Acquired Reactive Perforating Collagenosis Research Articles

Systematic review and meta-analysis Literature-based Clinical Retrospective Analysis of Acquired Reactive Perforating Collagenosis

Objective: Acquired reactive perforating collagenosis(ARPC) is a rare dermatosis that usually associated with systemic disease. The associations, characteristics and treatment rigemen of ARPC are not well understood.This study was performed to retrospectively evaluate the diagnosis and treatment statuses of acquired reactive perforating collagenosis (ARPC). Methods: A systematic review of the PubMed, Embase, and Scopus databases was independently performed by two reviewers. Data were extracted and qualitatively analyzed. The quality of the articles was assessed by using the Newcastle–Ottawa scale. The data are described in the manuscript. The study was registered with PROSPERO, CRD 42023448576. Results: In total, 189 patients (98 women, 91 men) in 124 studies were analyzed. Their mean age was 57.31 ± 15.21 years. A total of 88.89% (n = 168) of the patients had one or more systemic diseases, of which 57.67% (n = 109) had diabetes mellitus, 29.10% (n = 55) had chronic renal failure, 25.93% (n = 49) had hypertension, and 13.76% had malignancy (n = 26). The most frequently affected area was the lower extremities (72.49%, n = 137), followed by the trunk (70.37%, n = 133) and the upper extremities (63.49%, n = 120). One hundred sixty (84.66%) patients had pruritus, and 32.80% (n = 62) of them were positive for the Koebner phenomenon. Oral antihistamines and topical steroids were the most common treatments in this study, followed by narrow-band ultraviolet B therapy and allopurinol. Management of concomitant systemic diseases also resulted in improvement of ARPC. Conclusion: In this review, ARPC was frequently associated with systemic diseases, especially diabetes mellitus, renal failure, hypertension, and malignancy. The lower extremities and trunk were the most frequently affected areas, and pruritus usually appeared. Antihistamines and topical steroids were the most commonly used treatments, and we recommend narrow-band ultraviolet B therapy as the first-line treatment. Management of systemic diseases and relief of pruritus should be considered simultaneously.

Dupilumab improve acquired reactive perforating collagenosis characterized by type 2 inflammation.

Acquired reactive perforating collagenosis (ARPC) is a clinically challenging disease with an unclear pathogenesis. To evaluate the efficacy and safety of dupilumab for the treatment of ARPC, and analyze the expression of type 2 inflammation-related molecules in ARPC lesions. This retrospective cohort study included 20 patients with ARPC; 10 received dupilumab and 10 received conventional therapy. The efficacy and safety of dupilumab were evaluated at 12 weeks. Immunohistochemical and immunofluorescence analyses of T- and B-cell markers, and type 2 inflammation-related cytokines, were performed on skin samples from ARPC patients, atopic dermatitis (AD) patients, and healthy controls. Significantly more patients showed improvements in the Investigator Global Assessment score (100% vs. 0%; p < 0.0001) and itching (90%/8.33%, P =.001) in the dupilumab group compared to the conventional group at 12 weeks. There were no adverse effects in the dupilumab group. The ARPC lesions showed enhanced dermal infiltration of CD3+ T-cells, with a predominance of Th2 cells, similar to AD lesions. IL-4 and IL-13 were co-localized with GATA3 in ARPC lesions. Dupilumab improved ARPC charaterized with type 2 inflammation.

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.

Dupilumab may be an alternative option in the treatment of acquired reactive perforating collagenosis combined with AD.

The management of acquired reactive perforating collagenosis (ARPC) is challenging. Here, we shared two cases of ARPC combined with elderly atopic dermatitis (AD) that did not respond well to conventional treatment but responded well to the monotherapy of dupilumab, which suggests that dupilumab may be an alternative option for the treatment.

Clinicopathological Differences between Acquired Reactive Perforating Collagenosis and Prurigo Nodularis

Background: Acquired reactive perforating collagenosis (ARPC) is a skin disease characterized by multiple itchy nodules, which is notably similar to the skin eruptions of prurigo nodularis (PN). The aim of this study was to prove the clinicopathological differences between ARPC and PN. Methods: We examined 22 patients with ARPC (6 males and 16 females) and 38 patients with PN (27 males and 11 females), diagnosed clinically and histologically. Transepidermal elimination of collagen which is characteristic histological findings for ARPC was found in all ARPC patients, but not in PN patients even in a serial section. Clinical findings, laboratory data, histological findings, immunohistochemical results, and efficacy of the therapies were compared between the two groups. Results: On evaluation of laboratory data, thymus and activation-regulated chemokine (TARC) levels and peripheral eosinophil counts were lower in ARPC than in PN. Histologically, we observed greater infiltration of neutrophils, histiocytes, and lymphocytes and more extensive capillaries in ARPC than in PN. Upon immunohistochemical analysis, CD163+ macrophages were found to be more abundant in the upper-to-deep dermis of ARPC, and both CD4+ T cells and CD8+ T cells were significantly increased in the mid-to-deep dermis of ARPC. The efficacies of topical steroid and antihistamines were improved by the addition of emollients in ARPC. Conclusion: This is the first report to show the histological differences in cell infiltration between the lesions of ARPC and PN. It is likely that inflammation mediated by neutrophils and M2 macrophages may be involved in the formation of ARPC lesions, as against PN lesions mediated by epidermal changes such as spongiosis. It is suggested that the addition of emollients can increase the efficacy of treatments for ARPC. However, further studies are necessary to define the precise pathomechanisms of ARPC and the effects of emollients on ARPC.

Successful treatment of acquired reactive perforating collagenosis induced by pregnancy with allopurinol: A case report with review of literature

Acquired reactive perforating collagenosis (ARPC) is a rare condition caused by transepidermal elimination of collagen, elastin fibers and keratin. To date, the pathogenesis of ARPC remains unknown. Different hypotheses were proposed, including superficial microtrauma due to pruritus and subsequent scratching, diabetes-induced microangiopathy, epidermal and dermal abnormalities in metabolic disorders, dermal microdeposits in patients with chronic renal failure and vasculopathy underlying chronic venous insufficiency and hypertension. In the past two decades, oral allopurinol had been found to be effective in treating ARPC. We report a case of a 36-year-old pregnant woman with itchy skin lesions on the trunk and four limbs since 36 weeks of her gestation. Initially, she was prescribed with oral antihistamine and topical steroid but did not respond well. Skin biopsy was performed and was compatible with ARPC. Normal renal and liver function tests were noted, and the HLA-B5801 test was negative. We treated the patient with allopurinol. Significant improvement was noticed at 1-month follow-up. To the best of our knowledge, this is the first case successfully treating ARPC induced by pregnancy with allopurinol. On careful follow-up of laboratory data and HLA-B5801 test screening, allopurinol may be another effective treatment option for ARPC patients after pregnancy.

Expression of the receptor for advanced glycation end products in acquired reactive perforating collagenosis

Acquired reactive perforating collagenosis (ARPC) is a rare skin disorder characterized by transepidermal elimination of dermal collagen. There is little data regarding the pathogenesis of ARPC. The receptor for advanced glycation end products (RAGE) is a multiligand transmembrane receptor that plays an important role in inflammatory responses and may be involved in the pathogenesis of ARPC. To explore the expression of RAGE in ARPC. Paraffin-embedded punch biopsy specimens of 41 patients with ARPC and of 11 healthy controls with normal skin were obtained from the Department Of Pathology. Clinical data of all patients were reviewed from the medical files. All specimens were stained immunohistochemically with antibody to RAGE (Anti-RAGE). The intensity of RAGE expression was assessed semi-quantitatively on epidermal cells, microvascular endothelium, dermal fibroblasts and inflammatory cells and graded as 0 (no staining), 1 (weak), 2 (moderate) and 3 (strong). The patients were divided into diabetic and nondiabetic groups for analysis. RAGE expression in microvascular endothelium, inflammatory cells and fibroblasts of patients with ARPC was more intense than normal tissues of healthy participants (P values are 0.005, 0.017 and P > 0.05). Our method of assessment of RAGE expression was semi-quantitative. We observed an overexpression of RAGE in lesional samples of patients with ARPC which was independent of the presence of diabetes.

Acquired reactive perforating collagenosis: a clinicopathologic study of 15 cases from Saudi Arabia

Perforating dermatoses include a family of disease characterized by transepidermal elimination of collagen. Despite being described many years ago in association with systemic disease, it is still clinically misdiagnosed. A retrospective clinical and pathologic review that will increase clinical and pathologic diagnostic awareness of this entity and explore associated systemic disease. In this report, we present from Saudi Arabia for the first time details of a series of patients seen at the King Abdulaziz Medical City, WR, Jeddah, Saudi Arabia, during the period 2002-2009. The study revealed 15 histologically confirmed cases of acquired reactive perforating collagenosis (ARPC). Patients were predominantly adult females with a mean age of 54 years. The lesions were pruritic, predominantly multiple, erythematous excoriated papules and umblicated nodules centrally crusted with an average duration of 6 months, involving the extremities with or without the trunk in 12 patients. Associated systemic disease included diabetes mellitus in 12 patients and chronic renal failure on dialysis in 8 patients. The provisional clinical differential diagnosis included perforating collagenosis in only eight patients. ARPC is not uncommon in Saudi Arabia. The noduloulcerative lesions involve predominantly the extremities, and diabetes mellitus is the most common associate. It is hoped that this article will increase awareness for recognition of this entity to start appropriate therapy.

Acquired reactive perforating collagenosis associated with papillary thyroid carcinoma: a paraneoplastic phenomenon?

Acquired reactive perforating collagenosis (ARPC) is an uncommon dermatosis characterized by transepidermal elimination of altered collagen. It is commonly seen in patients with diabetes mellitus and/or chronic renal insufficiency. Rarely, it has been reported in association with malignancy and other conditions. We report a 30-year-old woman with insulin-dependent diabetes mellitus who presented with multiple, discrete, violaceous, hyperkeratotic papules on the extensor aspects of both legs, characteristic of ARPC. Four months later, metastatic papillary thyroid cancer was diagnosed. This case further supports the possibility that ARPC may represent a paraneoplastic phenomenon.

Up-regulation of transforming growth factor-β3 and extracellular matrix proteins in acquired reactive perforating collagenosis

Acquired reactive perforating collagenosis (ARPC) is an uncommon itchy dermatosis of unknown etiology. We aimed to study clinical features of ARPC and to characterize the expression profiles of proteins which are involved in extracellular matrix remodeling and wound repair. Seventeen patients with ARPC were included in the study. Immunohistochemical analyses were performed for CD34, factor VIIIa, vascular endothelial growth factor, matrix metalloproteinase-1 (MMP-1), tissue inhibitor of metalloproteinase-1 (TIMP-1), transforming growth factor-beta3 (TGF-beta3), Smad-3, and Smad-7. Twelve patients (70.6%) had diabetes mellitus with disease duration of 14.6 +/- 13.1 years (mean +/- standard deviation). In all patients, chronic kidney disease was evident; two patients were receiving hemodialysis. Preexisting scabies infection was observed in 7 patients (41.2%). CD34 staining was significantly stronger in vessels of perilesional than those of lesional skin (P = .024). TGF-beta3, MMP-1, and TIMP-1 immunoreactivity was significantly stronger in lesional skin as compared with perilesional skin (P = .016, P = .0065, and P = .035, respectively). Although Smad-3 and Smad-7 immunoreactivity did not significantly differ in lesional and perilesional skin, there was a significant correlation between the protein expression of TGF-beta3 and Smad-3 (r = 0.56; P = .02), Smad-7 (r = 0.64; P = .006), and TIMP-1 (r = 0.56; P = .018) expression. We did not perform polymerase chain reaction studies on mRNA expression. Our clinical data indicate that ARPC is etiopathogenetically linked to chronic kidney disease. Overexpression of TGF-beta3 and extracellular matrix proteins may represent antecedent tissue repair and therefore may be considered a significant event in the resolution of ARPC lesions.

Acquired Reactive Perforating Collagenosis Successfully Treated with Narrow-band Ultraviolet B

© 2009 The Authors. doi: 10.2340/00015555-0685 Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Acquired reactive perforating collagenosis (ARPC) is associated with diabetes mellitus and/or chronic renal failure. The disease lesions consist of many dome-shaped nodules, which have a central umbilication containing firm keratotic plugs. Histologically, transepidermal elimination of degenerated collagen fibres and marked neutrophil infiltrations are characteristic features. ARPC is usually refractory to treatment; however, there have been reports of ARPC treated with phototherapy. In the present case, we treated the patient with narrow-band ultraviolet B (NBUVB) phototherapy. After 20 exposures (total 18.96 J/cm2), all of the nodules had completely disappeared.

Characteristics of acquired reactive perforating collagenosis

The status of the patient's associated disease can generally affect the onset or healing of acquired reactive perforating collagenosis (ARPC). We treated eight cases of ARPC and noted that the patients had similar findings. However, it was not clear why ARPC developed in the patients with these diseases. Nevertheless, several factors related to the diseases associated with ARPC could affect the degeneration of collagen fibers or the production of dermal products. Some patients had diseases that were characterized by fibrosis and an increased amount of reticular fibers. Factors related to tissue remodeling might act not only in diseases associated with ARPC but also in ARPC itself.

Acquired Reactive Perforating Collagenosis in a Patient with Lung Fibrosis

Reactive perforating collagenosis (RPC) is a rare disorder characterized by the transepidermal elimination of altered collagen. The inherited form of RPC begins in early childhood, but acquired reactive perforating collagenosis (ARPC) begins in adult life. ARPC is associated with diabetes mellitus, renal disease, and malignancy. ARPC with lung fibrosis has not previously been reported in the literature, and the relationship between ARPC and lung fibrosis has not been studied. The etiological relationship between the two disorders appears to be uncertain. Although their association in this case could be due to chance, it may be due to the transforming growth factor beta abnormalities seen in both diseases. In this report, we describe a case of ARPC with lung fibrosis and propose an etiological association between the two diseases.