Peeling skin syndrome is also associated with 6 syndromes that are each caused by a different genetic defect. The various syndromes include peeling skin syndrome 1, 2, 3, 4, 5, and 6.
Peeling skin syndrome 1 Peeling skin syndrome 1 is caused by a genetic defect in the
corneodesmosin (CDSN) gene. This gene localizes to the human epidermis and other epithelia. The protein experiences a chain of cleavages during corneocyte maturation. Its symptoms include short stature, abnormality of metabolism/homeostasis, scaling skin, pruritus, erythema, asthma, brittle hair, and abnormality of hair texture.
Peeling skin syndrome 2 Peeling Skin Syndrome 2 is caused by a genetic defect in the
TGM5 gene. Transglutaminase 5 is best for catalyzing the cross-linking of proteins and the conjugation of
polyamines to proteins. It also adds to the development of the cornified cell envelope of keratinocytes. Its symptoms include excessive wrinkling of palmar skin, skin erosion, hyperpigmentation of the skin, ichthyosis, and allergy.
Peeling skin syndrome 3 Peeling skin syndrome 3 is caused by a genetic defect in the
carbohydrate sulfotransferase (CHST8) gene. This condition is characterized by asymptomatic lifelong and non-stop dropping of the stratum corneum of the dermis. Its symptoms begin during the second half of the primary decade of existence and encompass generalized white scaling taking place over the upper and lower extremities.
Peeling skin syndrome 4 Peeling skin syndrome 4 is caused by a genetic defect in the
cystatin A (CSTA) gene, an intracellular thiol proteinase inhibitor that has an essential role in desmosome-mediated intercellular adhesion inside the lower levels of the dermis. Symptoms include well-circumscribed peeling of skin on the extremities and neck, generalized dry skin with fine scaling (and sparing of face),
hyperkeratosis, and
palmoplantar keratoderma Peeling skin syndrome 5 Peeling skin syndrome 5 is caused by a genetic defect in the
serpin (serpin family member 8) gene. This gene is produced by platelets and can bind to and inhibit the function of furin, which is a serine protease involved in platelet functions. It is also characterized by superficial peeling of the dorsal and palmar pores and skin of the hands and feet; the pores and skin of the forearms and legs may also be involved. Its symptoms include superficial peeling of small areas of the skin that involve the dorsal and palmar surfaces of the hands and feet, superficial scaling of forearms and legs, and acanthosis.
Peeling skin syndrome 6 Peeling skin syndrome 6 is caused by a genetic defect in the
filaggrin (filaggrin family member 2) gene. The function for this gene is vital for normal intercellular adhesion within the cornified cell layers. It is also critical for the integrity and mechanical strength of the stratum corneum of the epidermis. Its symptom include dryness of the skin, peeling of the skin. erythema at lesion sites, bullae, and hyper-pigmentation. == Signs and symptoms==