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December 27, 2016 00:07

Pachyonychia innate .Causes.Symptoms .Diagnostics.Treatment

pachyonychia congenital (synonym: Jadassohn-Lewandowski syndrome, congenital polikeratoz Jadassohn-Levapdovskogo) - version of ecto-mesodermal dysplasia.Inheritance heterogeneous, autosomal recessive, sex-linked.Most men suffer from.

causes and pathogenesis of congenital pachyonychia unclear.In the urine there is a high level of hydroxyproline.

Symptoms pachyonychia innate.Dermatoses begins immediately after birth or in the first days of life.The main symptom is loss of the nail plate, t. E. Onychodystrophy of hypertrophic type.Thickened nail plate to the 1 cm (pachyonychia) become dense, yellowish to brown color on their surfaces have longitudinal stripes.Pronounced subungual hyperkeratosis.Some patients develop the phenomenon of paronychia.For a long time the defeat of nails may be the only symptom of the disease.Then develop other symptoms of congenital pachyonychia: alopecia, sometimes diffuse palmar-plantar keratoderma, rash on the skin of the trunk and limbs - keratosis pilaris in a red cone-sh

aped keratotic papules, ichthyosiform rash, hyperpigmentation, skin abnormalities, etc. On the mucous membranes of the tongue, larynx.nose appear leukoplakia lesions, there is corneal damage the eye, which leads to loss of vision.Observe the development of dental abnormalities of the musculoskeletal system, disorders of the nervous, endocrine and cardiovascular systems, mental retardation.

In clinical practice, there are four types of congenital pachyonychia, which are characterized by the following symptoms:

  • congenital pachyonychia, keratoderma, follicular keratosis body, leukoplakia of mucous membranes and cornea;
  • congenital pachyonychia symmetrical keratoderma, keratosis body, leukoplakia of mucous membranes;
  • congenital pachyonychia symmetrical keratoderma, follicular keratosis of the body;combination of the keratoses and pachydermia with congenital anomalies of the mesoderm (osteopathy, etc.).

Histopathology.Histological study of the pathological area is defined by a significant thickening of the epithelium as a result of hyperkeratosis, intracellular edema, parakeratosis.Possible dyskeratosis, inflammation in the upper dermis.The inflammatory infiltrate around the vessels consist of lymphocytes, histiocytes.There akantokeratoliz.

pachyonychia Treatment of congenital little success.We recommend long-term use of vitamin A, riboflavin, folic acid, gelatin, according to testimony - hormonal preparations.Good effect have aromatic retinoids.

External used hot soda baths, 2-10% -s' salicylic ointment ureaplast, ointments to include 5-10% strength salicylic, lactic acid, resorcinol - for the removal of hyperkeratotic layers on the hands and feet, and in the subsequent- the application of mitigating ointments, cryotherapy with liquid nitrogen, electrocoagulation.The nail plate is removed with the help of ureaplasta followed by rubbing into the nail bed corticosteroid ointments, creams with vitamin A.

Marked good effect of the use of topical retinoids (adapalene, tretinoin, and others.).