Kyrle’s disease: Skin condition with itchy skin lesions

Kyrle’s disease is a rare skin disease in which the patient has itchy skin lesions on various areas of the body. The condition has no specific cause, but several conditions have been associated with Kyrle disease, such as kidney disease and diabetes mellitus. A thorough investigation of the underlying systemic disease and prompt treatment are of utmost importance to prevent complications caused by the underlying condition. There are various options for the treatment of Kyrle’s disease. A combination of several treatments is often necessary to achieve effective results, although this is not possible for every patient and the skin lesions may also return. Josef Kyrle first described this disease in the medical literature in 1916.

  • Synonym of Kyrle’s disease
  • Name of skin disease
  • Epidemiology
  • Causes
  • Risk factors: Kidney failure and diabetes mellitus
  • Symptoms: Itchy skin lesions
  • Diagnosis and examinations
  • Treatment of skin condition
  • Prognosis

 

Synonym of Kyrle’s disease

Hyperkeratosis follicularis et parafollicularis in cutem penetrans is the synonym of Kyrle’s disease.

Name of skin disease

Some scientists consider Kyrle’s disease to be a separate entity. Others claim that the condition is a variant of perforating folliculitis, which is supported by the presence of perforating (penetrating) skin lesions in patients with chronic renal failure receiving dialysis treatment. These scientists see clinical and histological overlapping features between Kyrle’s disease, perforating folliculitis, and even reactive perforating collagenosis. Furthermore, it is also possible, according to some scientists, that Kyrle’s disease and Flegel’s disease (hyperkeratosis lenticularis perstans) are different manifestations of the same disease process.

Epidemiology

Kyrle’s disease is a rare skin condition worldwide, but exact prevalence or incidence data were not found in the sources consulted (October 2020). The skin disease is more common in African Americans, possibly related to the high incidence of diabetes mellitus and renal failure in this population. Kyrle’s disease is six times more common in women than in men. Finally, the symptoms usually manifest when the patient is between thirty and fifty years old.

Causes

The cause of Kyrle disease is unknown as of October 2020. Many theories have been proposed in the literature, including infectious causes, abnormal keratinization, defective differentiation of the epidermis and dermoepidermal junction, increased serum and tissue concentrations of fibronectin, uremia (renal failure) and hyperphosphatemia (diabetes mellitus). The high incidence of systemic disease associated with the development of Kyrle disease suggests an underlying systemic condition as the main cause. There may also be a genetic predisposition to the disease, with different inheritance patterns (both autosomal dominant and autosomal recessive).

Risk factors: Kidney failure and diabetes mellitus

There is a significant association of Kyrle’s disease with renal failure. This skin complication occurs in patients who have already undergone long-term hemodialysis for renal failure (end-stage renal disease). Other rare associated conditions include diabetes mellitus, liver disease (rare), congestive heart failure (poor pumping of blood by the heart), posterior subcapsular cataract and hypothyroidism (underactive thyroid gland).

Symptoms: Itchy skin lesions

The face, mucous membranes, palms, and soles of the feet are usually spared, but the skin disease may affect any other possible areas on the body, such as the legs (most commonly), the trunk, the neck, the abdomen, the arms, the back of the hand, the upper back, the head (rarely),The skin condition is characterized by very itchy hyperkeratotic (keratinized and thickened) and ulcerative nodules and papules (bumps ) with a diameter of two to eight millimeters, which sometimes merge into a plaque. The firm red to dark brown skin lesions have a central, sunken keratin plug (keratin is a protein present in the skin, hair and nails). The skin lesions occur singly or are grouped in linear stripes or plaques. Itching is almost always present, but in some patients the skin lesions are asymptomatic.

Diagnosis and examinations

A blood test is needed / Source: Frolicsomepl, PixabayDiagnostic examination After a thorough physical examination, diagnostic examinations are necessary to confirm Kyrle’s disease. A biopsy of the affected skin shows abnormalities characteristic of Kyrle’s disease. Furthermore, the doctor carries out examinations into the underlying condition. For example, a blood test is necessary to detect diabetes and other abnormalities. The doctor should also perform liver function tests to identify underlying liver disease. A urinalysis may reveal kidney disease.

Differential diagnosis

The clinical picture of Kyrle’s disease is sometimes reminiscent of the following conditions:

  • cutaneous squamous cell carcinoma (form of skin cancer)
  • elastosis perforans serpiginosum
  • folliculitis (inflammation of hair follicles with pain and itching of the skin)
  • hyperkeratosis lenticularis perstans (Flegel’s disease)
  • keratoacanthoma (benign, fast-growing skin tumor)
  • keratosis follicularis (Darier disease: symptoms of skin, nails and mucous membranes)
  • keratosis pilaris (skin condition with red bumps on the skin)
  • lichen planus (disease with symptoms of the mouth, skin and genitals)
  • nodular prurigo (skin condition with very itchy bumps)
  • perforating folliculitis
  • perforating peromibic calcific elastosis
  • reactive perforating collagenosis

 

Treatment of skin condition

The doctor must detect and treat the underlying condition, as this will prevent complications from arising due to the underlying problem. There are several topical (applied to the skin), oral (taken by mouth) and other systemic treatment options for treating the skin lesions of Kyrle disease. The doctor may test several treatments or combinations before obtaining satisfactory results. The patient receives a (combination) treatment with:

  • clobetasol ointment
  • CO 2 laser surgery
  • cryotherapy
  • doxepin
  • phototherapy (treatment via light)
  • isotretinoin
  • soothing anti-itch lotions with menthol and camphor against severe itching
  • oral (taken by mouth) antihistamines
  • salicylic acid and urea (keratolytics)
  • moisturizers

 

Prognosis

In many patients, the skin lesions heal spontaneously over time, or else when the doctor has successfully treated the underlying condition. However, recurrences do occur. The ultimate prognosis depends on the nature of any underlying systemic disease. The quality of life is often affected by the skin condition.

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