What is a perforating disorder?
Perforating disorders are characterized by transepidermal elimination of altered keratin or dermal connective tissue material. These disorders include perforating folliculitis (as shown below), Kyrle disease, elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis.
What is perforating dermatosis?
Perforating dermatoses are a group of conditions characterized by transepidermal elimination of dermal material (collagen, elastic tissue or necrotic connective tissue).
What causes perforating collagenosis?
The underlying cause of reactive perforating collagenosis is unknown, but an abnormal response to superficial trauma (eg, scratching) may be involved. Papules have been reported following scratches, acne spots, insect bites, and scabies.
What is Kyrle’s disease?
Kyrle’s disease (KD) is an acquired perforating dermatosis associated with an underlying disorder such as diabetes mellitus or chronic renal failure. It presents as multiple discrete, eruptive papules with a central crust or plug, often on the lower extremities.
How do you treat perforating folliculitis?
Tretinoin 0.1% cream has cleared some perforating folliculitis lesions but has not prevented the development of others. A case of successful treatment using 13-cis -retinoic acid has been reported. A 2004 study of 5 perforating folliculitis patients showed good clinical responses with narrowband UV-B therapy.
Is perforating collagenosis curable?
Familial reactive perforating collagenosis is a lifelong condition with lesions becoming larger and more numerous with age. Individual lesions in both the familial and acquired forms are self-healing but often recur.
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.
What is Elastosis Perforans Serpiginosa?
Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. This condition classically presents as small papules arranged in serpiginous or annular patterns on the neck, face, arms, or other flexural areas.
What does Calciphylaxis mean?
Calciphylaxis (kal-sih-fuh-LAK-sis) is a serious, uncommon disease in which calcium accumulates in small blood vessels of the fat and skin tissues. Calciphylaxis causes blood clots, painful skin ulcers and may cause serious infections that can lead to death.
What causes perforating folliculitis?
Perforating folliculitis is associated with renal failure and diabetes mellitus. It has also been reported in the setting of HIV infection, in two dialysis patients with markedly elevated serum silicon levels, in a patient with antisynthetase syndrome, and in a case of cystic fibrosis.
What is dermal Elastosis?
Elastosis refers to degenerative changes in the dermal tissue with increased deposition of elastin material. Elastosis is a prominent feature of several skin conditions. They are distinguished by clinical features and histopathological features seen on skin biopsy.
What does calciphylaxis look like?
Signs and symptoms of calciphylaxis include: Large purple net-like patterns on skin. Deep, very painful lumps that ulcerate creating open sores with black-brown crust that fails to heal — typically in skin areas with high fat content, such as the stomach and thigh, although they can occur anywhere.
Is calciphylaxis always fatal?
What is the outlook? Calciphylaxis is often a fatal condition. According to a study published by the American Journal of Kidney Diseases , people with Calciphylaxis have a one-year survival rate of less than 46 percent. Death is usually a result of complications, such as infections and sepsis.
What does Kyrle disease look like?
Disease at a Glance Kyrle disease is rare condition that affects the skin. The main symptoms involves the developing 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 is Favre Racouchot syndrome?
Favre-Racouchot syndrome is a disorder consisting of multiple open and closed comedones in the presence of actinically damaged skin. The disease was originally described in 1932 by Favre and reviewed in detail by Favre and Racouchot in 1951.
What do calcium deposits under the skin look like?
Calcinosis cutis is the deposit of calcium under the skin. This can happen anywhere on the body. One rare form of it can occur on the face or upper body after a case of acne. The deposits usually show up as whitish bumps on the skin’s surface.
Can Covid cause calciphylaxis?
New research throughout the COVID-19 pandemic has shown an increased inflammatory and coagulopathic complication of COVID-19. We present a case of a patient with nonuremic calciphylaxis following treatment for severe COVID-19 and no known cause of hypercoagulability.
What causes Kyrle disease?
The cause of the disease is currently unknown. However, Kyrle disease 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 are the different types of perforating dermatosis?
Currently, two major perforating diseases include reactive perforating collagenosis and elastosis perforans serpiginosa. In some literature, acquired perforating dermatosis is a third major group and is a catch-all term for cases of perforating disease arising in adults, usually associated with a systemic disease.
What is reactive perforating collagenosis?
Reactive perforating collagenosis is the most common type of primary perforating dermatosis. It is characterised by the transepithelial elimination of collagen from the dermis through the epidermis to the skin surface. Who gets reactive perforating collagenosis?
What is acquired perforating dermatosis (APD)?
In some literature, acquired perforating dermatosis is a third major group and is a catch-all term for cases of perforating disease arising in adults, usually associated with a systemic disease.
Is reactive perforating dermatosis inherited or acquired?
While used for both the inherited and acquired forms, the name reactive perforating dermatosis is also used for the more common acquired type. This reflects that there is overlap with the other acquired perforating disorders of perforating folliculitis and Kyrle disease. Both conditions fall within the group of perforating dermatoses.