What is that
Follicular keratosis is a dermatological disease that creates alterations in the keratinization of the hair follicles.
"Follicular keratosis" and "pilar keratosis" are synonyms and indicate a condition of hyperkeratosis, that is to say an abnormal thickening of the stratum corneum of the epidermis: in this article we will first examine a rare variant of the disorder - called Siemens transferring spinulosic follicular keratosis - then the inverse follicular keratosis will be briefly examined and, finally, the connection between follicular keratosis and Darier's disease will be briefly analyzed.
Follicular keratosis spinulosa decalvante by Siemens
Siemens transferring spinular follicular keratosis is a rare variant of keratosis pilare which occurs in different parts of the body. In detail, Siemens transferring follicular keratosis belongs to the group of skin diseases defined as rare genodermatoses.
Transmission
Descaling follicular keratosis is genetically transmitted as a recessive trait linked to the X chromosome: for this reason, affected women are generally healthy carriers (the same concept we analyzed in color blindness). This theory of genetic transmission, however, has been questioned by some recent research: it seems, in fact, that the follicular keratosis of Siemens, in some subjects, is transmitted as an autosomal dominant trait; consequently the disease can manifest itself in both men and women with the same probability.
Signs and Symptoms
Follicular keratosis begins at puberty with the formation of keratotic papules of the follicles: it is a disease that affects the skin, eyes and hair follicles at the level of the ears, palms, soles of the feet, neck and face. The affected areas show a progressive loss of eyelashes and eyebrows, beard and hair (for this reason, follicular keratosis is often associated with generalized alopecia). The eyelids are also damaged, as they become thicker; at the ocular level, yes it also witnesses a slow but progressive degeneration of the cornea.
Other symptoms associated with this form of follicular keratosis are represented by: photophobia, facial erythema, inflammation on the face caused by bacterial infections, atopy and palmar-plantar keratoderma.
Treatment
Unfortunately, science has not yet found the suitable cure for the complete resolution of the disorder: it is certain, however, that the signs left by Siemens follicular keratosis can be alleviated and kept under control by applying corticosteroid-based ointments. Furthermore, in some cases, but not all, the disease responds positively to therapy with systemic retinoids.
In the event that follicular keratosis is also accompanied by bacterial infections and inflammatory sores, the use of antibiotics is recommended, obviously with a medical prescription.
In any case, before undergoing any therapy, the biopsy of the affected skin tissue, of medical competence, is essential to ascertain the Siemens transferring spinular follicular keratosis.
Reverse Follicular Keratosis
This disease is also known as "irritated seborrheic keratosis": it is a rare disorder that affects the face. It is called "seborrheic" due to the histological characteristics it presents, similar to seborrheic keratosis. In inverse follicular keratosis, however, there are also squamous areas arranged in a vortex, which progressively extend on the surface of the epidermis. It manifests itself with papillomas at the level of the eyelid: they are not pigmented and can develop into more serious forms, so as to prevent a correct vision to the subject.
The only possible treatment to cure inverted follicular keratosis is surgical excision of the papilloma.
Darier's disease
Relationship between Follicular Keratosis and Darier's Disease
Follicular keratoses are also found in Darier's disease, an autosomal dominant cutaneous disorder manifesting as oily, yellow or brown keratotic papules. Darier's disease is also known as follicular keratosis or Darier-White disease. "and also belongs" to the group of rare genodermatoses.
This form of follicular keratosis occurs mainly in the seborrheic areas of the face and trunk and, more specifically, its manifestations affect the forehead, scalp, ears, neck, back and upper chest.
Darier's disease refers to follicular keratosis since it causes bullous papules similar to those that occur in keratosis: in this regard, both skin manifestations are considered variants of verruciform acrokeratosis (rare form of dermatosis).
However, given the particular characteristics of the papules with which the pathology manifests itself, Darier's disease could be misinterpreted as seborrheic dermatitis.
Signs and Symptoms
As mentioned, Darier's disease is characterized by the formation of fatty, yellow or brown keratotic papules, which are associated with characteristic nail changes.
The papules can join to the point of forming real plaques, decidedly unsightly. Such lesions can become irritated and infected, becoming malodorous and thus constituting a major problem for the patient. The nails, on the other hand, are fragile and have a characteristic and anomalous "V shape".
Furthermore, on the backs of the hands and feet there are dotted keratoses which represent a typical sign of this pathology.
Symptoms of Darier's disease can be worsened by exposure to the sun or artificial UVB rays, as well as negatively affected by heat or sweating.
Treatment
Treatment with isotretinoin can be useful to counteract the typical hyperkeratosis associated with this disease, as can products based on lactic acid or urea. The use of topical corticosteroids, on the other hand, can help counteract irritation. In severe cases, the doctor may also prescribe the administration of retinoids orally.
As an alternative to drug treatment, surgical measures, laser removal and dermabrasion can be adopted to eliminate the bullous papules typical of Darier's disease and follicular keratosis.
