POIKILODERMA (poikilodermia; Greek poikilos motley + derma skin) — the complex of dystrophic changes of skin which is characterized by the mesh hyperpegmentation alternating with scattered sites of an atrophy of skin, and teleangiectasias.
The item happens acquired and inborn, symptomatic and independent. Symptomatic P. can be display of beam dermatitis (see. Dermatitis ), parapsoriasis (see), dermatomyositis (see), sclerodermas (see), and also fungoid mycosis (see. Mycosis fungoid ) and other lymphoma. To independent a wedge, to forms carry an atrophic vascular poikiloderma (some researchers doubt that this form can be considered independent since further quite often it is diagnosed as fungoid mycosis), an inborn poikiloderma and a reticular poikiloderma of Sivatt (see. Melanosis ).
Etiology and pathogeny Items are not clear. In atrophic vascular P.'s pathogeny disturbance of food of skin is of great importance that is promoted by frequent coolings, endocrine and nervous breakdowns, lnfektsionno-toxic factors, etc. Inborn P. is result of an ektomezodermalny dysplasia of hereditary genesis.
At P. thinning of epidermis, accumulation of melanin in a basal layer of skin, smoothing of nipples of a derma with expansion of gleams of vessels are observed, around to-rykh infiltrate from lymphocytes, histiocytes and melanophores polosovidno lies.
Atrophic vascular Poikiloderma (synonym: Jacobi's poikiloderma, a mesh erythematic atrophodermia of Müller, mesh atrophic dermatitis of Gluck, an eritemato-atrophic Poikiloderma of Milian — Perena, etc.) is described in 1906 by Jacobi (E. Jacobi). Meets seldom, are ill at any age. Course of a disease chronic. The face, a neck, a trunk and extremities are surprised. Along with the main changes — teleangiectasias, a nevus pigmentosus and atrophic sites — on skin petechias, a peeling, miliary henna-red follicular (is more rare not follicular) small knots can be observed. The affected skin on certain sites is tense that gives it a nek-swarm similarity to a scleroderma. Ulcerations of skin, damage of a mucous membrane of a mouth, and also a febricula, joint pains, a small face edema and extremities, an itch are possible.
Inborn Poikiloderma (synonym: The poikiloderma is inborn atrophic vascular, Thomson's syndrome) it is described in 1923 by the Australian dermatologist Thomson (M. S. Thomson). The disease is inherited on autosomal recessively type. It is shown in the early childhood, sometimes since the birth. In the beginning rashes have character of a diffusion or spotty erythema, then teleangiectasias, a mesh brown hyperpegmentation, sites of an easy atrophy of face skin, neck, buttocks, axillary areas, extremities develop. Triangular shape of the person with a wide forehead and a small chin, dystrophy of hair and teeth is characteristic, anomalies of a skeleton, a growth inhibition are possible. A wedge, inborn P.'s options are inborn P. with bulging and inborn P. with a warty hyperkeratosis.
Inborn P. is observed also at Rotmunda syndrome (see), an inborn dyskeratosis (a synonym Tsinsser's syndrome — Engmena — Cole) which is observed at males and is characterized, except P., by dystrophy of nail plates (up to their full rejection), a leukoplakia of mucous membranes of language, cheeks, the sky and a leukokeratosis of an anus, intestines, bodies of urinogenital system (see. Leukoplakia ), atrophy of a balanus, ladonnopodoshvenny keratosis, dystrophy of teeth, changes of blood (pancytopenia).
On limited sites of skin of P. it is observed at seldom meeting Blum's syndrome (a synonym an inborn telangiectatic erythema with a nanism), inherited on autosomal recessively type. At such patients aberation chromosomes (in culture of leukocytes) and disturbance of synthesis of serumal immunoglobulins are revealed. Damage of skin develops on the first year of life after insolations in the form of the erythematic centers on a face, auricles, against the background of to-rykh there are teleangiectasias, the small atrophy and a hyperpegmentation is more rare. The actinic cheilitis in the form of vesicular and cortical damages of lips is characteristic, the White's disease on skin of a trunk and extremities is possible. One of the main signs of a syndrome of Blum is the proportional microsomia: the average weight of such newborns, according to Herman (J. German, 1969) — 1988. Further children badly grow and put on weight a little. Mental development at the same time does not suffer. At patients with Blum's syndrome signs of a pituitary nanism, tendency to respiratory and piokokkovy infections and development of an acute leukosis are noted. Besides, at Blum's syndrome various inborn defects — dystrophy of skin and its appendages, malformations of a skeleton in a look klino-and syndactylias, asymmetries of necks of femurs, horse foot, etc., anomalies of teeth, the Cryptorchism are possible.
Diagnosis Items establish on the basis a wedge, the picture confirmed in doubtful cases gistol, a research of skin. Differential diagnosis is carried out with xeroderma pigmental (see), Incontinentia pigmenti (see), and also differentiate various forms of a poikiloderma.
Treatment Items carry out by vitamins A, C, E, P, PP; corticosteroid and anabolic hormones, at Blum's syndrome — photoprotective agents. At symptomatic P. treatment is directed to a basic disease.
Forecast for life depends on a form P. At Blum's syndrome, an inborn dyskeratosis often there comes the lethal outcome from an acute leukosis, malignant new growths.
For prevention P.'s aggravations it is necessary to avoid overcooling, injuries, infections, excess insolation.
Bibliography: Dermatological sindromo-logiya, under the editorship of R. S. Babayants, Yerevan, 1974; Suvorova K. N. and And A. A N-tonyev. Hereditary dermatosis, page 160, M., 1977; German J. Grip ra L. P. Chromosomal breakage in diploid cell lines from Bloom’s syndrome and Fanconi’s anemia, Ann. Genet., t. 9, p. 143, 1966; R o d e r-mund O. E. u. Hausmann D. Das Thomson-Syndrom, ein Beitrag zu den kongenitalen Poikilodermien, Derm. Mschr., Bd 163, S. 601, 1977, Bibliogr.; W o d-n i a n s k at P. "Ober die Formen der con-genitalen Poikilodermie, Arch. klin. exp. Derm., Bd 205, S. 331, 1957, Bibliogr.
S. S. Kryazheva.