive Perforating Collagenosis of Diabetes Mellitus and Chronic Renal Failure

We report, herein, a case of reactive perforating collagenosis in a 52-year-old man with diabetes mellitus and chronic renal failure on hemodialysis. He had multiple, pinhead to pea sized, round or oval shaped, central umbilicated papules on the face, trunk, and extremities for 3 months duration. The papules developed after scratching due to pruritus. Histopathologic examination showed a cup-shaped depression of the epidermis filled with parakeratotic material, necrobiotic collagen and inflammatory cells. At the base of the depression, the epidermis showed interruption through which basophilic bundles of collagen ex-tend in a vertical direction from the dermis. The skin lesions improved after treatment with oral antihistaminics:

A Case of Reactive Perforating Collagenosis / 대한피부과학회지

Reactive perforating collagenosis, first described by Mehregan et al in ]967, is a skin disorder characterized by transepidermal eliniination of altered collagen fibers. This rare disease appears as recurrent, umbilicated, crusted papules in response to minor trauma. We report here a case of reactive perforating collagenosis in a 21-year-old man. Examination of the skin revealed a number of centrally umbilicated, asymptomatic papules filled with keratotic plugs in various development and regression. The lesions were distributed on the dorsa of the hands, face, and neck. The biopsy spccimen showed a few of thin bundles of collagen which v ere extruded from the dermis through the atrophic epidermis and the turinels within the epidermis, reaching the buttom of the plug.