What is Kyrle Disease?
Kyrle disease is a rare condition that affects the skin and is considered as a type of acquired perforating dermatosis, a term used to describe skin conditions where the skin’s top layer pushes through lower layers. Other conditions in this category include reactive perforating collagenosis, elastosis perforans serpinginosa, and perforating folliculitis. It was first identified in 1916 by Josef Kyrle, an Austrian pathologist and skin doctor, in a woman with diabetes who had widespread hard, raised skin bumps. The original name used by Kyrle for this condition was “hyperkeratosis follicularis et parafollicularis in cutem penetrams”. The term acquired perforating dermatosis is now often used as a blanket term for all skin disorders in adults that show these characteristics, due to considerable similarities in symptoms, follicle-related and non-follicle-related appearances, and variations in skin tissue examination at different stages of disease.
However, there’s still debate about how Kyrle disease should be classified. Some experts use the terms Kyrle disease and acquired perforating dermatosis interchangeably, while others describe it as a type of prurigo nodularis, a skin condition characterized by itchy bumps, and see it as an end-stage of a skin disorder known as excoriated folliculitis.
Kyrle disease usually appears as several distinct raised skin eruptions with a hard core in the middle on the lower body parts. In skin tissue examinations, there’s a distinct pushing out of abnormal skin cells. The exact causes of Kyrle disease are unknown, but most cases seem to occur as a result of long-lasting systemic diseases, most commonly stages 4 and 5 of kidney failure and diabetes mellitus. Some small case studies have suggested a genetic link, implying Kyrle disease could sometimes be a primary skin disorder. Treatment can be challenging and mainly focuses on managing any underlying health issues. Agents that cause the outer layer of skin to peel off are usually recommended as the first step in treatment.
What Causes Kyrle Disease?
The exact cause of Kyrle disease, a skin disorder, remains unknown. Various theories have been suggested, including infection, abnormal skin cell formation, defects in the creation of skin and the layer below the skin, high levels of a protein called fibronectin in the body, kidney failure, and high blood sugar levels from conditions like diabetes. The frequent occurrence of Kyrle disease alongside other systemic illnesses suggests that it might be linked to an underlying health disorder.
Some small case studies suggest that there could be a genetic link to Kyrle disease, with cases found in both dominant and recessive inheritance patterns – which means the disease could be passed down from parents to their children. However, the familial predisposition hasn’t been thoroughly examined. So, more research is needed to understand the significance of genetics in this disease.
Risk Factors and Frequency for Kyrle Disease
Kyrle disease often appears in adulthood, typically between the ages of 30 and 50, with women being more likely to get it than men, at a ratio of 6:1.
- Kyrle disease is usually linked with long-term kidney disease and diabetes, especially when diabetes has led to kidney disease. About 10% of patients undergoing hemodialysis (kidney treatment) could also get Kyrle disease.
- However, it has also been found, although rarely, in people with tuberculosis, lung fungal infection, itch mites, a type of skin inflammation (atopic dermatitis), AIDS, a type of skin condition (neurodermatitis), cancer, liver problems, heart failure, and hormone-related disorders.
Signs and Symptoms of Kyrle Disease
Kyrle disease is a skin condition that usually shows up as raised, hard bumps and nodules with a hard center, much like a plug. These skin lesions are most often found on the lower parts of the body, particularly the calf, the front part of the lower leg, and the back. However, they can also appear on the arms and in the head and neck areas. In rare cases, the disease can affect the palms of the hands and soles of the feet. Plus, there might be instances of “Koebnerization,” a phenomenon where skin lesions form at sites of injury.
Testing for Kyrle Disease
Kyrle disease is identified through specific symptoms and a detailed analysis of skin tissues. A hardened area of skin (parakeratotic plug) found in a portion of the skin where the outermost layer of the skin (epidermis) is lowered or depressed is a key sign. This plug can sometimes involve hair follicles. A type of cell debris known as basophilic debris is also found in the plug.
Together with this detailed analysis of the skin and its features, doctors also check your blood glucose level to see if you might have diabetes, as this can be an underlying cause of Kyrle disease. Tests are also done to evaluate the health of your liver and kidneys, as liver and kidney disease are other underlying conditions that can be associated with Kyrle disease.
If you are diagnosed with Kyrle disease, your primary care doctor and other specialists need to closely monitor any other systemic diseases you have. This is because the management of these underlying conditions can influence the course and management of Kyrle disease itself. In essence, managing Kyrle disease often involves managing the diseases that accompany it.
Treatment Options for Kyrle Disease
As of now, there are no high-quality studies that have compared different treatments for Kyrle disease, which is a rare skin disorder resulting in itchy, scaly bumps on the skin. Therefore, treatment recommendations are based mainly on individual stories or small studies. The first line of treatment usually involves applying substances like salicylic acid or urea to the skin which help soften, loosen, and remove scales.
Moisturizing creams and oral medications aimed at reducing itchiness have also proven helpful in providing relief. There are other treatment options to consider as well, such as electrocautery (using heat to remove the bumps), cryotherapy (freezing the bumps), laser therapy, topical retinoids (medications applied to the skin that can help control skin growth), isotretinoin (a potent medication often used for severe acne), and treatments involving ultraviolet A radiation. Some patients have found oral clindamycin, a kind of antibiotic, useful too.
However, there’s a chance that the skin bumps may come back once the treatment is stopped. Interestingly, for Kyrle disease patients who also have kidney disease and are undergoing dialysis, skin bumps have completely disappeared after a successful kidney transplant.
In severe cases, where the disease doesn’t respond to these treatments, surgical removal of the skin bumps might be necessary.
What else can Kyrle Disease be?
If you are dealing with Kyrle disease, it’s important to know that the condition needs to be distinguished from other skin issues that lead to the formation of bumps or nodules with hard central cores. Some of these conditions include:
- Prurigo nodularis (some researchers believe Kyrle disease may be a type of prurigo nodularis)
- Multiple keratoacanthomas (these can appear similar to Kyrle disease when examined under a microscope)
- Excoriated lesions (also known as prurigo simplex)
- Folliculitis
- Arthropod hypersensitivity reaction
- Perforation of foreign material from outside or within the body
- Dermatofibromas
If lesions appear in a more linear distribution (a response known as koebnerization), other skin conditions should be considered that might include:
- Psoriasis
- Lichen planus
- Verrucae (warts)
It’s important to note that diagnosing these conditions can be complex, especially since patients with acquired perforating disorders often have concurrent folliculitis and prurigo nodularis. Plus, the way these conditions appear under a microscope can also vary. Finally, people with a history of diabetes, kidney disease, or those who are on hemodialysis are more likely to have an acquired perforating disorder.
What to expect with Kyrle Disease
The future health outcome, or prognosis, for Kyrle disease largely depends on the seriousness of other related health conditions the patient may have. These conditions can include diabetes, chronic kidney disease, and liver disease. The discomfort of Kyrle disease comes from the skin lesions and intense itching it causes.
So, it’s importantly to note that any health issues or life-threatening risks are more often connected directly with these other diseases, rather than the Kyrle disease itself.
Possible Complications When Diagnosed with Kyrle Disease
Kyrle disease only affects the skin and it does not cause systemic complications. However, it can reveal the presence of other unmanaged systemic disorders.
Preventing Kyrle Disease
Patients need to understand their health condition and also manage any related health concerns. For instance, patients with diabetes should closely regulate their blood sugar levels. Those with kidney disease need ideal treatment for uremia, a high level of waste products in the blood due to kidney failure. Once this is treated, Kyrle disease, a skin condition, usually goes away. Also, it’s crucial for patients with liver disease and heart failure to have optimal treatment and care.
