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Linear hyperkeratotic umbilicated papules on the extensor surface of the extremities and the trunk (A, B, C, D). Note Koebner phenomenon from scratching.
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Acquired perforating dermatosis (APD) is a skin disorder occurring in the patients with chronic renal failure (CRF), diabetes mellitus (DM) or both. The purpose of this study was to clarify the clinical and histopathological features of APD, and evaluate role of scratching in the pathogenesis of APD. Twelve patients with APD associated with CRF and...
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Citations
... A Morbus Kyrle felnőttkorban ritka, gyermekkorban extrém ritka bőrbetegség, a pontos diagnózis felállításához a szövettani mintavétel elengedhetetlen. Leggyakrabban krónikus vesebetegséggel, diabetes mellitussal (3,16), de akár májbetegséggel, kongesztív szívelégtelenséggel, hyperlipidaemiával, vagy HIV fertőzéssel is társulhat. ...
Four major forms of primary perforating dermatoses have been described. Diagnostic workup may be challenging, the highly advised skin biopsy demonstrates the transepidermal elimination of dermal connective tissue. Hyperkeratosis follicularis et parafollicularis in cutem penetrans also known as “Kyrle’s disease”, is classified as a subtype of acquired perforating dermatoses. Kyrle’s disease is extremely rare in children and is often associated with underlying chronic conditions.
... The frequency and key clinical characteristics of skin problems are high in patients with insulin-dependent (IDDM) or noninsulin-dependent (NIDDM) diabetes [5]. The most common dermatological issues in IDDM and NIDDM patients were vitiligo and psoriasis, cutaneous [6] necrobiosis lipoidica [7], diabetic dermopathy [8], diabetic bullae [9], yellow skin [10], eruptive xanthomas [11], perforating disorders [12], acanthosis nigricans [13], and oral leucoplakia [14]. ...
Aim:
Analyse the diabetes mellitus (DM) of a person through the facial skin region using vision diabetology. Diabetes mellitus is caused by persistent high blood glucose levels and related complications, which show variation in facial skin regions due to reduced blood flow in the facial arteries. Materials and Method. In this study, 200 facial images of diabetes patients with skin conditions such as Bell's palsy, rubeosis faciei, scleroderma, and vitiligo were collected from existing face videos. Moreover, face images are collected from diabetic persons in India. Viola Jones' face-detecting algorithm extracts face skin regions from a diabetic person's face image in video frames. The affected skin area on the diabetic person's face is detected using HSV colour model segmentation. The proposed multiwavelet transform convolutional neural network (MWTCNN) extracts the features for diabetic measurement from up- and downfacial scaled images of diabetic persons.
Results:
The existing deep learning models are compared with the proposed MWTCNN model, which provides the highest accuracy of 98.3%.
Conclusion:
The facial skin region-based diabetic measurement avoids pricking of the serum and is used for continuous glucose monitoring.
... 5 Altered collagen may be induced by hypoxic states due to vessel wall thickening associated with diabetic microvasculopathy. 6 Furthermore, advanced glycation end (AGE) collagens, promoted by hyperglycemia and oxidative states, may promote the elimination of collagen. Keratinocytes cultured on AGE collagens displayed higher levels of differentiation markers. ...
... Perforating disorders of the skin are a group of diseases with transepidermal elimination of material from the upper dermis. Some authors have classically subdivided the condition into primary and secondary [1,2]. It is further divided into four types according to the eliminated dermal material: Kyrle's disease (KD), perforating reactive collagenosis (PRC), elastosis perforans serpiginosa (EPS), and perforating folliculitis (PF) [1][2][3][4]. ...
... Some authors have classically subdivided the condition into primary and secondary [1,2]. It is further divided into four types according to the eliminated dermal material: Kyrle's disease (KD), perforating reactive collagenosis (PRC), elastosis perforans serpiginosa (EPS), and perforating folliculitis (PF) [1][2][3][4]. Transdermal elimination of collagen, elastic fibers, and degenerated follicular contents with or without collagen or elastic fibers is seen in PRC, EPS, and PF, respectively. In contrast, transdermal elimination of keratotic material with no collagen or elastic fibers is observed in what is known historically as KD [3]. ...
... The PRC subtype was described in most of these cases, and hence, this may necessitate thorough further investigation, especially if the patient has a history of malignancy. Lesions occur predominantly on the trunk and extensor limb surfaces [2,6,7]. The incidence in North America ranges between 4.5 and 10% of patients receiving chronic hemodialysis, among whom is a high proportion of black patients [2,6]. ...
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.
... 8 Multiple hypotheses regarding the pathogenesis of acquired perforating dermatoses exist, identifying possible triggers for degeneration and elimination of collagen fibers, to include trauma, microvasculopathy, and biochemical collagen alterations such as microcrystalline calcium salt deposition. [9][10][11] Although some cases of calcinosis cutis have been shown to resolve spontaneously over the course of months, treatment for calcinosis cutis should be considered depending on the severity of symptoms. 4 There is no standard therapy, although various treatment modalities have been reported to be beneficial, to include topical sodium thiosulfate cream, warfarin, bisphosphonates, minocycline, ceftriaxone, diltiazem, aluminum hydroxide, probenecid, intralesional corticosteroids, intravenous immunoglobulin, curettage, surgical excision, carbon dioxide laser, and extracorporeal shock wave lithotripsy. ...
Iatrogenic calcinosis cutis represents a subset of calcinosis cutis resulting secondary to treatments or procedures. We present the first report of calcinosis cutis resulting from the intraosseous infusion and one of a few cases with associated transepidermal elimination. A previously healthy 2-year-old female presented with a new-onset unilateral shin rash 1 week following hospitalization for a near-drowning event. A dermatologic exam revealed multiple small, tender, firm, chalky-white papules with surrounding erythema, in addition to two erythematous macules superior and medial to the papular lesions, corresponding to prior intraosseous access sites. The rash persisted despite trials of topical mupirocin and acyclovir cream, prompting a referral to a dermatologist. An excisional biopsy was performed, revealing circumscribed dermal deposits of acellular basophilic material connected to the overlying epidermis through an invaginated keratin plug. A von Kossa silver stain highlighted the deposits, confirming the diagnosis of perforating calcinosis cutis. The lesions did not recur following the excisional biopsy. Iatrogenic calcinosis cutis may be seen as a complication of the infusion of calcium-containing fluids via intraosseous access, in addition to the more commonly observed peripheral intravenous access. Awareness of this disorder is important in order to distinguish it from an infectious mimic and guide the selection of therapy.
... The association of APD in patients with diabetes mellitus and CKD is well documented in published literature, although the exact pathophysiology of APD is unknown. 6 Copper has been implicated in the pathogenesis of acquired elastosis perforans serpiginosa due to its association with penicillamine, a copper chelator used in Wilson disease. 7,8 One proposed mechanism for penicillamine causing elastosis perforans serpiginosa may be indirect inhibition of the copperdependent enzyme lysyl oxidase that is necessary for cross-linkage of elastic and collagen fibers within the dermis. ...
... Perforating dermatosis comprises a group of conditions in which transepidermal elimination of material from the upper dermis occurs (including collagen, elastic fibers, necrotic connective tissue, and keratin, among others) [1,2,3,4]. Acquired perforating dermatosis (APD), a term introduced in 1989 by Rapini et al. [5], encompasses Kyrle's disease, perforating folliculitis, acquired elastosis perforans serpenginosa, and acquired reactive perforating collagenosis, which were previously thought to be separate disorders. APD occurs in adult patients and is most frequently found in patients with diabetes or chronic kidney disease (CKD), particularly among those undergoing hemodialysis [1,2,10], in whom the prevalence of APD can reach 11% [3,4,6]. ...
... Acquired perforating dermatosis (APD), a term introduced in 1989 by Rapini et al. [5], encompasses Kyrle's disease, perforating folliculitis, acquired elastosis perforans serpenginosa, and acquired reactive perforating collagenosis, which were previously thought to be separate disorders. APD occurs in adult patients and is most frequently found in patients with diabetes or chronic kidney disease (CKD), particularly among those undergoing hemodialysis [1,2,10], in whom the prevalence of APD can reach 11% [3,4,6]. APD is of particular concern when the cause of renal failure is diabetic nephropathy [2]. ...
... APD occurs in adult patients and is most frequently found in patients with diabetes or chronic kidney disease (CKD), particularly among those undergoing hemodialysis [1,2,10], in whom the prevalence of APD can reach 11% [3,4,6]. APD is of particular concern when the cause of renal failure is diabetic nephropathy [2]. Other associated pathologies have been identified, such as human immunodeficiency virus (HIV), tuberculosis, neoplasia, liver disease, hypothyroidism, or hyperparathyroidism [1,3,8]. ...
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.
... [2,3] Although the pathogenesis of APD is still uncertain, microtrauma, in predisposed patients, can trigger transepidermal elimination and degeneration of the collagen fibers. [4] In addition, insufficient blood supply as a result of microangiopathy may contribute to collagen damage and local inflammatory reactions. Leukocyte infiltration seems to be a predisposing factor for focal necrobiosis of tissue. ...
... It's pathophysiology in diabetes has been elucidated: in fact, hyperglycemia alters structural proteins and results in the production of terminal glycation products [2]. This results in glycation of the extracellular matrix, which causes the production by keratinocytes of involucrin, a molecule that promotes their expulsion as well as that of collagen through the epidermis [3]. ...
Acquired perforating dermatosis (APD) is an uncommon disease characterized by lesions exhibiting transepidermal elimination of collagen or elastic fibers. APD affects adults and is associated with systemic diseases, mainly diabetes mellitus and renal failure. We report a new Moroccan case of this rare entity. It’s about a 62-year-old patient, followed for diabetes mellitus and renal failure under dialysis and who presented an extremely itchy rash, made of nodules with a keratotic center and associated with multiple scratching lesions. The skin biopsy showed a perforated epidermis with the removal of material containing keratin and collagen. the outcome was favorable with oral corticosteroids with a follow-up of one year. APD is a rare dermatosis that affect considerably the quality of life of patients. its treatment remains suspensive pending the treatment of the cause.
... It may be difficult to make any conclusions about associations of APD with malignancy, since the case numbers in our study were so low but an occult neoplasm should be kept Periodic acid-Schiff (PAS) positive wall thickness increase, which is an indicator of angiopathy, has been reported by some authors. 1,3,5,21,24 But PAS positivity had not been observed in our study. 28.8% of our patients were followed by ophthalmology due to diabetic retinopathy. ...
... Some authors believe that microangiopathy plays a role in the pathogenesis of the disease. It is thought that microangiopathy may cause necrosis after microtrauma following scratching and as a result of hypoxia due to vasculopathy necrotic material (collagen etc.) may be eliminated through the epidermis.16,21,24 ...
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.
