Dermatosis Perforante Adquirida (Enfermedad de Kyrle) / Acquired perforating dermatosis (Kyrle disease)

RESUMEN La enfermedad de Kyrle se define como una enfermedad cutánea perforante, de etiología aún no conocida, infrecuente, con tendencia a la cronicidad, caracterizada por un trastorno de la queratinización con eliminación transepidérmicade componentes de la matriz extracelular, se la asocia con enfermedades sistémicas principalmente la diabetes mellitus y la insuficiencia renal crónica en hemodiálisis entre otras, aunque también se puede manifestar en personas sin antecedentes patológicos previos. Presentamos el caso de una paciente de 42años condiagnóstico de enfermedad de Kyrle,sin otra patología asociada hasta el momento de la evaluación, realizamos además, una revisión bibliográfica sobre el tema.

ABSTRACT Kyrle's disease is defined as a perforating skin disease, of an unknown etiology, infrequent, with a tendency to chronicity, characterized by a disorder of transepidermal keratinization, and elimination of components of the extracellular matrix, it is associated with mainly systemic diseases decompensated diabetes mellitus and chronic renal failure on hemodialysis among others, although it can also manifest itself in those without any previous pathology. We present the case of a 42-year-old female patient diagnosed with Kyrle's disease and without any associated pathology.

Dermatosis Perforante Adquirida / Acquired perforating dermatosis

RESUMEN Los trastornos perforantes constituyen un grupo de enfermedades caracterizadas por la extrusión transepidérmica de material dérmico. La etiopatogenia es desconocida, sin embargo, la forma adquirida generalmente se presenta asociada a enfermedades sistémicas como insuficiencia renal y diabetes mellitus, entre otras. El prurito es el síntoma cardinal de esta patología, y clínicamente presenta pápulo-nódulos centrados por costras queratósicas. En cuanto al tratamiento, la dermatosis perforante adquirida (DPA) es de difícil control, con tendencia a la cronicidad. Presentamos el caso de una paciente de género femenino de 57 años de edad, con antecedente de diabetes,hipertensión arterial (HTA), hipotiroidismo e insuficiencia renal, con prurito generalizado de varios meses de evolución.

ABSTRACT Perforating disorders are a group of diseases characterized by transepidermal extrusion of dermal material, it includes reactive perforating collagenosis, perforating folliculitis, elastosis perforansserpiginosa and Kyrle's disease, and in recent years the term acquired perforating dermatosis (APD) has been used to refer to the perforating dermatoses that occur in adult patients associated with systemic disorder (kidney failure, diabetes mellitus, hypothyroidism, among others). The etiopathogenesis is still controversial and it is believed that several factors participate in the process, such as traumatism produced by chronic pruritus in predisposed patients, diabetic microangiopathy may contribute to collagen damage and to the microdeposition of substances that are no removed by dialysis, causing local inflamatory reaction. The itching constitudes the main symptom of this pathology, and clinically presents a papulo-nodules centered by keratosis crusts. Differential diagnoses include: nodular prurigo, multiple keratoacanthomas, sarcoid, and other perforating dermatosis. Treatment is difficult, with great tendency to chronicity. We report the case of a 57 year-old female patient, with history of type 2 diabetes, hypothyroidism and renal failure, with itching of several months of evolution.

Giant variant of acquired perforating dermatosis, clinically masquerading as a sarcoma: A report of a rare case

Acquired perforating dermatosis is a group of disease characterized by transepidermal elimination of altered dermal constituents of unknown pathogenesis. The giant variant was first described in 2006, as an emerging entity with seven reported cases to date. Here is an 83-year-old male presented with a 4-year history of gradually enlarging soft tisssue mass with ulcerartions at the left knee joint. Imaging revealed an extra-articular, single, heterogeneous, multinodular mass, suspicious for a soft tissue sarcoma. Wide local excision of the mass showed fleshy, hemorrhagic nodules communicating with epidermal ulcers. Microscopy showed cystic spaces straddling dermis and subcutis, containing eosinophilic, amorphous, granular material extruding through epidermal craters, surrounded by exuberant myofibroblastic proliferation. Trichrome and van-Gieson stains confirmed that the extruded material is collagen and the histology was compatible with the giant variant of acquired perforating collagenosis. Awareness of histological appearance prevents misdiagnosis and overtreatment of this entity, masquerading as a sarcoma clinically.

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.

A Clinicopathologic Study of Thirty Cases of Acquired Perforating Dermatosis in Korea

BACKGROUND: Acquired 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. OBJECTIVE: The 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. METHODS: We retrospectively reviewed the medical records and biopsy specimens of 30 patients who were diagnosed with APD. RESULTS: The 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%). CONCLUSION: In 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.

Crusted Scabies Misdiagnosed as a Cutaneous Side Reaction due to Allopurinol in a Patient with Acquired Perforating Dermatosis / 대한피부과학회지

Crusted (Norwegian) scabies is a clinical variant of human infestation with Sarcoptes scabiei, and is characterized by extensive, heavily crusted skin lesions. It usually affects immunocompromised patients. The diagnosis is commonly missed, and can lead to mismanagement. Here we report a case of crusted scabies misdiagnosed as a cutaneous side reaction due to allopurinol in a 48 year old male patient with acquired perforating dermatosis who had been treated with allopurinol for about 20 weeks. The lesion showed pruritic, excessive hyperkeratotic scaly crusts and fissures on the hands, and on pelvic, genital and inguinal areas. His skin lesions disappeared after treatment with 10% crotamiton cream for 2 weeks.

Treatment of Acquired Perforating Dermatosis in Chronic Renal Failure with Allopurinol / 대한피부과학회지

The perforating disorder is characterized by transepidermal elimination of altered dermal substances, which comprises Kyrle's disease, perforating folliculitis, elastosis perforans serpiginosa, and reactive perforating collagenosis. The term 'acquired perforating dermatosis' has been suggested for cases associated with renal disease and/or diabetes mellitus. Recently, resolution of lesions in acquired reactive perforating collagenosis by allopurinol has been reported. We, hereby, report a patient with acquired perforating dermatosis associated with chronic renal failure due to diabetic nephropathy, who was treated with allopurinol. The histopathological findings were compatible with Kyrle's disease.

Acquired Perforating Dermatosis in Patients with Chronic Renal Failure and Diabetes Mellitus

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. Twelves patients with APD associated with CRF and DM were enrolled in the study. In six patients who required hemodialysis, the lesions appeared 2-5 yr (mean 3 yr) after the initiation of dialysis, 18-22 yr (mean 19.3 yr) after the occurrence of DM. The other patients who did not receive hemodialysis noted the lesions 4-17 yr (mean 9.5 yr) after the onset of DM. All patients had an eruption of generally pruritic keratotic papules and nodules, primarily on the extensor surface of the extremities and the trunk. The histologic features of our cases showed a crateriform invagination of the epidermis filled by a parakeratotic plug and basophilic cellular debris. The period of treatment for patients who suffered from severe (7 cases) or very severe (3 cases) on the pruritus intensity was longer than that of patients who had mild pruritus (2 cases). These data showed that scratching appear to play a critical part in the pathogenesis of APD.

Acquired Perforating Dermatosis in Renal Failure / 대한피부과학회지

BACKGROUND: Acquired perforating dermatosis (APD) is a skin disorder occurring in patients with diabetes mellitus and/or chronic renal failure requiring dialysis. OBJECTIVE: The purpose of this study was to clarify the clinical and histhopathological features of APD. METHOD: Twelve patients with APD who had biopsies done at Asan Medical Center were retrieved for this study. Clinical data and histopathologic specimens were reviewed. RESULTS AND CONCLUSION: 1. Sex ratio was 3:1 (9 male: 3 female) and mean age of onset was 52.1 years. 2. All patients had pruritic umbilicated papules with central keratotic plugs. The most predilection site was trunk. 3. Nine patients had an associated underlying non-insulin dependent diabetes mellitus 4. Skin lesions had developed on pre-dialysis in three patients, and after mean duration of 3.3 years on dialysis in remaining nine. 5. Histopathological examinations revealed the features of perforating folliculitis, Kyrle's disease or reactive perforating collagenosis. But we did not find any specimen resembling elastosis perforans serpiginosa. 6. It makes sense to designate the diseases as acquired perforating dermatosis rather than as one of the separate perforating diseases.