Kyrle's Disease Research Articles

“Cutaneous manifestations in chronic renal failure patients on haemodialysis”

Introduction: Chronic renal failure presents with wide range of cutaneous manifestations. Many newer changes have been described since the advent of haemodialysis which prolongs the life expectancy giving time for these changes to manifest. Aims & Objectives: To find the array of various dermatological conditions that can occur in patients of CRF on haemodialysis. Materials and Methods: A total 100 patients with chronic renal failure on haemodialysis and having at least single cutaneous manifestation was included in the study. Results: Of the total 82% of the patients complained of certain skin problems however on examination all patients had at least one skin problem attributable to chronic renal failure. The most prevalent finding among them was Xerosis (52%), Pruritus (32%), Diffuse cutaneous hyperpigmentation (22%), Kyrle’s disease (71%) of the total of acquired reactive perforating dermatosis(7%), Fungal infections (16%), Bacterial infections (10%) and Viral in (10%), Purpura (3%) and other Dermatosis (13%).The nail changes observed were Half and Half nails (20%), Koilonychia (18%), Onychomycosis (15%), Sub-ungual hyperkeratosis (8%), Beaus lines (2%) .Hair changes were sparse body hair (10%), dry lustreless hair (4%), and sparse scalp hair (10%). Oral changes observed were Ulcerative stomatitis (29%), Angular chelitis (12%. Other manifestations of CRF observed were like gynaecomastia, psuedokaposis sarcoma, and nephrogenic fibrosing dermopathy. Conclusion: CRF is associated with a complex array of cutaneous manifestations caused either by the disease or by the treatment. The early recognition of cutaneous signs can relieve suffering and decrease morbidity. Keywords: Chronic renal failure, Haemodialysis, Pruritus, Xerosis, Kyrles disease, Nephrogenic Fibrosing Dermopathy

Kyrle disease: report of a Tunisian case

Kyrle disease (KD) was first described in 1916 by Kyrle under the name of hyperkeratosis follicularis and parafollicularis in cutem penetrans [1]. As he described, this is a disorder characterized by the formation multiple hyperkeratotic follicular and parafollicular papular lesions with a central keratotic plug. It is considered as a variant of primary perforating dermatoses which share the same pathophysiology: a transepidermal elimination of dermal substances [2]. The prognosis depends essentially on the association with some systemic disorders such as diabetes mellitus, renal failure, liver disease, congestive cardiac failure hyperlipidemia, etc [2].

Kyrle's Disease Presenting in an Extensive Distribution along Lines of Blaschko.

A 24-year-old lady presented with the complaints of asymptomatic dark, raised lesions over her body since past 4 years. Dermatological examination revealed hyperkeratotic, hyperpigmented papules in a Blaschkoid distribution over trunk and extremities. The hyperkeratotic lesions could be removed leaving behind a small crater. Skin biopsy revealed hyperkeratosis, parakeratosis with epidermal invagination, focal cornified plug with basophilic degenerated material and dyskeratotic keratinocytes. Dermis showed dilated capillaries with moderate amount of perivascular lymphocytic infiltrate and granulomatous lymphocytic infiltrate in the deep dermis. A diagnosis of Kyrle's disease was made and patient was started on oral acitretin 25 mg daily with satisfactory response to therapy. This is the first reported instance of Kyrle's disease occurring in an extensive Blaschkoid distribution.

Kyrle’s Disease: A Rare Skin Manifestation of Systemic Diseases

A 39-year-old man of diabetic nephropathy presented with pruritic papulo-nodular brown lesions in both arms and legs on extensor surface characterized by central keratin plugs. Lesions were non-tender and numerous, crusted lesions which histologically were perforating disorders, showing features of Kyrle disease. The blood sugar of the patient was kept under control & hemodialysis was started. Dermatological lesions were treated by Isotretinoin, vitamin A, Tretinoin cream and oral antihistamines.DOI: http://dx.doi.org/10.3329/jom.v15i2.20689 J MEDICINE 2014; 15 : 144-145

Kyrle’s Disease: A Case Report

A case of Kyrle's disease in a known patient of Type 2 DM and CKD presented with left ventricular failure is reported. He had papulo-nodular brown lesions in both arms and legs on extensor surface characterized by central keratin plugs. Lesions were non-tender and numerous. Intense pruritus was present. He recovered with anti-failure regime and hemodialysis. Dermatological lesions were treated by Isotretinoin, vitamin A, Tretinoin cream and oral antihistamines. I. Case-report A 39 years old Hindu male was admitted with shortness of breath and cough for 2 days. On examination, B.P was 180/110 mm Hg. and bilateral coarse crackles, raised JVP and S3 were present. He was a known diabetic for the past 10 years , developed nephropathy for last 2 years and was undergoing maintenance hemodialysis for last 5 months. On general examination, he was found to have multiple brown papulo-nodular lesions with central keratin plug and silvery scales distributed over the anterior aspect of both thighs and legs (Fig-1) . There were also numerous papules of varying size on the extensor aspect of both elbows (Fig-2) . The lesions were non-tender and associated with severe pruritus. With treatment, he was relieved of LVF. Subsequently dialysis was done for CKD. Necessary investigations (FBS, PPBS, Blood urea, serum creatinine, serum sodium and potassium, USG abdomen) were done for type-2 D.M. and CKD .The dermatological lesions were diagnosed to be Kyrle's disease.

Kyrle 病

Kyrle 病

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.

Kyrle's Disease: Treatment with Minocycline and Topic Tacalcitol

Kyrle's disease is a rare perforative skin dermatosis. It was first described in 1916 by J. Kyrle, under the name of hyperkeratosis follicularis et parafollicular in cutem penetrans. It refers to a disorder of keratinization, which usually appears as scattered or grouped hyperkeratotic papules on the extremities and the trunk characterized by extrusion of keratotic material into an epidermal invagination.

Perforating disorders of the skin

Perforating disorders of the skin, is an often overlooked entity characterized by transepidermal elimination of material from the upper dermis and are classified histopathologically according to the type of epidermal disruption and the nature of the eliminated material. They include Kyrle's disease, perforating folliculitis, reactive perforating collagenosis, and elastosis perforans serpiginosa. The aim of this study was to delineate the clinical and histopathological features of perforating disorders of the skin. In our study, we reviewed last 2 years skin biopsies received by us. Hematoxylin and eosin sections were re-examined and histochemical stainings (elastic van Gieson and Masson trichrome stains) were also used for histopathological evaluation. We reviewed five cases of perforating disorders of skin which included two cases of Kyrle's disease, two cases of reactive perforating collagenosis and a single case of perforating folliculitis. Two patients had family history of perforating dermatosis in their siblings and three had associated systemic disease. Perforating disorders of the skin should be considered when ulcer with keratotic plugs is found.

Collagénose perforante réactionnelle

Reactive perforating collagenosis (RPC) belongs to the group of perforating dermatoses, which comprises elastosis perforans serpiginosa, RPC, perforating folliculitis and Kyrle's disease. RPC was initially described as a distinctive form of transepithelial elimination of altered collagen related to superficial trauma. Two types are distinguished: a hereditary type (MIM 216700), which is rare and begins during early childhood, and a second type, called acquired RPC, which is more frequent, appears in adults and is associated with other diseases, diabetes mellitus, renal insufficiency, solid tumors, lymphomas and AIDS. We report the case of a young man whose illness began during infancy, militating in favor of a diagnosis of a hereditary form of RPC. The description of similar lesions in the patient's brother confirmed our diagnosis. A 26-year-old man, the child of consanguinous parents, presented crusted papular lesions on his hands. The cutaneous lesions, located on the external side of the limbs, had been present since childhood, with flares during winter. Histologic analysis showed a cup-shaped depression in the epidermis containing keratinous material with extruded degenerated collagen towards the cutaneous surface. Treatment with topic retinoids did not result in any real resolution of the disease. The patient reported the presence of similar lesions in his brother, which was consistent with our diagnosis. The pathogenesis of hereditary RPC is still unknown, even if superficial trauma is suspected as the cause of RPC. In contrast, in diabetes, acquired RPC pathogenesis has recently been related to advanced glycation end-products of collagen.

Cutaneous Manifestations of Chronic Kidney Disease-An Observational Study in 100 Cases

Chronic Kidney Disease (CKD) presents with an array of cutaneous manifestations. Newer changes are being described since the advent of haemodialysis, which prolongs the life expectancy, giving time for these changes to manifest. This cross sectional study was performed in 100 cases of CKD admitted in nephrology department of Dhaka Medical College Hospital (DMCH) and Bangabandhu Sheikh Mujib Medical University (BSMMU) from April 2008 to August 2008 to evaluate the prevalence of dermatologic problems. Among them most belong to 2nd to 5th decade, 68 are male and rests are female. Glomerulonephritis (44%), Diabetes mellitus (22%), Obstructive uropathy (13%) and Hypertensive nephropathy (12%) are found common causes of CKD. Among these patients 38% patients were treated with conservative treatment, 31% with intermittent peritoneal dialysis (IPD), 19% with haemodialysis and 12% with some form of immunosuppressive therapy. Total 88% of study population had some form of skin disorder; pallor was the most common (82%), while xerosis (61%), pruritus (53%), pigmentation (37%) and bacterial infection (37%) were other common problems. Purpura and fungal infection was 29% and 27% respectively. Viral infection (9%), dermatitis (4%), gynaecomastia (1%), kyrle's disease (3%) are relatively less common findings. Lindsay's nail was seen in 23% of patients and was more prevalent in glomerulonephritis and diabetic patients with prevalence of 13% and 9% respectively. Other nail changes included koilonychia (4%), subungual hyperkeratosis (1%), splinter hemorrhages (3%) onychomycosis (8%) and Beau's lines (1%). So, CKD is associated with a complex array of cutaneous manifestations caused either by the disease or by treatment.DOI: http://dx.doi.org/10.3329/fmcj.v7i1.10296Faridpur Med. Coll. J. 2012;7(1): 33-36

A case of uremic pruritus and Kyrle disease: Effective treatment with amitriptyline

A case of uremic pruritus and Kyrle disease: Effective treatment with amitriptyline

A prospective study of cutaneous abnormalities in patients with chronic kidney disease

There are diverse ways in which the skin is affected by chronic kidney disease (CKD). Various specific and nonspecific skin abnormalities are observed in patients with CKD. The aim of the study was to document the prevalence of skin diseases that commonly occur in patients with CKD on medical treatment and dialysis. A total of 99 patients with CKD were examined for evidence of skin diseases. Ninety-six had at least one cutaneous abnormality attributable to CKD. The most prevalent finding was xerosis (66.7%), followed by pallor (45.45%), pruritus (43.4%), and cutaneous pigmentation (32.3%). Other cutaneous manifestations included dermatitis (27.27%); Kyrle's disease (17.17%); fungal (8.08%), bacterial (11.1%), and viral (5.05%) infections; purpura (10.1%); gynecomastia (4.04%); and yellow skin (5.05%). The common nail changes were half and half nails (36.36%) and onycholysis (13.13%). CKD is associated with various cutaneous abnormalities caused either by the disease or by treatment, the most common being xerosis and pruritus. The dermatologic complications can significantly impair the quality of life in certain individuals; therefore, earlier diagnosis and treatment is important to improve their quality of life.

A rare case study of a female patient suffering from Kyrle disease (hyperkeratosis follicularis etparafollicularis in cutem penetrans)

The article presents a literature review and case study of a rare form of dermatitis in a female patient aged 61 - Kyrle disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans). It also provides a detailed description of the clinical picture and differential diagnostics of the disease. The following typical changes were observed based on the results of histological examination: epidermic invaginations filled with corneous masses and areas of focal parakeratosis. Essential improvement in skin process dynamics was achieved against the background of treatment with Acitretin.

Kyrle’s disease successfully treated with allopurinol

Kyrle’s disease successfully treated with allopurinol

Challenging the schemes: acantholytic (Darier's disease‐like) Kyrle's disease

Challenging the schemes: acantholytic (Darier's disease‐like) Kyrle's disease

Rare presentation of Kyrle's disease in siblings.

Background:Kyrle's disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings.Materials and Methods:Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction.Results and Conclusions:A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle's disease. This helps to differentiate the rare primary Kyrle's disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.

Verrucous tuberculid mimicking Kyrle disease

Cutaneous tuberculosis shows wide clinical variation depending upon the virulence of the organism and host immunity. Tuberculids are difficult to diagnose since the organism often cannot be identified or isolated by microscopy, culture or polymerase chain reaction. The diagnosis rests primarily on the identification of a tuberculous focus elsewhere in the body and response to antitubercular therapy. We present the clinicopathological features of an unusual tuberculid in a 30-year-old man, remarkably mimicking Kyrle's disease. He presented with multiple, symmetrically distributed follicular and perifollicular verrucous papules, nodules and plaques on the face, earlobes and extremities since 4 years. Histopathology revealed parakeratotic follicular plug invaginating into the dermis with multiple caseating epithelioid cell granulomas. PCR for M. tuberculosis was negative. The identification of associated axillary tubercular lymphadenitis, strongly positive Mantoux reaction, tuberculoid granulomas on histopathology and complete resolution of the lesions with antitubercular therapy helped in making the diagnosis. We propose the term "verrucous tuberculid" for this entity.

Regression of skin lesions of Kyrle's disease with metronidazole in a diabetic patient

We describe here in a case of a 41-year-old man, with diabetes mellitus, who presented manifestations of Kyrle's disease. Administration of metronidazole, 500 mg twice daily for 1 month, resulted in complete regression of skin lesions with no recurrence during 12 months of follow-up. This successful antibiotic treatment is to support the role of infectious agents (anaerobic bacteria) in the pathogenesis of Kyrle's disease.