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Wyprysk z hiperkeratozą - Objawy, Diagnoza i Leczenie
Pathophysiology
The skin is composed of three layers: the epidermis, the dermis (composed of the superficial papillary and deeper reticular dermis), and the hypodermis. The skin has structural differences among the different areas of the body in terms of epidermal and dermal thickness, distribution of appendages, and pigmentation. The epidermis is composed of multiple layers of maturing keratinocytes: the basal layer (stratum basale), the squamous layer (stratum spinosum), the granular layer (stratum granulosum), and the cornified layer (stratum corneum). This stratified epithelium is in a constant process of self-renewing and exfoliation that takes 20-40 days to complete. The cells in the outer layer are the most differentiated in the keratinocyte line, composed almost entirely of keratin lamels of high molecular weight, and those are the ones that undergo desquamation, completing the maturation cycle.
When the epidermis is exposed to repetitive injury, it usually elicits an increased proliferative rate of the keratinocytes and accelerates their maturation. Keratinocytes also tend to produce more keratin, thus increasing the stratum corneum's thickness.
Genetic mutations resulting in hyperkeratosis is seen in ichthyoses and keratoderma. There are several damages in keratin-encoding genes such as KRT1 and KRT10, which cause defects in keratin structure. Defective keratin causes irregular aggregates of intermediate filaments, which leads to cellular collapse and blistering. The barrier function is then compromised, and the skin reacts with compensatory hyperproliferation, which leads to hyperkeratosis.
ROGOWIEC
Choroba genetyczna cechująca się hyperkeratynizacją skóry i paznokci. Jest to pogrubienie ograniczone do powierzchni dłoniowych i podeszwowych, pojawiające się zwykle z powodu mutacji.
GŁÓWNE OBJAWY ROGOWCA:
- nadmiernie zrogowaciały naskórek
- żółte lub woskowe zabarwienie skóry
- zgrubiałe i przerosłe płytki paznokciowe
Odmiany rogowca
Unna-Thost pojawia się ok. 1-2 roku życia. Zmiany rogowe są symetryczne, występuje nadpotliwość dłoni i stóp.
Keratoma disseminatum pojawia się po 20 roku życia. Charakteryzuje się drobnymi, rozsianymi wykwitami. Z wiekiem może ich przybywać.
Keratoma trnsgrediens et progrediens pojawia się w pierwszych miesiącach życia. Ogniska hyperkeratotyczne występują poza dłońmi i stopami i najczęściej znajduję się na łokciach i kolanach.
Evaluation
Dermoscopy is noninvasive and allows visualization of the skin structures in the epidermis, dermo-epidermal junction, and superficial dermis.
A biopsy is essential in cases in which the clinical setting is overlapping with different entities having distinctive histopathologic findings. For an ideal full-thickness biopsy, it is important to include the hypodermis. This can be performed with a simple 3 mm punch that minimizes scarring in the affected area. Any smaller size is at risk of being non-diagnostic.
Patch tests can be useful for identifying the causative allergen if an allergic dermatitis is suspected. Clinical clues are the presence of persistent, pruritic, eczematous eruptions in which any other identifiable cause has been excluded. If the patient tests positive, they should be encouraged to avoid the specific allergen. A follow-up after a few weeks of allergen avoidance is strongly recommended.