Dermatosis perforante adquirida e Índice de Calidad de Vida en Dermatología en pacientes en hemodiálisis / Acquired perforating Dermatology Life Quality Index in dermatology in patients receiving hemodialysis

Introducción: La dermatosis perforante adquirida (DPA) es un trastorno frecuente en pacientes en hemodiálisis, y el efecto en la calidad de vida está poco descrito. Investigamos la prevalencia de DPA en pacientes en hemodiálisis, medimos y comparamos la calidad de vida asociada a DPA. Métodos: Desarrollamos un estudio prospectivo, observacional y descriptivo. Invitamos a pacientes mayores de 18 años en hemodiálisis. Se obtuvieron datos de su expediente electrónico, y se realizó exploración dermatológica. Se aplicó el Índice de Calidad de Vida en Dermatología (DLQI). Se hizo un análisis descriptivo de las variables demográficas, de las características clínicas y de los hallazgos de dermatoscopia, así como la comparación de los puntajes del DLQI. Resultados: La muestra fue de 46 pacientes, con una prevalencia de DPA del 11%. Los pacientes con DPA eran más delgados y jóvenes en comparación con los pacientes sin DPA. El tiempo en hemodiálisis fue mayor en los pacientes con DPA en comparación a los pacientes sin DPA, con una mediana de 90 versus 32 meses (p=0,015). La afección en calidad de vida fue mayor en los pacientes con DPA en comparación a los pacientes sin DPA, con un algún efecto en todos los pacientes con DPA y un 33% en los pacientes sin DPA (p=0,001). Los pacientes con DPA tuvieron con más frecuencia prurito en comparación con los pacientes sin DPA (p=0,007). Conclusiones: La edad, el tiempo en hemodiálisis y el índice de masa corporal se asocian con la presencia de DPA. Los pacientes con DPA tuvieron una prevalencia más alta de prurito y mayor afección en la calidad de vida en dermatología en comparación con los pacientes sin DPA. (AU)

Introduction: Acquired perforating dermatosis (APD) is a frequent disorder in hemodialysis patients and the effect on the quality of life is poorly described. We investigated the prevalence of APD in hemodialysis patients, measured and compared APD-associated quality of life. Methods: We developed a prospective, observational, and descriptive study. We invited patients over the age of 18 in hemodialysis. Data was obtained from their electronic file and a dermatological examination was performed. The Dermatology Life Quality Index (DLQI) was applied. Descriptive analysis of demographic variables, clinical features, and dermoscopy findings, as well as comparison of DLQI scores, was made. Results: The sample consisted of 46 patients, with a prevalence of APD of 11%. Patients with APD were leaner and younger compared to patients without APD. The time on hemodialysis was longer in patients with APD compared to those without APD, with a median of 90 versus 32 months (P=.015). The impact on quality of life was greater in patients with APD compared to those without APD, with some effect in all patients with APD and 33% in patients without APD (P=.001). Patients with APD had more frequent pruritus compared to those without APD (P=.007). Conclusions: Age, time on hemodialysis and BMI are associated with the presence of APD. Patients with APD had a higher prevalence of pruritus and a greater impact on quality of life in dermatology compared to patients without APD. (AU)

Acquired Perforating Dermatosis and Dermatology Life Quality Index in Patients Receiving hemodialysis.

INTRODUCTION: Acquired perforating dermatosis (APD) is a frequent disorder in hemodialysis patients and the effect on the quality of life is poorly described. We investigated the prevalence of APD in hemodialysis patients, measured and compared APD-associated quality of life. METHODS: We developed a prospective, observational, and descriptive study. We invited patients over the age of 18 in hemodialysis. Data was obtained from their electronic file and a dermatological examination was performed. The Dermatology Life Quality Index (DLQI) was applied. Descriptive analysis of demographic variables, clinical features, and dermoscopy findings, as well as comparison of DLQI scores, was made. RESULTS: The sample consisted of 46 patients, with a prevalence of APD of 11%. Patients with APD were leaner and younger compared to patients without APD. The time on hemodialysis was longer in patients with APD compared to those without APD, with a median of 90 versus 32 months (p = 0.015). The impact on quality of life was greater in patients with APD compared to those without APD, with some effect in all patients with APD and 33% in patients without APD (p = 0.001). Patients with APD had more frequent pruritus compared to those without APD (p = 0.007). CONCLUSIONS: Age, time on hemodialysis and BMI are associated with the presence of APD. Patients with APD had a higher prevalence of pruritus and a greater impact on quality of life in dermatology compared to patients without APD.

Acquired perforating dermatosis in patients on peritoneal dialysis: a report of 3 cases.

Background: Acquired perforating dermatosis (APD) is a heterogeneous group of unfrequented diseases (2.5 cases for 100,000 habitants) associated with multiple pathologies like end-stage renal disease and other concomitant conditions such as diabetes mellitus (DM). Case Description: We described 3 cases of APD in patients on peritoneal dialysis (PD), one of them with a giant variant of reactive perforating collagenosis (RPC). The first case is a 28-year-old man with chronic kidney disease on PD and a lousy control of disturbances of calcium and phosphorus metabolism that develops an APD. The second case is a 44-year-old man with DM, chronic kidney disease (CKD) on PD, and poor control of disturbances of calcium and phosphorus metabolism that develops an RPC. The third case is a 58-year-old man with DM, rheumatoid arthritis, hypothyroidism, CKD and bad control of calcium and phosphorus metabolism that develops a giant variant of RPC with poor evolution. Conclusions: CKD and concomitant conditions such as DM present an increased risk of developing APD. Poor control of calcium and phosphorus metabolism is frequently found in patients with CKD and seems to be related to the development of APD in our cases. With the description of these cases, we want to emphasize the importance of knowing this rare disease, in order to promptly refer to Dermatology and start treatment.

Acquired perforating dermatosis: A clinicopathologic study, and the features of dermoscopy and reflective confocal microscopy of 37 cases.

BACKGROUND: Acquired perforating dermatosis (APD) is a rare skin condition characterized by degenerated materials eliminated from the dermis. Several retrospective studies on APD have been reported; however, few data are available on Chinese APD and their features on dermoscopy and reflective confocal microscope (RCM) assays. OBJECTIVE: The aim of this study was to retrospectively evaluate the clinical and histopathologic data of 37 acquired perforating dermatosis cases, and assess their features on dermoscopy and RCM. METHODS: Thirty-seven APD patients were retrospectively enrolled in our study. We characterized the clinical histopathological features, concomitant diseases, treatment responses, and the dermoscopy and RCM findings. RESULTS: Pruritus was the most common symptom, with the lower extremities as the most predilection sites (86.5%, n = 32; 91.9%, n = 34, respectively). Concomitant diseases were found in 34 patients (92.6%), among which diabetes mellitus was the most common, followed by thyroid nodules, allergic dermatosis, and chronic renal insufficiency. Dermoscopy and RCM assays were performed in 11 patients. The typical RCM images were hyperreflective cord-like structures from the epidermis to dermis. Dermoscopy features of fully developed lesions showed central ulceration with peripheral hairpin-like or loop-like capillaries with characteristic garland arrangements. CONCLUSION: APD is an uncommon skin disorder associated with various systemic conditions in Chinese individuals. Thyroid disorders are an overlooked complication and may play an important role in the development of APD. The results of this study indicate that noninvasive dermoscopy and RCM examination are helpful in the rapid diagnosis and early intervention of APD.

Acquired Perforating Dermatosis as a Paraneoplastic Feature: A Case Report, Literature Review, and Novel Association.

Kyrle's disease is an uncommon form of acquired transepidermal elimination dermatosis frequently associated with diabetes mellitus and chronic kidney disease. An association with malignancy has been sporadically reported in the literature. Here, we describe the clinical course of a diabetic patient with end-stage renal disease who developed this disorder as a herald to a regionally advanced renal cell carcinoma. We provide a focused literature review and rationale for the definitive categorization of acquired perforating dermatosis as a potential paraneoplastic manifestation of systemic malignancies. Clinicopathological correlation and prompt communication among clinicians for occult malignancies are always warranted. Furthermore, we describe a novel association of one of the subtypes of acquired perforating dermatosis with such malignancies.

Generalized Lesions of Kyrle's Disease: A Rare Case.

Kyrle's disease (KD) is a rare type of acquired perforating dermatosis (APD) associated with various systemic diseases, particularly chronic kidney disease and diabetes mellitus (DM). It most commonly occurs at the lower extremities. Generalized lesions of KD are rare. We report a case of generalized KD in a 29-year-old woman with chronic kidney disease and DM. Physical examination revealed multiple hyperkeratotic and hyperpigmented papules, plaques, and nodules with central umbilication and keratotic plugs on almost all parts of the body. Histopathological examination showed keratinized epithelial layer with acanthosis and hyperkeratosis, invagination with the formation of keratin plugs, and basophilic cell debris accompanied by parakeratosis and abnormal keratinization of epithelial cells. These histopathological findings fulfilled the Constantine and Carter criteria for KD. This condition is characterized clinically by umbilicated, round, erythematous or hyperpigmented papules and nodules with central crusts or keratotic plug, predominantly involving the extensor surfaces of the extremities and the trunk. Although uncommon, it may also involve the face or the scalp. Nevertheless, generalized lesions involving faces are rarely found in KD.

Dermoscopy Features of Acquired Perforating Dermatosis Among 39 Patients.

In this report, we concluded there are four dermoscopic features of APD including a yellow-brown homogeneous structureless area in the center of the lesion, dotted and linear vessels distribution radially and a dam shape uplift at the periphery, as well as a white irregular ring surrounding the lesion. There are three features, including the yellow-brown homogeneous structureless area in the center of the lesion, the dotted and linear vessels distribution radially and the white irregular ring surrounding the lesion were correspond to the report of Emma Ormerod et al.These features are also similar to those previously discribed in three separated reports of seven cases with APD. In our report, we found a new dermoscopic features: the dam shape uplift at the periphery. These finding may be contributed to improve the rate of clinical diagnosis of APD.

Acquired perforating dermatosis with associated complicated cellulitis and amputation in a hemodialysis patient.

INTRODUCTION: Cutaneous manifestations related to chronic kidney disease (CKD) are common and associated with high morbidity. Acquired perforating dermatosis (APD) occurs mostly in diabetic or CKD patients, namely those undergoing hemodialysis. CASE REPORT: A 58-year-old male with type 2 diabetes, with long-term insulin use, several microvascular and macrovascular complications, and on maintenance hemodialysis for 5 years presented with a 1-week history of painful, pruritic, umbilicated papules and some punctiform necrotic lesions on his left forearm, both hands, and both amputation stumps. There was no evidence of infection or vascular alterations, and the patient was not responsive to an initial course of topical corticosteroid. These lesions rapidly evolved to larger, coalescent necrotic injuries, with aggravated pain, intense left-hand skin peeling, and the appearance of similar lesions in the trunk, requiring hospital admission. Calciphylaxis and APD were suspected. Skin biopsy was performed and directed treatment initiated, including intradialytic sodium thiosulfate. Histology findings were compatible with APD and also excluded findings suggestive of vasculitis or calciphylaxis. Soon after, difficult-to-treat cellulitis of the left hand and forearm progressed to critical ischemia and amputation. Microbiology analysis revealed Serratia marcescens as the causative agent. DISCUSSION: To our knowledge, there are no previously described cases of APD-related cellulitis. Its treatment can be particularly challenging, as lesions can persist and relapse, and chronic scars can develop. S. marcescens behaves as an opportunistic and difficult-to-treat pathogen, complicating the prognosis. CONCLUSION: APD can be associated with cellulitis and all of its complications in patients with underlying severe vasculopathy. Awareness of this complication in APD with early referral and aggressive treatment might improve the outcomes and quality of life of such patients.

Evaluation of clinicopathological and treatment characteristics of 80 patients with acquired perforating dermatosis.

Acquired perforating dermatosis (APD) is a group of a rare dermatological disorder characterized by elimination of dermal connective tissue through epidermis. We aimed to evaluate the characteristics of patients diagnosed with APD and to determine the differences in comorbidities according to subtypes of APD. A retrospective, observational, cross-sectional study was designed. Patients diagnosed with APD between January 2008 and January 2019 were reviewed. Eighty patients were included in the study. 61.2% (n = 49) of the patients were female and 38.8% (n = 31) were male with a mean age of 58.4 ± 12.5 years. 82.5% (n = 66) of the patients were diagnosed with reactive perforating collagenosis (RPC) and 17.5% (n = 14) of perforating folliculitis (PF). The most common concomitant disease was diabetes mellitus (82.5%). 5.0% of the patients had malignancy. The comorbidity rate in RPC group was higher than PF (P < .05). Topical steroid was the most frequently (90.0%) used treatment. Complete response was obtained 55.0% of patients. Exitus was observed in 23.8% (n = 19) of patients in a mean 17.6 ± 25.7 months follow-up period. APD may be associated with many diseases. Comorbidities are more frequent in RPC group. This situation warns us to evaluate patients with RPC in more detail for underlying diseases. High mortality rate related to the underlying systemic diseases suggests being careful in terms of mortality in patients diagnosed with APD.

Clinical characteristics and prognosis of acquired perforating dermatosis: A case report.

Acquired perforating dermatosis (APD) is an uncommon skin disease characterized by umbilicated hyperkeratotic lesions, and involves the transepidermal elimination of dermal components, including collagen and elastic fibers. The disease can affect patients with systemic disorders, especially those with chronic renal failure or diabetes mellitus. The current paper described four cases of patients with APD and investigated the clinical characteristics and prognosis of APD, as well as its possible link with systemic disorders. In each of the four cases, the patient had systemic disorders before the onset of APD, three had concomitant renal and thyroid disorders and one had hepatocirrhosis secondary to chronic hepatitis C. The results of the present study showed that APD occurred after the transient worsening of the original systemic disease. Furthermore, it was revealed that dermatosis symptoms were alleviated upon remission of the original systemic disorder, without specific dermatological treatment. Dermatosis symptoms improved in all four patients, indicating that the management of the associated systematic diseases was essential for the successful clinical outcomes of APD.

Dermatosis perforante adquirida. Reporte de 2 casos en pacientes con diabetes mellitus y enfermedad renal crónica en hemodiálisis / Acquired perforating dermatosis. Case report of two patients with diabetes mellitus and chronic renal disease undergoing hemodialysis

Las dermatosis perforantes (DP), constituyen un grupo heterogéneo de enfermedades hereditarias o adquiridas caracterizadas por la migración transepidérmica de componentes de la matriz extracelular de la dermis (colágeno, tejido elástico o tejido conectivo necrótico) luego de un proceso inflamatorio y/o degenerativo. La etiopatogenia de la dermatosis perforante adquirida (DPA) es desconocida. Sin embargo presenta una fuerte asociación con traumatismos leves, insuficiencia renal crónica (IRC) y diabetes mellitus, y con menor frecuencia, con endocrinopatías, enfermedades infectocontagiosas y neoplasias, entre otras. En pacientes que realizan hemodiálisis, las DPA pueden observarse en el 10 al 12% de los casos. El prurito es el síntoma cardinal de esta entidad. En algunos pacientes su intensidad puede incluso llegar a ser discapacitante, y las excoriaciones por rascado secundarias al mismo llegan a generar extensas lesiones con alteración de la función de la piel como barrera cutánea. Se presentan dos casos clínicos, ambos con antecedentes de diabetes mellitus e IRC en etapa terminal, con requerimiento de terapia de reemplazo renal (TRR), en hemodiálisis trisemanal. Consultan por una dermatosis pruriginosa generalizada de meses de evolución. Al examen físico presentan lesiones sobreelevadas, hiperpigmentadas, con centro umbilicado rodeadas por un halo eritematoso, localizadas en rostro, tronco y miembros. El examen histopatológico revela una DPA

Perforating dermatoses (PD) represent a heterogeneous group of inherited or acquired diseases characterized by transepidermal migration of dermis extracellular matrix components (collagen, elastic tissue or necrotic connective tissue) following an inflammatory and/or degenerative process. The etiopathogenesis of acquired perforating dermatosis (APD) is unknown. However, it is strongly associated with minor trauma, chronic renal failure and diabetes mellitus and, less frequently, with endocrinological diseases, infectious diseases, neoplastic growth, among others. Acquired perforating dermatoses are diagnosed in 10-12 % of patients undergoing hemodialysis. Itching constitutes the main symptom of this disorder. In some cases, it may be incapacitating and secondary excoriation due to scratching may cause extensive lesions altering the skin barrier function. We report the cases of two patients undergoing hemodialysis three times a week, both with a history of diabetes mellitus and end-stage chronic renal failure requiring renal replacement therapy (RRT). These patients presented with generalized itching dermatosis which had been going on for months. Upon physical examination, raised, hyperpigmented lesions with an umbilicated center and surrounded by an erythematous halo were observed on the face, trunk and limbs. Histopathological examination revealed APD.e

Clinicopathologic and Dermoscopic features of acquired perforating dermatosis: A case report

@#Acquired perforating dermatosis represents one of the perforating skin disorders showing transepidermal elimination of dermal contents. Dermoscopy showing concentric zones of a central keratotic plug, white-gray structureless area and peripheral brown pigmentation may aid in diagnosis and in sample selection for biopsy.

Non-Healing Perianal Ulcers in a Healthy Elderly Male: An Unusual Case of Perforating Dermatosis.

BACKGROUND: Perforating dermatoses are a rare group of dermatologic conditions characterized by transepidermal elimination of dermal material that can be primary or acquired secondary to systemic disease. OBJECTIVE: We present an atypical case of perforating dermatosis resembling elastosis perforans serpiginosa (EPS) presenting with perianal ulcers in an elderly male with no systemic disease or medications and outline his successful treatment course. CONCLUSIONS: Perianal ulcers in an otherwise healthy individual is an unusual presentation for perforating disorders but should be considered in cases not responding to traditional treatment options.