Acquired Perforating Dermatosis Research Articles

Acquired Perforating Dermatosis: Clinical and Histopathological Analysis of 95 Patients From One Center.

Acquired perforating dermatosis (APD) is a disease group characterized by transepidermal elimination of dermal connective tissue materials such as collagen, elastic fibers, and keratin through the epidermis and observed with pruritic skin lesions. In this study, we aim to clarify the clinical, histopathological, and dermoscopic characteristics of APD, identify the associated systemic disease, and figure out treatment options. This study was designed as a single-center retrospective, observational, cross-sectional study. We evaluated all accessible APD cases between January 2004 and June 2022 in a tertiary care hospital. A total of 95 patients with confirmed APD were included in the study. Sixty percent of the patients were women and 40% were men. The median age at diagnosis was 63.1 years (35-85 years). The most common site of lesions was the lower extremities which were detected in 86.31% of the patients. The concomitant systemic disease was identified in 84.21% of the patients. The most common systemic disease was type 2 diabetes mellitus (65.26%). Antihistamines and topical corticosteroids were the most commonly prescribed treatment agents. Transepidermal elimination of dermal connective tissue components is a feature of APD and the disease usually presents with pruritic papules and nodules with central keratotic crust or plug. The diagnosis of APD requires a clinical examination and histological investigation. APD is usually accompanied by systemic comorbidities. There are several topical and systemic medications available for APD, however, sometimes the therapy might be challenging.

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

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. 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. 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. Pruritus was the most common symptom, with the lower extremities as the most predilectionsites (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. 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: A Disorder Treatable with Mycophenolate Mofetil.

Acquired perforating dermatosis (APD) is an adult skin disease characterized by an umbilicated papulonodular rash with transepidermal elimination of dermal components such as collagen and/or elastin. It is frequently associated with multiple medications and diseases such as diabetes and chronic renal failure. It is a disabling disease with severe pruritus in 83.3% of cases and generalized ulcerating lesions that are associated with infections and scarring. Nearly 10% of renal patients are affected. Supportive measurements of disease activity and previous medications failed to halt its natural progression. In our study, we documented significant improvements in the severity of the disease as measured by the eczema area and severity index (EASI), in 32 patients with the renal disease through the use of mycophenolate mofetil (MMF), with EASI decreasing from 31 [interquartile range (IQR) = 4] to 3 (IQR = 4) by the 3rd month. Moreover, such changes persisted for up to 2 years despite a decrease in the dose of MMF to half after 1 year. In conclusion, our study showed that MMF is a safe and effective immunosuppressive drug for short- and intermediate-term therapy of severe APD and confirmed its autoimmune etiology.

LONGSTANDING, REPETITIVELY MISDIAGNOSED KYRLE’S DISEASE IN A PATIENT WITH END-STAGE RENAL DISEASE: A CASE REPORT

Kyrle’s disease (KD) is a rare variant of acquired perforating dermatosis (APD), associated with systemic diseases in adults, particularly chronic kidney disease (CKD) and diabetes mellitus (DM). Hyperkeratotic papules of KD are clinically important as a sign of a systemic disorder that are often misdiagnosed, so clinicopathological correlation is needed to establish the diagnosis. We report a rare case with longstanding KD, associated with end-stage renal disease (ESRD) on hemodialysis for 25 years that was repetitively misdiagnosed as folliculitis, excoriations and prurigo nodularis. The KD skin changes started to develop during earlier stages of CKD, before the kidney disease was suspected, and continued to appear in flares with extensive lesions when the systemic disease in the background was not under control. Dermoscopy revealed a 3-zonal concentric pattern, characterized by bright whitish scales in the centre, a structureless whitish-grey area surrounding the central crusts, and a peripheral, rose/brown pigmentation. Histopathological examination revealed moderately acantathotic epidermal invagination filled with a keratotic plug admixed with cellular debris and neutrophils, and a modest parakeratosis. Our goal is to emphasize that the accurate and timely diagnosis is vital to be able to monitor patients for a life threating, systemic disease such as kidney failure, and attaining better management of the dermatological status which can become longstanding and hindering, as well as to outline the importance of the multidisciplinary approach to improve outcomes in patients affected by KD.

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.

Dermoscopic features of acquired perforating dermatosis: a retrospective analysis of 19 cases

Acquired perforating dermatosis (APD) comprise a group of trans-epidermal elimination disorders. The clinical picture is characterized by the presence of multiple pruritic, keratotic, follicular/non-follicular papules, and nodules commonly over the extremities and trunk. 1 Dermoscopic features of APD are sparsely reported in the literature. 2, 3 We aimed to study the various dermoscopic features of APD. This retrospective descriptive study was conducted in a tertiary care Hospital of South India, after approval from the institutional ethical committee. Only the biopsy-proven cases of APD, from January 2014 to December 2016, were included in the study. The clinicodemographic data and clinical and dermoscopic images were retrieved for all the patients. The dermoscopic images were taken using a nonpolarized contact dermoscope and attached Nikon SLR camera.

Successful Treatment of Acquired Perforating Dermatosis in a Peritoneal Dialysis Patient Using Oral Isotretinoin

Acquired perforating dermatosis (APD) triggers severe itching, and is observed most often in patients with diabetes mellitus (DM) and chronic kidney disease (CKD). Various treatments, such as topical steroids, topical and oral retinoids, allopurinol, and ultraviolet B (UVB) phototherapy, have been used. However, as the precise etiology is unknown, there are a lack of well-established treatment guidelines. We report a peritoneal dialysis patient with a diagnosis of APD who was resistant to topical steroids, phototherapy, allopurinol, and topical retinoids. Severe pruritus persisted, which compromised his ability to lead a normal life. As a final treatment option, we used oral isotretinoin and the patient experienced a dramatic improvement.

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.

Acquired perforating dermatosis: clinicopathologic study of a 10-year period at a tertiary teaching hospital.

Acquired perforating dermatosis (APD) comprises an uncommon group of skin disorders that develop in adulthood in association with systemic diseases. The aim of this study was to characterize clinicopathologic features and treatment outcomes in a series of patients diagnosed with APD. Retrospective study of all patients diagnosed with an APD over a 10-year period (2009-2018) at a tertiary teaching hospital in Lisbon, Portugal. Fifty-seven patients with APD were identified. Thirty-five patients presented lesions in multiple anatomic areas (61.4%), and the lower limbs were the most common location. Forty-six patients reported pruritus (80.7%), which was classified as severe in 21 of them (36.8%). An underlying systemic disease was identified in 53 patients (93.0%). Diabetes mellitus (DM) and chronic kidney disease (CKD) were the most common associated systemic diseases, but psychiatric disorders, malignancies, and chronic infections were present in a significant number of patients. The combination of topical steroids with antihistamines was the most prescribed initial treatment, but only 37.8% of the patients had a complete response. Acitretin, systemic steroids, and phototherapy were the treatments associated with the best outcome. Acquired perforating dermatosis can be associated with many systemic disorders that have pruritus as a common factor. Chronic viral infections and an occult malignancy should be sought, particularly in the absence of DM and CKD. The management of APD is challenging and is best achieved with the control of the underlying systemic diseases.

Unusual eruption in association with sorafenib: a case of acquired perforating dermatosis, reactive perforating collagenosis type

The term, acquired perforating dermatoses (APD), represents a group of skin conditions that develop in adulthood and are characterized by transepidermal elimination of dermal connective tissue. This appears clinically as a papulonodule with a keratotic core. Although APD is typically associated with diabetes mellitus, chronic renal failure, and several other conditions causing generalized pruritus, there have been reports in the literature describing an association of APD with select drugs including sorafenib. We present a case of acquired perforating dermatosis in a patient with HIV and hepatocellular carcinoma undergoing treatment with sorafenib.

Acquired perforating dermatosis: clinicopathological study of 31 cases, emphasizing pathogenesis and treatment.

Acquired perforating dermatosis (APD) is a rare group of skin disorders of unknown aetiology and pathogenesis and is associated with several systemic diseases. We review the clinicopathological features, associated systemic diseases and treatment response in a series of APD patients. We conducted a retrospective observational study of all patients histologically diagnosed with APD in Hospital San Jorge (Huesca, Spain) between 2002 and 2014. Demographic and clinical features were collected from medical records. Statistical analyses were carried out using SPSS software (version 20.0; IBM Corp, Armonk, NY, USA). The study population consisted of 31 patients (19 women and 12 men), with a mean age of 54 years. Reactive perforating collagenosis (n = 15, 45%) was the most common histopathologic type. The most frequently affected area was the lower limbs (66.6%, n = 22), and pruritus was present in 19 patients (61.3%, P = 0.005). Arterial hypertension (30.3%) was the most common associated condition. Five patients were receiving treatment with infliximab when diagnosed with APD. Most patients responded to topical steroids and oral antihistamines (n = 15). This study is retrospective, and the sample size is limited. Acquired perforating dermatosis is an underdiagnosed dermatosis frequently associated with systemic disorders. Its pathogenesis may involve vascular damage, not only in patients with diabetes, but also in those with arterial hypertension and chronic venous insufficiency. Control of pruritus and underlying extracutaneous disorders, as well as discontinuation of the treatment with biologics, are important aspects of the management of this dermatosis.

A clinicopathologic study of thirty cases of acquired perforating dermatosis in Korea.

BackgroundAcquired 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.ObjectiveThe 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.MethodsWe retrospectively reviewed the medical records and biopsy specimens of 30 patients who were diagnosed with APD.ResultsThe 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%).ConclusionIn 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.

Clinicopathological features of 25 patients with acquired perforating dermatosis

Acquired perforating dermatosis (APD) is a rare group of skin disorders characterized by transepidermal elimination of dermal tissue material. There are only a few studies of APD. We aimed to review the clinicopathologic features of patients with APD and evaluate the outcomes of treatments. The medical records of 25 patients who were clinically and histopathologically diagnosed as having APD between 2006 and 2013 were retrospectively reviewed. The histopathologic sections and the results of elastic, trichrome and periodic acid Shiff (PAS) staining were evaluated. Of the patients, 17 (68%) had ARPC, 7 (28%) had perforating folliculitis, 1 (4%) had Kyrle's disease. Male/female ratio was 1.3. The mean age was 51.8 ± 12.8 years. The disease duration ranged between 15 days and 96 months. Diabetes mellitus was the most common disease associated with APD (48%). The walls of vessels were PAS positive in the lesions of 6 of 13 diabetic patients. After various treatments, almost all lesions regressed in 15 (60%) patients. Acquired perforating dermatosis may present with various clinical features and is mostly associated with diabetes mellitus. Diabetes mellitus may be involved in the pathogenesis of APD via microangiopathy and other mechanisms.

Acquired perforating dermatosis and Addison’s disease due to disseminated histoplasmosis

Acquired perforating dermatosis (APD) is a rare disorder characterized by transepidermal elimination of contents from dermis with minimal disruption of surrounding structures, believed to be due to altered expression of dermal proteins. Its occurrence in patients with systemic mycosis has never been reported. We report a 60-y gentleman who presented with features of adrenal insufficiency (nausea vomiting, hypotension and increased pigmentation) for 4 mo, multiple hyperpigmented pruritic nodules with central keratinous plug over extensor surface of both lower limbs along with hepatosplenomegaly of one month duration. Investigations revealed low cortisol (2.3 μg/dl; normal: 5–34 μg/dl), elevated ACTH (68 pg/ml; normal: 5–15 pg/ml), enlarged bilateral adrenals with hepatosplenomegaly on CT. Methanamine silver staining of fine needle aspiration from the adrenals and bone marrow aspiration showed numerous oval yeast cells suggestive of histoplasma. Histopathology of biopsy of one of the skin nodules revealed transepidermal elimination process characterized by invagination of epidermis with extrusion of collagen bundles suggestive of APD. Patient improved with hydrocortisone replacement and there was clinical improvement with resolution of skin lesions following amphotericin-B and itraconazole therapy. This is probably the first reported case of APD in a patient with disseminated histoplasmosis who had presented with Addison’s disease.

Acquired perforating dermatosis: a clinical and dermatoscopic correlation

Acquired Perforating Dermatosis (APD) is a perforating disease characterized by transepidermal elimination of dermal material [1,2]. This disease usually develops in adulthood. APD has been reported to occur in association with various diseases, but is most commonly associated with dialysis-dependent chronic renal failure (CRF) or diabetes mellitus (DM) [1,2,3,4]. Morton et al found that APD occurs in up to 10% of patients undergoing hemodialysis [5]. Additionally, Saray et al found that sixteen of twenty-two cases with APD were associated with CRF [3].

Predictors of Acquired Perforating Dermatosis in Uremic Patients on Hemodialysis: A Case-Control Study

Objectives. The purpose of this study was to identify the predictors of acquired perforating dermatosis (APD) in patients on maintenance hemodialysis (HD). Methods. A case-control study was performed at our outpatient HD center between January 1, 2000 and March 31, 2011. Patients on HD with APD were matched (1 : 2) for gender and age with controls on HD. Conditional logistic regression was used to identify factors associated with APD. Results. A total of 19 patients with APD and 38 age and gender matched patients were enrolled in the study. Univariate logistic regression showed that APD was significantly associated with diabetes mellitus (DM), reduced levels of intact parathyroid hormone (iPTH) and albumin (Alb), reduced dialysis adequacy (Kt/V) and urea reduction rate (URR), and elevated levels of high-sensitivity C-reactive protein (hsCRP). Multivariate logistic regression identified reduced iPTH (hazard ratio (HR): 0.983; P = 0.026) and Alb (HR: 0.099; P = 0.047) and elevated hsCRP (HR: 1.210, P = 0.024) as risk factors for APD. Conclusions. iPTH, hsCRP, and Alb are predictors for APD in HD patients.

AGE-Modified Collagens I and III Induce Keratinocyte Terminal Differentiation through AGE Receptor CD36: Epidermal–Dermal Interaction in Acquired Perforating Dermatosis

To clarify the molecular mechanism underlying the transepidermal extrusion of dermal collagen in acquired perforating dermatosis (APD) associated with diabetes mellitus and renal failure, we studied the interaction between advanced glycation end product (AGE)-modified extracellular matrix proteins and keratinocytes (KCs) in a cell culture system. The expression of involucrin (INV) and keratin 10 was significantly enhanced in normal human KCs grown on AGE-modified collagen I or III compared with cells grown on unmodified collagen I or III. Glycated collagens I and III preferentially induced the expression of AGE receptor CD36, but not of other AGE receptors. KCs induced to terminal differentiation demonstrated markedly elevated CD36 expression. Glycated collagen I- and III-induced INV expression was partially blocked by the anti-CD36 antibody (Ab). These substrates also induced epidermal matrix metalloproteinase 9 (MMP-9) expression. Lesional skin from APD patients reacted moderately or strongly with the anti-CD36 Ab as well as the anti-MMP-9 Ab in the epidermal cells surrounding the collagenous materials being eliminated. These results suggest that exposing KCs to AGE-modified interstitial collagen (types I and III) by scratching induces terminal differentiation of KCs via the AGE receptor (CD36), leading to the upward movement of KCs together with glycated collagen.

Acquired perforating dermatosis: clinicopathological features in twenty‐two cases

The term of acquired perforating dermatosis (APD) comprises the perforating dermatoses occurring in adult patients. Clinical and histological features of the disease are not uniform, and may resemble any of the four classic perforating disorders: elastosis perforans serpiginosa, reactive perforating collagenosis, perforating folliculitis or Kyrle's disease. Chronic renal failure and/or diabetes mellitus usually accompany this skin disease. The aim of this study was to delineate the clinical and histopathological features of acquired perforating dermatosis and to investigate the potential relationship between this disease and associated conditions. Twenty-two patients with acquired perforating dermatosis were enrolled in this study. Clinical findings of acquired perforating dermatosis and the spectrum of associated diseases were investigated. Haematoxylin and eosin sections were re-examined, and immunohistochemical stainings (elastic van Gieson and Masson trichrome stains) and periodic acid-Schiff stain were also used for histopathological evaluation. Different clinical types of lesions resembling reactive perforating collagenosis, perforating folliculitis or Kyrle's disease were observed. Histopathological features were consistent with any of the four types of perforating dermatoses. Most of the patients (86.4%) had at least one systemic disease. Chronic renal failure (72.7%) and diabetes mellitus (50%) were the most commonly associated conditions. Most of the patients with diabetes mellitus (90.9%) had chronic renal failure due to diabetic nephropathy. All of the patients with chronic renal failure were on dialysis treatment. The other associated conditions were hepatitis (27.3%), anti-HCV Ab-positivity (13.6%), hypothyroidism (9.1%) and tuberculosis lymphadenitis (4.5%). Of the 22 patients, 13.6% were otherwise healthy, and 9.1% were renal transplant recipients. Clinicopathological findings of our study indicate that the cases with APD represent the broad spectrum of perforating disorders rather than the variants of the same disease. Although APD is frequently associated with diabetes mellitus and chronic renal failure, this skin disorder may also develop in patients with other systemic disorders, and in those without any medical problems. This skin disease is probably linked to dialysis treatment in patients with chronic renal failure due to diabetes mellitus or other causes.

Acquired perforating dermatosis: A report on 4 cases associated with scabies infection

To the Editor: Acquired perforating dermatosis (APD) belongs to a group of perforating skin disorders with an obligatory transepidermal elimination of dermal components (TEE). They are divided into primary and secondary forms: primary perforating dermatoses include idiopathic, distinct entities such as elastosis perforans serpiginosa, perforating folliculitis, Kyrle's disease, and reactive perforating collagenosis. A secondary form is APD, formerly known as acquired reactive perforating collagenosis.1 APD has been reported to be associated with diabetes mellitus, chronic renal failure, malignant neoplasia, liver disorders, and HIV infection.2 Pruritus is the most prominent feature, and linear configuration and Koebner phenomenon can be observed occasionally. The TEE tissue is variable in APD, where collagen and/or elastic fibers can be found.3 Clinically, umbilicated papules and nodules with a central adherent, brownish-greenish keratotic plug characterize APD, histologically showing TEE of altered fibers into a cup-shaped epidermal depression. These findings combined with an onset after the age of 18 years have been described by Faver et al4 as obligatory diagnostic criteria for APD. Topical steroids, systemic retinoids, phototherapy, h1-antihistamines, allopurinol, doxycycline, cryotherapy, and liquid nitrogen have been used for treatment with varying degree of success. Recently, Kurschat et al5 reported one case of APD triggered by scabies infection. Accordingly, we here describe 4 cases of scabies-provoked APD.

UVB for APD

Some patients with diabetes mellitus or chronic renal failure develop acquired perforating dermatosis (APD). The abnormal transepidermal elimination of degenerated connective tissue that produces the clinical features of APD may respond to topical steroids, but some cases prove more difficult to treat. Japanese investigators tested …