What is Acquired Perforating Dermatosis?
Perforating dermatoses are a group of skin conditions marked by a rash with bumps that eliminate parts of the skin. Traditionally, there are four main types of primary perforating dermatosis. Each one is characterized by how it eliminates skin components:
Kyrle disease (KD) eliminates abnormal skin cells, reactive perforating collagenosis (RPC) gets rid of collagen fibers, elastosis perforans serpiginosum (EPS) removes elastic fibers, and perforating folliculitis (PF) clears the contents of hair follicles, which can include collagen or elastic fibers. However, there is some debate about the exact classification of KD, with some experts considering it a specific type of perforating dermatosis, and others suggesting it might be a variant of another skin condition, known as prurigo nodularis.
There’s also a secondary form of this condition called acquired perforating dermatosis (APD). This term refers to the dermatoses affecting adults with certain health conditions like diabetes and kidney failure, regardless of what skin material is eliminated. APD usually manifests as skin lesions with a central depression, typically appearing on the trunk and extremities of the body.
The exact cause of APD remains unclear, but some experts believe that physical harm and impairment of the small blood vessels might trigger the elimination and break down of collagen fibers in the skin.
What Causes Acquired Perforating Dermatosis?
The exact cause of this condition is still not fully known, but it seems that some people may have a genetic tendency to develop it. Some of these cases are often found together with diabetes, chronic kidney disease, and liver disease.
Risk Factors and Frequency for Acquired Perforating Dermatosis
Acquired perforating dermatosis, or APD, is a skin condition that appears to affect men and women equally, and usually shows up in the fifth decade of life. We don’t yet know exactly how many people have this disease, but one study found an estimated rate of 2.53 cases for every 100,000 people per year. However, this number might not be entirely accurate, since some cases of APD might be missed or mistaken for other skin issues.
A type of APD called acquired reactive perforating collagenosis (ARPC) is the most common form, whereas KD and PF are less frequently seen. People with systemic diseases, especially chronic renal failure (CRF), diabetes (DM), or both, have often had APD. It’s even been suggested that the process of dialysis could play a big role in diabetic patients developing this condition.
- APD affects both men and women equally.
- It typically shows up when people are in their 50s.
- The estimated rate is 2.53 cases per 100,000 people a year, but this could be underestimating the real number.
- ARPC is the most common form of APD.
- People with chronic renal failure, diabetes, or both are often diagnosed with APD.
- Dialysis could be a key factor in diabetics developing APD.
While rarer, there are other diseases that may be linked with APD, including liver dysfunction, HIV infection, underactive thyroid gland (hypothyroidism), overactive parathyroid glands (hyperparathyroidism), eczema (atopic dermatitis), and various cancers such as liver cancer, pancreas cancer, Hodgkin’s disease and myelodysplastic syndrome.
Signs and Symptoms of Acquired Perforating Dermatosis
Acquired perforating dermatosis (APD) is a skin condition that can show up in many ways. For example, it can create tiny or larger bumps or nodules on the skin. These bumps could be hard or have dents in them (umibilicated). There might be red or non-red skin patches linked to hair follicles or not, varying with the type of APD.
Because itching is a frequent symptom, the person may scratch often, leading to scabs or scratches on the skin. They may also develop new skin issues due to a response called Koebnerization, which is when skin injuries lead to new instances of certain skin disorders.
The skin bumps usually appear on the outer parts of the lower legs and arms but can also occur on the body, scalp, or any place that gets scratched or rubbed due to itchiness.
Faver and his team listed the following as pointers for diagnosing Acquired Reactive Perforating Collagenosis (ARPC):
- The condition shows up after the age of 18.
- There are bumps or papules on the skin with an attached plug in the center.
- There is removal of dead collagen tissue within a skin-layered hole.
Testing for Acquired Perforating Dermatosis
To diagnose acquired perforating dermatosis, a doctor considers the patient’s medical history and the physical appearance of skin lesions. However, the most crucial step in identifying this skin condition is through a histopathology test, which is a detailed examination of the microscopic structure of tissues. The dermatoscope, a tool used to examine the skin, often shows bright white clouds and grey areas without any particular structure during this dermatosis. This visual feature can help differentiate it from similar conditions like prurigo nodularis, another type of skin disorder.
Despite these observations, multiple skin biopsies are usually required to confirm the diagnosis. Biopsies involve taking small samples of the affected skin for further microscopic examination.
Additionally, the doctor may recommend blood tests to measure glucose levels and check how well the liver and kidneys are functioning. These tests are crucial because they can help identify any underlying diseases connected to the skin condition.
Treatment Options for Acquired Perforating Dermatosis
No specific guidelines or established studies exist for treating acquired perforating dermatosis, a skin condition. The proposed treatments are based on previously published individual case reports or small collections of patient’s results.
The two main objectives of the treatment are to manage any underlying health issues and alleviate itching, which is often associated with the condition.
Initial treatment methods often involve systemic or topical corticosteroids, retinoids, and agents such as urea or salicylic acid that break down keratin, a skin protein. Moisturizers and oral antihistamines are also commonly prescribed to ease itching.
However, sometimes these treatments are not enough, and more options need to be explored. There are reports of successful results using tetracyclines, retinoids, phototherapy, and allopurinol. Tetracyclines have anti-inflammatory effects and are powerful suppressors of enzymes that break down skin structural proteins. Retinoids can help stabilize skin cells and safeguard collagen, which gives skin its strength and structure, from damage and cell death. Allopurinol may interfere with the process of collagen becoming sugary due to its antioxidant effects, which counteract harmful substances in the body.
Phototherapy, using specific types of UV light, has shown some success in managing itchiness and skin lesions. Finally, combining different treatments rather than using one treatment alone may be more effective.
What else can Acquired Perforating Dermatosis be?
When diagnosing skin conditions, doctors need to consider a range of possibilities. These could include:
- Folliculitis (inflammation of hair follicles)
- Prurigo nodularis (itchy lumps on the skin)
- Arthropod bites (bites from insects and spiders)
- Porokeratosis (a rare, inherited skin condition)
- Scabies (a skin infestation by mites)
- Keratosis pilaris (rough, bumpy skin typically on the arms and legs)
- Excoriated dermal diseases (skin conditions that can lead to broken skin, such as lichen planus and granuloma annulare)
- Multiple keratoacanthomata (a type of skin growth)
- Hyperkeratosis lenticularis perstans (a rare type of skin lesion)
- Perforating pseudoxanthoma elasticum (a rare genetic disorder affecting the skin, eyes, and cardiovascular system)
Having a wide range of possibilities means that doctors need to carry out thorough investigations to accurately diagnose a patient’s skin condition.
What to expect with Acquired Perforating Dermatosis
After treatment, skin lesions might completely disappear, leaving behind sunken scars or areas of darker skin. There are only a few recorded cases of these skin lesions clearing up on their own.
Possible Complications When Diagnosed with Acquired Perforating Dermatosis
The most frequent issue that arises with Acquired Perforating Dermatitis (APD) is that the lesions often get infected.
Preventing Acquired Perforating Dermatosis
Preventing a disease with many causes can be challenging to pinpoint specific strategies. Nonetheless, the disease can considerably affect the patients’ quality of life and emotional well-being. It is essential for patients to feel reassured and well-informed about their condition. This not only promotes a positive mindset, but also fosters proper adherence to treatment plans, which can lead to more favorable results.
