Kyrle disease

Last updated
Kyrle disease
Other namesHyperkeratosis follicularis et parafollicularis in cutem penetrans
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg

Kyrle disease is identified as a form of an acquired perforating disease. Other major perforating diseases are elastosis perforans serpiginosa and reactive perforating collagenosis. Recently, however, there is a controversy on categorizing Kyrle disease with perforating dermatosis or a subtype of acquired perforating collagenosis. [1]

Contents

Kyrle disease was first described by Josef Kyrle in 1916 when a diabetic woman presented generalized hyperkeratotic nodules. [2] The disease is distinguished by large papules with central keratin plug on the skin, usually on the legs of the patient and is often in conjunction with liver, kidney or diabetic disorders. It can affect both females and males with a 6:1 ratio. The papules usually show up on the patient with an average age of 30 years. [1] [3] Kyrle disease is a rare disease unless there is a high count of patients with chronic kidney failure. The disease seems to be more prevalent in African Americans, which can be correlated to the high incidence of diabetes mellitus and kidney failure in the population. [4]

Signs and symptoms

Kyrle disease symptoms are chronic and have an onset during adulthood between the ages of 30 and 50 years of age. However, there were reported cases of early onset as early as 5 years of age and late onset as late as 75 years of age. The main symptom is the development of small papules into painless lesions that are surrounded by silvery scales. The lesions are painless, however, there is a chance that the patient may experience extreme urges to itch them. In time, these lesions grow up to a radius of 0.75 inch and develop into red-brown nodules with a central plug of keratin. As more lesions develop, they can come together and form larger keratotic plaques. These lesions are usually observed on the lower extremities, however, can also develop on the upper extremities, such as, the arms, the head and the neck. The only parts of the body that Kyrle disease do not form are the palms, soles, and mucous membranes. Lesions may heal spontaneously without treatment, however, new ones will develop in its place. [1]

Other symptoms that may be observed: [5]

Causes

The causes of Kyrle disease are unclear and can be idiopathic. The only correlation that has shown light is the frequent association with an underlying disorder, such as, diabetes mellitus, chronic kidney disease, hyperlipoproteinemia, liver abnormalities, and congestive heart failure. However, there had been cases where Kyrle disease was seen without any conjunction with the previous mentioned disorders. [3] Due to the causes of Kyrle disease is unknown, the best way to prevent the disease is to prevent the disorders that are usually reported in conjunction with it.

Mechanism

The pathophysiology of Kyrle disease is unclear. Some scientists believe that it may be a variation of prurigo nodularis. The theory that most scientists agree upon is that Kyrle disease is an elimination of keratin and other cellular material across the epidermis. Keratinization in Kyrle disease form at the basilar layer that is lower than the normal proliferation region in the epidermis. This causes an inflammatory response which results with the keratin, along with other cellular material and connective tissue, to be forced out the epidermis. [6] [7] [8] Another reason for an inflammatory response may be due to an alteration of the dermal connective tissue. This is theorized because this step is a main reason for inflammatory responses in other skin diseases, such as, elastosis perforans serpiginosa and perforating collagenosis. [9]

Diagnosis

Since many other skin disorders can be characterized by abnormal papules or nodules, a dermatologist will determine if a patient has Kyrle disease by the depth of penetrating keratotic plugs, localized distribution of the plugs, size of plugs, and the age of onset. A physician will also have to test for disorders, such as, diabetes, hepatic, and renal disease to help bolster the diagnosis of Kyrle disease. [1] Other underlying diseases that Kyrle disease is observed with are tuberculosis, pulmonary aspergillosis, scabies, atopic dermatitis, AIDS, neurodermatitis, and endocrinological disorders. [10]

The inheritance of Kyrle disease is unknown as reported cases point to both autosomal dominance and autosomal recessiveness. [3]

Treatment

The best treatment for Kyrle's disease is to treat the underlying disease if present as life expectancy is also determined by the underlying disease. However, if there are no other diseases associated with Kyrle disease, treatment of the lesions is the course of action. There is a chance of the lesions healing without treatment but new ones will develop.

Medical care

Isotretinoin, high doses of vitamin A and tretinoin cream can be utilized. [1] Also, emollients, oral antihistamines, and antipruritic creams that contain menthol and camphor may be helpful because the lesions can become very itchy. [4]

Radiation therapy

UV irradiation can be utilized after curetting the hyperkeratosis with a combination medication treatment of oral retinoids, psoralen and Ultraviolet A radiation. [1]

Surgical care

Surgical options are considered the final option for treating Kyrle disease. The use of a carbon dioxide laser, electrocautery, or cryosurgery to rid of limited lesions can be implemented. Patients with darker skin must take extra precaution as these options can lead to dyspigmentation. In addition, performing on patients that had Kyrle disease due to diabetes mellitus or have poor circulation can lead to poor healing. [4]

Prognosis

Morbidity and mortality range from both extremes as the significance correlate with the underlying systemic disease. [4]

Recent research

There seems to be beneficial responses to clindamycin therapy as the lesions regress. This leads to the hypothesis that microorganisms may be playing a role in the initial stages of Kyrle disease. [2]

A family with Kyrle disease were examined which their skin lesions were benign. However, when three of the young adult members were closely examined, they had posterior subcapsular cataracts and two of those three developed multiple tiny yellow-brown anterior stromal corneal opacities. In order to determine if there is any correlation between Kyrle disease and the ocular observations, more cases of Kyrle disease are to be analyzed. [11]

All in all, since Kyrle disease is relatively rare, more cases need to be studied and analyzed in order to understand the underlying pathogenesis and to improve the management of the disease.

Related Research Articles

Epidermolytic hyperkeratosis Medical condition

Epidermolytic ichthyosis (EI), also known as bullous epidermis ichthyosis (BEI), epidermolytic hyperkeratosis (EHK), bullous congenital ichthyosiform erythroderma (BCIE), bullous ichthyosiform erythroderma or bullous congenital ichthyosiform erythroderma Brocq, is a rare and severe form of ichthyosis this skin disease affects around 1 in 300,000 people.

Skin condition Any medical condition that affects the integumentary system

A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment.

Boil Medical condition

A boil, also called a furuncle, is a deep folliculitis, infection of the hair follicle. It is most commonly caused by infection by the bacterium Staphylococcus aureus, resulting in a painful swollen area on the skin caused by an accumulation of pus and dead tissue. Boils which are expanded are basically pus-filled nodules. Individual boils clustered together are called carbuncles. Most human infections are caused by coagulase-positive S. aureus strains, notable for the bacteria's ability to produce coagulase, an enzyme that can clot blood. Almost any organ system can be infected by S. aureus.

Seborrheic keratosis Medical condition

A seborrheic keratosis is a non-cancerous (benign) skin tumour that originates from cells in the outer layer of the skin. Like liver spots, seborrheic keratoses are seen more often as people age.

Hyperkeratosis Medical condition

Hyperkeratosis is thickening of the stratum corneum, often associated with the presence of an abnormal quantity of keratin, and also usually accompanied by an increase in the granular layer. As the corneum layer normally varies greatly in thickness in different sites, some experience is needed to assess minor degrees of hyperkeratosis.

Febrile neutrophilic dermatosis Medical condition

Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.

Degos disease Medical condition

Degos disease, also known as Köhlmeier-Degos disease or malignant atrophic papulosis, is an extremely rare condition caused by blockage of arteries and veins. Individuals with this condition will develop papules. Those diagnosed with this disease may also develop complications due to impairment of internal organs. The exact underlying mechanism is still unknown, and an effective treatment is still being developed. There are fewer than 50 living patients presently known worldwide, and fewer than 200 reported in medical literature. However, many individuals may go undiagnosed due to rarity of the disease. Most individuals develop symptoms between the ages of 20–50; however, cases outside of this age range have been reported as well.

Granuloma annulare Medical condition

Granuloma annulare (GA) is a common, sometimes chronic skin condition which presents as reddish bumps on the skin arranged in a circle or ring. It can initially occur at any age, though two-thirds of patients are under 30 years old, and it is seen most often in children and young adults. Females are two times as likely to have it than males.

Zunich–Kaye syndrome Medical condition

Zunich–Kaye syndrome, also known as Zunich neuroectodermal syndrome, is a rare congenital ichthyosis first described in 1983. It is also referred to as CHIME syndrome, after its main symptoms. It is a congenital syndrome with only a few cases studied and published.

Josef Kyrle was an Austrian pathologist and dermatologist who was a native of Schärding.

Paraneoplastic pemphigus (PNP) is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes.

Acquired perforating dermatosis is clinically and histopathologically similar to perforating folliculitis, also associated with chronic kidney failure, with or without hemodialysis or peritoneal dialysis, and/or diabetes mellitus, but not identical to Kyrle disease.

Reactive perforating collagenosis is a rare, familial, nonpuritic skin disorder characterized by papules that grow in a diameter of 4 to 6mm and develop a central area of umbilication to which keratinous material is lodged. The cause of reactive perforating collagenosis is unknown.

Dermatosis neglecta Medical condition

Dermatosis neglecta is a skin condition in which accumulation of sebum, keratin, sweat, dirt and debris leads to a localized patch of skin discoloration or a wart-like plaque. It is caused by inadequate hygiene of a certain body part, usually due to some form of disability or a condition that is associated with pain or increased sensitivity to touch (hyperesthesia) or immobility.

An arsenical keratosis is a growth of keratin on the skin caused by arsenic, which occurs naturally in the earth's crust and is widely distributed in the environment, Arsenical compounds are used in industrial, agricultural, and medicinal substances. Arsenic is also found to be an environmental contaminant in drinking water and an occupational hazard for miners and glass workers. Arsenic may also causes other conditions including: Bowen's disease, cardiovascular diseases, developmental abnormalities, neurologic and neurobehavioral disorders, diabetes, hearing loss, hematologic disorders, and various types of cancer. Arsenical keratoses may persist indefinitely, and some may develop into invasive squamous cell carcinoma. Metastatic arsenic squamous cell carcinoma and arsenic-induced malignancies in internal organs such as the bladder, kidney, skin, liver, and colon, may result in death.

Warty dyskeratoma

Warty dyskeratoma, also known as an Isolated dyskeratosis follicularis, is a benign epidermal proliferation with distinctive histologic findings that may mimic invasive squamous cell carcinoma and commonly manifests as an umbilicated lesion with a keratotic plug, WD have some histopathologic similarities to viral warts but it's not caused by HPV and the majority of these lesions display overall histopathologic features consistent with a follicular adnexal neoplasm. Usually limited to the head, neck, scalp or face and vulva. Lesions are generally solitary and sporadic and may be associated with a follicular unit. Oral involvement, particularly the hard palate, and genital involvement have been reported. it can also be thought of as one of the manifestations of focal acantholytic dyskeratosis, an epidermal reaction pattern that can be seen in several disorders, including Darier's disease and Grover's disease. But the main Difference between Darier disease and Warty dyskeratoma, is that Darier disease inherited dermatosis consisting of multiple keratotic papules on the face, trunk, and extremities, while WD occurs as an isolated, noninherited, single keratotic nodule mainly confined to the head and neck as mentioned earlier.

Porokeratosis Medical condition

Porokeratosis is a specific disorder of keratinization that is characterized histologically by the presence of a cornoid lamella, a thin column of closely stacked, parakeratotic cells extending through the stratum corneum with a thin or absent granular layer.

Inflammatory linear verrucous epidermal nevus Medical condition

Inflammatory Linear Verrucous Epidermal Nevus is a rare disease of the skin that presents as multiple, discrete, red papules that tend to coalesce into linear plaques that follow the Lines of Blaschko. The plaques can be slightly warty (psoriaform) or scaly (eczema-like). ILVEN is caused by somatic mutations that result in genetic mosaicism. There is no cure, but different medical treatments can alleviate the symptoms.

Fungal folliculitis Inflammation of hair follicles due to fungal infection

Majocchi's granuloma is a skin condition characterized by deep, pustular plaques, and is a form of tinea corporis. It is a localized form of fungal folliculitis. Lesions often have a pink and scaly central component with pustules or folliculocentric papules at the periphery. The name comes from Professor Domenico Majocchi, who discovered the disorder in 1883. Majocchi was a professor of dermatology at the University of Parma and later the University of Bologna. The most common dermatophyte is called Trichophyton rubrum.

References

  1. 1 2 3 4 5 6 "Kyrle disease. DermNet NZ". dermnetnz.org. Retrieved 2015-12-11.
  2. 1 2 Akçalı C, Baba M, Seçkin D, Kayaselçuk F, Güleç T (2007). "Kyrle's Disease: A Case Report". Journal of the Turkish Academy of Dermatology.
  3. 1 2 3 Bhambri S, Del Rosso JQ, Mobini N, Janda P (2008). "Kyrle's Disease". Cosmetic Dermatology.
  4. 1 2 3 4 "Kyrle Disease: Background, Pathophysiology, Epidemiology". 2018-08-14.{{cite journal}}: Cite journal requires |journal= (help)
  5. ↑ "Symptoms of Kyrle disease - RightDiagnosis.com". www.rightdiagnosis.com. Retrieved 2015-12-11.
  6. ↑ Carter VH, Constantine VS (June 1968). "Kyrle's disease. I. Clinical findings in five cases and review of literature". Arch Dermatol. 97 (6): 624–32. doi:10.1001/archderm.1968.01610120014002. ISSN   0003-987X. PMID   5652970.
  7. ↑ Constantine VS, Carter VH (June 1968). "Kyrle's disease. II. Histopathologic findings in five cases and review of the literature". Arch Dermatol. 97 (6): 633–9. doi:10.1001/archderm.1968.01610120023003. PMID   4172447.
  8. ↑ Rapini RP, Herbert AA, Drucker CR (August 1989). "Acquired perforating dermatosis. Evidence for combined transepidermal elimination of both collagen and elastic fibers". Arch Dermatol. 125 (8): 1074–8. doi:10.1001/archderm.1989.01670200050007. PMID   2757403.
  9. ↑ "Kyrle Disease: Background, Pathophysiology, Epidemiology". 2018-08-14.{{cite journal}}: Cite journal requires |journal= (help)
  10. ↑ Saray, Y.; Seçkin, D.; Bilezikçi, B. (2006-07-01). "Acquired perforating dermatosis: clinicopathological features in twenty-two cases". Journal of the European Academy of Dermatology and Venereology. 20 (6): 679–688. doi:10.1111/j.1468-3083.2006.01571.x. ISSN   0926-9959. PMID   16836495. S2CID   19794920.
  11. ↑ Tessler HH, Apple DJ, Goldberg MF (October 1973). "Ocular findings in a kindred with Kyrle disease. Hyperkeratosis follicularis et parafollicularis in cutem penetrans". Arch. Ophthalmol. 90 (4): 278–80. doi:10.1001/archopht.1973.01000050280005. ISSN   0003-9950. PMID   4746641.
This page is based on this Wikipedia article
Text is available under the CC BY-SA 4.0 license; additional terms may apply.
Images, videos and audio are available under their respective licenses.