Kyrle's Disease Research Articles

A case report on Kyrle’s disease in patient with diabetes mellitus

Kyrles disease classified under acquired perforating dermatosis, is a rare skin condition predominantly affecting 30-50-year-old females. Characterized by pruritic hyperkeratotic and ulcerated nodules, the disease involves the transepidermal clearance of aberrant endogenous components, leading to inflammatory reactions and expulsion of keratin and cellular material. The etiology though not fully understood, suggests changes in dermal connective tissue may play a role. In case report, a 56-year-old male with type II diabetes mellitus presented knee pain and 3 mm papules on lower limbs. Laboratory findings revealed hematological and metabolic abnormalities, and a skin biopsy confirmed Kyrle’s disease features. Laboratory reports indicated abnormalities in hematology, blood glucose, lipid profile, hepatic and renal function. Biopsy results confirmed acanthosis, hyperkeratosis, dysplsia and granulomatous inflammation. The association of Kyrle’s disease and metabolic abnormalities, emphasizing diabetes and chronic renal failure. Potential pathways involve oxidative damage, endoplasmic stress, and vasculopathy. Treatment options include keratolytics, emollients, antihistamines. Kyrle’s disease often linked to diabetes and chronic renal failure, is a chronic condition with variable remission periods. Early detection and appropriate management are crucial for improving patients’ quality of life. Understanding the association with metabolic abnormalities guides effective therapeutic approaches for this rare dermatological condition.

A case of Kyrle's disease successfully treated with topical benzoyl peroxide

A case of Kyrle's disease successfully treated with topical benzoyl peroxide

Kyrle’s Disease: A Case Report and Literature Review

BACKGROUND: Perforating dermatoses are heterogeneous skin disorders characterized by transepidermal elimination of dermal tissue components. They can be characterized by the primary or secondary pathogenesis. Primary perforating dermatoses can be divided into four types, according to the eliminated dermal materials: Kyrle disease, perforating reactive collagenosis, elastosis perforans serpiginosa, and perforating folliculitis. The secondary form is also known as acquired perforating dermatosis and designates adult patients with coexisting systemic diseases, regardless of the dermal materials eliminated. The aim of this review is to investigate the potential relationship between Kyrle disease and associated conditions, reporting a rare case of this disease in a patient with chronic kidney disease. CASE PRESENTATION: We report the case of a 55-year-old male patient who presented generalized pruritus and skin lesions on the upper limbs and trunk for about 6 months. Pathological history revealed systemic arterial hypertension and chronic kidney disease (CKD), for which the patient had been on hemodialysis since more than 3 years. CONCLUSION: We reviewed the literature, reporting a rare case of KD in a patient with chronic renal failure on hemodialysis. KD is an acquired dermatologic condition that lacks a standardized treatment approach and can be notoriously difficult to cure. This article provides an overview of the most frequent comorbidities and treatment methodologies previously reported in the literature. If KD is suspected, underlying systemic disorders need to be investigated.

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.

Acquired perforating dermatoses show increased levels of cutaneous advanced glycation end-products.

Acquired perforating dermatoses (APDs) are characterized by transepidermal elimination of skin materials. Altered glycation of dermal components may be involved in pathogenesis. To assess whether patients affected by APDs have increased levels of cutaneous advanced glycation end-products (AGEs). A cross-sectional controlled study involving a total of 109 patients was conducted, enrolling 29 patients consecutively diagnosed with primary APDs [reactive perforating collagenosis (RPC), elastosis perforans serpiginosa (EPS), perforating folliculitis (PF) and Kyrle disease (KD)], 40 age- and sex-matched healthy controls (HCs) and 40 patients with mild atopic dermatitis (AD). The levels of cutaneous AGEs were measured using a validated fluorescence technique. The median skin autofluorescence value in patients with APDs was significantly higher [2.7 arbitrary units (AU), interquartile range (IQR) 1.9-3.9 AU] compared with HCs (1.8 AU, IQR 1.6-2.3 AU; P < 0.001) and patients with AD (2.1 AU, IQR 1.9-2.3 AU; P = 0.01). Median values were 3.5 AU (IQR 2.7-4.6 AU) for RPC, 1.83.5 AU (1.4-2.4 AU) for EPS, 3.1 AU (2.4-4.4 AU) for PF and 2.6 AU (2.3-3.1 AU) for KD. Our results may suggest a possible physiopathological role of AGEs in the transepidermal elimination mechanisms involved in certain APDs.

Kyrle’s disease (KD): “An Update with review of literature” A Spongebob Skin pores simulation

Kyrle’s disease (KD) is a Chronic skin condition first described by Austrian pathologist Josef Kyrle in 1916. Kyrle referred to this condition as hyperkeratosis follicularis &amp; parafollicularis in cutem penetrans. These diseases are characterized by the phenomenon of transepidermal elimination of denatured dermis an acquired form of perforating dermatosis [14]. It is characterized by keratotic crater plugs that develop in hair follicles penetrating the epidermis and extending into the dermis [4]. This trans epithelial migration and elimination of proteinaceous components is associated with systemic disorders like renal, liver diseases, chronic heart failure and diabetes mellitus. We present two case scenarios of a young Males with multiple chronic papular eruptions along with a review of literature for Kyrle’s disease (KD).

The diagnostic process of Kyrle's disease in a 65-year-old patient.

The diagnostic process of Kyrle's disease in a 65-year-old patient.

Clinical practice guide for the treatment of perforating dermatosis

Perforating dermatoses are a heterogeneous skin disease group defined by transepidermal elimination of various skin materials. Four classical forms of primary perforating dermatosis have been described, where the transepidermal elimination mechanism represents the hallmark of the disease: acquired reactive perforating collagenosis, elastosis perforans serpiginosa, Kyrle's disease and perforating folliculitis. Acquired reactive perforating collagenosis presents with transepidermal elimination of collagen fibers. Elastosis perforans serpiginosum presents with the elimination of elastic fibers. Kyrle's disease presents with transepidermal elimination of abnormal keratin. In perforating folliculitis, it is the content of the follicle. We established diagnostic criteria and severity classification. In addition, the Japanese guideline for treatment of perforating dermatoses was updated using the Medical Information Network Distribution Service (MINDS) methodology. The guideline is based on a systematic published work review completed from 1989 to 2019, and on a formal consensus and approval process. Most medical published work on the treatment is limited to individual case reports and small series of patients. The guideline covers treatment options considered relevant by the expert panel and approved in Japan at the time of the consensus conference.

A clinical study of cutaneous manifestations in diabetes mellitus

Background: Diabetes mellitus (DM) is the most common endocrine disorder1.At least 30% of diabetics have some cutaneous involvement during the course of their disease 1. Type II patients do develop more frequent cutaneous infections, whereas type I patients develop more autoimmune- type cutaneous lesions 2,3. Objective: To evaluate the prevalence and pattern of skin manifestations among diabetic patients. Methods: 100 patients of diabetes mellitus with skin manifestations who consecutively attended DVL outpatient department and willing to participate in the study were enrolled in this study. Skin examination was done and findings entered to computer database for analysis. Results: Among 100 patients, there were 56 males and 44 females the youngest patient being 19 years old and eldest was 77 years old. Cutaneous infections were the most commonly observed dermatoses (51%), followed by dermatoses more commonly associated with diabetes mellitus (36%), non specific dermatoses (23%), neuropathic and ischemic diabetic skin disease (5%), due to microangiopathy (4%), metabolic disorders (3%) and cutaneous reactions to therapy (3%).Among cutaneous infections, fungal infections (38%) are the most common dermatoses followed by bacterial infections (11%) and viral infections (2%). Tinea corporis was the most common fungal infection and furunculosis (3%) was most common bacterial infection. Among the common dermatoses associated with diabetes mellitus (36%) majority presented with pruritus secondary to xerosis (12 patients, 12%) followed by 11 cases (11%) of psoriasis, 4 cases of vitiligo, 2 patients each (2%) of acrochordons, lichen planus and macular amyloidosis, 1 patient each (1%) of acanthosis nigricans, alopecia universalis and kyrles disease. Among patients with non specific dermatoses, eczema was most common followed by pemphigus, acne vulgaris and hidradenitis suppurativa. Conclusion: Infections were the most common cutaneous manifestations in diabetes, followed by dermatoses most commonly associated with diabetes. Cutaneous manifestations are more common in patients who have overall poor glycemic control. Cutaneous manifestations can heighten the suspicion of a physician regarding the diagnosis of diabetes and help to prevent systemic derangements by early institution of appropriate treatment.

The treatment of Kyrle's disease: a systematic review

Kyrle's disease (KD) is a cutaneous disease that develops in individuals with underlying systemic disease, particularly chronic renal failure and diabetes mellitus (DM), and is associated with a high burden of disease linked to itch. The intensely pruritic, hyperkeratotic papulonodular rash seen in KD dramatically impairs patients' quality of life and increases their risk of mortality. Unfortunately, no guidelines or evidence-based regimens have been specifically developed for KD, making the treatment of this disease particularly challenging for physicians. This article aims to provide the first comprehensive, up-to-date overview and analysis of treatment options employed for KD. A search of the PubMed/MEDLINE and Scopus databases was performed for articles regarding the treatment of KD, published in English between 1990 and 2019. Seventy-three articles were identified, of which eighteen met the inclusion criteria. We discovered that a wide variety of treatment regimens for KD have been reported in the literature, including oral antibiotics, immunosuppressants, phototherapy, topical/systemic retinoids, topical keratolytics and various combination therapies, which include some of the aforementioned treatments, in conjunction with oral/topical/injectable steroids, emollients and/or antihistamines. The use of a combination regimen is the most commonly practiced therapeutic approach to KD. Topical corticosteroids and depot corticosteroid injections repeatedly appeared in many of the regimens encountered during our search. While no definitive recommendations can be made based on existing literature, this article provides physicians with a summative outline that can help guide management and be referenced when other treatment efforts fail. The increasing prevalence of renal disease, DM and other chronic diseases will inevitably lead to rising rates of KD in the upcoming years. While randomized controlled trials are greatly needed, novel antipruritic immunomodulatory drugs targeting specific interleukin receptors (IL-4/13/31) and intracellular signalling (e.g.Janus kinase) pathways may have a potential role in the treatment of this disease.

Ulcerative nodules on the entire body

Ulcerative nodules on the entire body

The novel use of mu-opioid receptor antagonism for the treatment of refractory pruritus in Kyrle's disease.

The novel use of mu-opioid receptor antagonism for the treatment of refractory pruritus in Kyrle's disease.

An Observational Study of Dermatological Manifestations in Patients of Chronic Renal Failure Undergoing Hemodialysis

Background: Chronic renal failure (CRF) presents with an array of cutaneous manifestations. Newer changes are being described since the advent of hemodialysis, which prolongs the life expectancy, giving time for these changes to manifest. Aim: The aim of this study was to evaluate the prevalence of dermatologic problems among patients with chronic renal failure (CRF) undergoing hemodialysis. Methods: ninty three .. patients with CRF on hemodialysis were examined for cutaneous changes. Results: all the patients complained of some skin problem. The most prevalent finding was xerosis (54.8%), followed by pallor (21.5%), pruritus (19.35%) and cutaneous pigmentation (18.3%). Other cutaneous manifestations included Kyrle's disease (5.4%); fungal (5.4%), bacterial (6.5%) and viral (3.2%) infections; purpura (9.7%). The nail changes included half and half nail (21%), koilonychia (39.78%), onychomycosis (1.08%), onychorrhexis (6.45%), splinter hemorrhages (1.08%), and Beau's lines (2%). Hair changes included sparse hair (18.28%), and brittle and lusterless hair (11.83%). Oral changes included macroglossia with teeth markings (23.65%), . Some rare manifestations of CRF like uremic frost, gynecomastia and pseudo-Kaposi's sarcoma were not observed. Conclusions: CRF is associated with a complex array of cutaneous manifestations caused either by the disease or by treatment. The commonest are xerosis and pruritus and the early recognition of cutaneous signs can relieve suffering and decrease morbidity.

A Case of Kyrle's Disease with Dermatoscopic Findings.

A Case of Kyrle's Disease with Dermatoscopic Findings.

Dermatological manifestations in chronic renal failure patients with and without hemodialysis: A study at a tertiary care centre

Background: Skin is an important diagnostic window to many diseases of internal organs including the renal system. Subtle changes in the skin may act as a clue to the underlying renal pathology. The cutaneous manifestations may be caused either by the disease or drugs used in the treatment of chronic kidney disease (CKD). Aim: The aim of this study is to investigate and compare cutaneous manifestations in patients with CKD on hemodialysiswith patients not on dialysis. Materials and Methods: One hundred patients with CKD were recruited, 50 each in maintenance hemodialysis (MHD) and nondialysis group. Detailed cutaneous examination and necessary investigations were done, and cutaneous manifestations were compared among the two groups. Results: Of the 100 patients, 72 were males and 28 were females. Mean duration of disease was 32.4 months in the dialytic group and 11.2 in the nondialytic group. The most common underlying disease that lead to CKD was diabetes mellitus. Specific cutaneous manifestations observed were Kyrles disease 4%, calcinosis cutis 1%, and uremic frost in 1%. The common nonspecific manifestations observed were xerosis in 59% (dialytic 61.03%, nondialytic 38.98%), pallor in 57% (dialytic 49.1%, nondialytic 50.9%), pruritus in 38%, (dialytic 63.16%, nondialytic 36.84%), pigmentation in 32% (dialytic, 65.63%, nondialytic 34.37%), purpura in 8% of dialytics, and none of the nondialytic patients had purpura. Cutaneous infections were found in 28%, of which fungal were 20%, bacterial 7%, and viral infections 2%. Nail changes were observed in 46% (dialytic 62%, nondialytic 30%), hair changes in 16%, and mucosal manifestations in 11% of the patients. Puncture marks were found in 74% of the dialytic patients. Xerosis, pruritus, and pigmentation had significantly higher prevalence in diabetic uremics compared to nondiabetic uremics. Conclusions: There was a significant association with the mean duration of disease, which is higher in dialytic patients compared to the nondialytic group. Xerosis, pruritus, and hyperpigmentation were common in dialytic patients.

Comparison of cutaneous manifestations of diabetic with nondiabetic patients: A case-control study

Background: Diabetes mellitus is a metabolic disorder characterized by raised fasting and postprandial blood glucose levels and a variety of multisystem complications. The prevalence of skin manifestations seems to be similar between type 1 and type 2 diabetes mellitus patients. Cutaneous manifestations usually develop following the diagnosis of diabetes, but in some patients, they are the initial presenting signs, thereby helping in the early diagnosis of diabetes mellitus. Objectives: The objective of this study is to describe the cutaneous lesions in patients with type 2 diabetes mellitus and to compare the pattern of skin manifestations in diabetics and nondiabetics in coastal Karnataka and neighboring districts of Kerala. Methodology: This case-control study included 400 patients with type 2 diabetes mellitus and cutaneous manifestations attending the outpatient Departments of Dermatology and General Medicine and those admitted under these departments. A total of 400 age and sex-matched nondiabetic patients were included as controls. Results: Cutaneous infections were noted in 148 (37%) diabetics of which fungal infections were the most common seen in 106 (26.5%) patients, followed by xerosis in 121 (30.25%) and acrochordons in 71 (17.75%) patients. Other dermatoses associated with diabetes mellitus noted were acanthosis nigricans (5.5%), scleredema diabeticorum (0.25%), diabetic bullae (0.5%), and Kyrle's disease (1%). Cutaneous changes associated with neurovascular complications included diabetic foot in 3%, diabetic dermopathy in 2%, and pigmented purpuric dermatosis in 0.25% of the diabetics. Conclusion: A joint effort between dermatology and general medicine is necessary for the early recognition and treatment of the skin conditions and also to ensure adequate metabolic control.

In search of cutaneous marker for retinopathy in diabetic patients: A pilot study

Background: Diabetes mellitus is one of the most common causes of microangiopathy. Approximately 30% of all diabetics show skin lesions during their lifetime. However, there is a paucity of data on the association of diabetic retinopathy with the skin changes. Objectives: The aim is to study noninfectious diabetes associated dermatoses in patients with diabetic retinopathy and to compare the frequencies of dermatoses in diabetics with retinopathy, diabetics without retinopathy and nondiabetics. Materials and Methods: We screened 400 diabetic patients and found 145 diabetics with cutaneous manifestations were positive for diabetic retinopathy after fundoscopic examination by a qualified ophthalmologist. One hundred and forty-five age- and sex-matched diabetics with cutaneous manifestations and without retinopathy, and another 145 age- and sex-matched nondiabetics with normal random blood sugar (RBS) levels and cutaneous manifestations formed the control groups. Statistical analysis was performed using SPSS version 16 and Chi-square test. Results: Acquired ichthyosis was the most common finding which was seen in 70 (48.27%) diabetics followed by acrochordons in 18 (12.41%) diabetics. Other noninfectious dermatoses associated with Diabetic Mellitus seen among the cases include diabetic foot in 7 (4.8%), psoriasis in 5 (3.44%), acanthosis nigricans in 4 (2.75%), pruritus in 3 (2.068%), vitiligo in 3 (2.068%) patients, Kyrle's disease in 3 (2.068%), and diabetic bullae in 2 (1.37%) patients. Conclusions: Diabetic retinopathy is not an uncommon cause of ocular morbidity. Our observation calls for the need of a holistic approach toward the diagnosis and treatment of diabetes and diabetes-associated skin and systemic complications.

Dermoscopy of Kyrle disease

Dermoscopy of Kyrle disease

Acquired cutaneous perforating disorders: Clues to diagnosis by silhouette

Inflammatory dermatoses are interpreted by pattern recognition. Primary perforating disorders including reactive perforating collagenosis, elastosis perforans serpiginosa, perforating folliculitis, and Kyrle's disease show clinical and histopathological overlap. This article highlights the importance of architectural based analysis by “silhouette” observed under scanning magnification for diagnosis of primary perforating disorders.

A study of cutaneous manifestations associated with diabetes mellitus

Background: Diabetes mellitus is a fairly common medical disorder that involves almost every specialty in its spectrum of clinical manifestation, and up to 1/3 rd of patients with diabetes mellitus are estimated to have cutaneous changes. In other words using broadest criteria all subjects with diabetes will diabetes will develop cutaneous manifestations of this disease. Methods: A total of 100 diabetic patients with cutaneous manifestations, who attended skin OPD at Civil Hospital, Belgaum, Karnataka, India and K.L.E’S Hospital and Medical Research Centre, were randomly selected. The present study was conducted over a period of 22 months from March 1995 to December 1996. Total 100 cases were included in this study. The male to female ratio is 1.6:1 approximately. Detailed history, physical examination, cutaneous and mucous membrane involvement examination was done. Diabetes Mellitus was confirmed by urine sugar and blood sugar estimation. The diagnosis of diabetes mellitus was done on the basis of criteria laid down by national diabetic data group. Urine and blood sugar estimation was done in all the cases. Results: Fungal infections were common cutaneous manifestation followed by bacterial infections and generalized pruritus. Those patients who were on oral hypoglycaemic drugs, insulin therapy found to have photodermatitis and localized lipoatrophy. The common associated skin diseases with diabetes mellitus were fungal infection (38%) and viral infections (2%). Unexplained generalized pruritus was observed in (17%) cases, followed by pruritus ani is (3%) and prurigo simplex in (1%). Cutaneous markers of diabetes i.e acrochordons were observed in 17% cases. The other manifestations observed were lichen planus (6%), PLE (3%), psoriasis (3%), vitiligo (2%), Kyrles disease (2%), infected eczema (2%), scabies (2%), lichen simplex chronicus (2%) and pemphigus vulgaris (1%). Conclusions: The physicians should be aware of cutaneous manifestations in diabetes mellitus. Where the ignorance of skin manifestations in diabetes or improper treatment may makes the condition worse. The early detection and early treatment of common skin manifestations in diabetes will prevent further complications or ineffectiveness due to treatment especially in cases of carbuncle, extensive tinea corporis , psoriasis, lichen planus, macro and micro angiopathies, trohic ulcers etc. A well-controlled diabetes by drugs, diet and exercise will allow the patient to lead a normal life like any other normal individuals.