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The skin displays follicular dyskeratosis (degeneration of the skin in hair follicules), which reflects as hypovitaminosis A (systemic Vitamin A deficiency). [15] The skin reactions are caused by an abnormality in the desmosome-keratin filament complex leading to a breakdown in cell adhesion. [16] [5]
Parry–Romberg syndrome (PRS) is a rare disease presenting in early childhood [1] characterized by progressive shrinkage and degeneration of the tissues beneath the skin, usually on only one side of the face (hemifacial atrophy) but occasionally extending to other parts of the body. [2]
Usually, pseudoxanthoma elasticum affects the skin first, often in childhood or early adolescence. [7] Small, yellowish papular lesions form and cutaneous laxity mainly affect the neck, axillae (armpits), groin, and flexural creases (the inside parts of the elbows and knees). [3] [8] Skin may become lax and redundant.
The two main types of human skin are: glabrous skin, the hairless skin on the palms and soles (also referred to as the "palmoplantar" surfaces), and hair-bearing skin. [3] Within the latter type, the hairs occur in structures called pilosebaceous units , each with hair follicle , sebaceous gland , and associated arrector pili muscle. [ 4 ]
The exact cause of this condition is not known, but it involves collagen degeneration and a granulomatous response in the layer of the skin called the dermis, often affecting the deeper fat layer and thickening dermal blood vessels. Diagnosis is confirmed through a skin biopsy showing inflammatory cell infiltrate and necrotising vasculitis.
Vacuolar interface dermatitis (VAC, also known as liquefaction degeneration, vacuolar alteration or hydropic degeneration) is a dermatitis with vacuolization at the dermoepidermal junction, with lymphocytic inflammation at the epidermis and dermis.
In the skin, this process leads to the abnormal replacement of annular squames with nucleated cells. Parakeratosis is associated with the thinning or loss of the granular layer and is usually seen in diseases of increased cell turnover, whether inflammatory or neoplastic. Parakeratosis is seen in the plaques of psoriasis and in dandruff.
The loose skin can be either generalised or localised. [4] Biopsies have shown reduction and degeneration of dermal elastic fibres in the affected areas of skin. [5] The loose skin is often most noticeable on the face, resulting in a prematurely aged appearance. The affected areas of skin may be thickened and dark.
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