What causes Kyrle disease?
The cause of the disease is currently unknown; however, it is often associated with certain conditions such as diabetes mellitus , kidney disease , liver abnormalities, and congestive heart failure. In some families, the condition appears to be inherited but an underlying genetic cause has not been identified.
What is Kyrle disease?
First described by Kyrle in 1916, Kyrle disease is a perforating skin condition characterized by the presence of large keratotic papules distributed widely throughout the body. The papules contain a central keratotic plug, which histologically correlates with keratin and necrotic debris.
What is acquired keratosis Follicularis?
Keratosis follicularis, also known as Darier disease, is a rare, genetic skin disorder. Affected individuals develop skin lesions that consist of thickened, rough bumps (papules) or plaques that may also be greasy or have a brown or yellow crust.
What is acquired perforating dermatosis?
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.
What is perforating folliculitis?
In perforating folliculitis, keratotic follicular papules develop, particularly over extensor surfaces. Microscopically, the disorder is characterized by disruption of the infundibular portion of the follicular wall, with transepidermal (transfollicular) elimination of connective-tissue elements and cellular debris.
Is perforating collagenosis curable?
In cases of acquired reactive perforating collagenosis, García-Malinis et al recommend discontinuing treatment with biologics. A 23-year-old patient with acquired reactive perforating collagenosis was successfully treated with an 8-week course of itraconazole, even though there was no fungal infection present.
How do you treat perforating collagenosis?
The main aim of treatment for reactive perforating collagenosis is to reduce itch and minimise skin trauma:
- Topical — emollients, topical steroids, topical retinoids.
- Systemic — antihistamines, oral retinoids, allopurinol.
- Other — phototherapy.
How is keratosis follicularis treated?
Retinoids taken by mouth (orally) have been effective in treating individuals with keratosis follicularis and are the drugs most often used to treat severe cases. Oral retinoids such as tretinoin and acitretin affect the entire body (systemic therapy). Oral retinoids can be associated with side effects.
What is Kyrle disease of the skin?
Summary Summary. Kyrle disease is rare condition that affects the skin. People with this condition generally develop large papules with central keratin (a protein found in the skin, hair and nails) plugs throughout their body. These lesions are typically not painful but may cause severe itching.
What are the symptoms of Kyrle disease?
Symptoms Symptoms. Kyrle disease is primarily characterized by large skin papules with central keratin (a protein found in the skin, hair and nails) plugs.
What is the pathophysiology of keratotic Kyrle disease?
Kyrle disease is characterized by transdermal elimination of keratotic material with no collagen or elastic fibers. This differentiates Kyrle disease from other acquired perforating disorders that exhibit transdermal elimination of collagen and elastic fibers.
What is a Kyrle papule?
Kyrle disease is characterised by the formation of large papules with central keratin plugs and is often associated with hepatic, renal or diabetic disorders. It can affect both men and women throughout life, although the average age at the time of presentation is 30 years.