Frambeziya

Description:

Frambeziya - antroponozny not venereal трепонематоз with the contact mechanism of transfer of the activator. It is characterized by damages of skin, mucous membranes, bones and joints.

The term "frambeziya" has the French origin (frambois – raspberry) and was offered in 1768 French by the dermatologist of Boissierde Sauvage. The first descriptions of clinical elements were published by Quiedo (1525), Bonitus (1642) and Piso (1648). After the end of World War II the frambeziya was the most widespread disease of the tropical countries. According to WHO data, only in the countries of the equatorial Africa still there are more than 50 million patients with a frambeziya or its versions as treponematoza like a frambeziya – pint, беджель vary in the clinical manifestations and epidemiological features depending on natural, ecological conditions. For many tropical countries the frambeziya, pint and беджель represent not only a medical, but also social problem since are followed by heavy system disturbances with disability and come to an end with an invalidism.

Frambeziya's symptoms:

According to the international nomenclature of clinical manifestations of the frambeziya offered in 1955 to Hackett heading the group of experts of WHO during a disease distinguish early and late stages. Primary and secondary periods, and to late – the tertiary period of a disease belong to an early stage of a frambeziya. Early manifestations of a frambeziya are characterized by plurality and a disseminirovannost of rashes, contageousness, defeat not only skin, but also mucous membranes, bones and joints. Distinctive features of clinical manifestations along with abundance of efflorestsention, their superficial arrangement are high quality, lack of necrotic evolutionary transformations and tendency to spontaneous permission. The late stage, on the contrary, differs in the ogranicheyny, localized, deep inflammatory nodes which are exposed to disintegration, destruction with participation not only skin but also a hypodermic basis, bones, sinews, joints.

The early stage of a frambeziya includes an incubation interval, primary affect and the secondary disseminated rashes. The incubation interval varies of 3-6 weeks up to 4 months. Unlike an incubation interval at syphilis which proceeds imperceptibly without prodrome, at a frambeziya the expressed prodromal phenomena are observed: fever, headaches, feverish state, ostealgia, joints, gastrointestinal frustration. Especially hard the phenomena a prodrome at children proceed.

Primary affect, or frambeziyny chancre (a frambezoma, a pianoma), forms on site implementations of Tr. pertenue Castellani in the form of a flat small knot of pasty density or a pustule with the papillomatous growths covered with the caseous yellowish-green separated or plentiful pus shrinking in massive crusts. Quite often in the basis of an element there is an ulceration to formation of the crateriform ulcer which bottom is covered with granulations with papillomatous multiple outgrowths, than it reminds a cancer ulcer. The basis of an ulcer, granulation and papillomatous growths differ in juiciness, softness of infiltrate, bleeding. Separated contains a large number of activators which easily are found by microscopy in a dark field of sight.

Under the influence of maceration of an epithelium purulent separated and implementations of the activator in new entrance gate around primary form are formed affiliated, so-called chancres satellites, at different stages of development. The subsequent their expansion deep into and on the periphery with an ulceration of fabric and a hyperplasia of nipples of a derma comes to the end with merge to formation of large conglomerates of infiltrative and ulcer character to papillomatous outgrowths and caseous separated. Frambezomam limfangita and lymphadenites – inflammatory, painful, often complicated by consecutive infection accompany. Healing of primary affect happens a miscellaneous and in the ways depending on defeat depth. Superficial frambezoma are resolved with formation of the hypopigmented or hyper pigmented spots with an easy peeling. Deep knotty and ulcer primary affects leave hems. Most often frambezoma are localized on the lower extremities, hands, an oral cavity, a red border of lips, generative organs.

Later 3-6 weeks from the moment of emergence of primary center there comes the first wave of a hematogenous and generalized early enanthesis and mucous membranes and infection of bones and joints. In 3-6 months these manifestations spontaneously disappear. There comes stage of latency which then is replaced by a recurrence of early frambeziyny defeats. A recurrence can be a little, and, than the infection, that stage of latency protyazhenny, and rashes less plentiful is longer. Approximately in 5 years there comes the late stage with knotty and ulcer elements which as well as early frambeziyny defeats, can be exposed to spontaneous permission in several months or years, being replaced by a latent phase of a disease.

Secondary enanthesis and mucous membranes calls frambezidam. Early frambezida are presented by polymorphic morphological elements: eritemato-desquamative, papular and papillomatous, lichenoid, giperkeratotichesky, erosive and erosive and ulcer. They are localized on any site of an integument and, as a rule, multiple, disseminated. Spotty frambezida differ in stagnant-brown coloring, a plentiful melkoplastinchaty peeling and tendency to merge. They exist not for long, are depigmented in the center, leaving the hyper pigmented border in a circle, then in 2-4 weeks pass into nothingness. Spotty frambezida are often combined with early papular and papillomatous forms. Papular elements dense, brownish-red color, are shelled and at a palpation cause a feeling of a grater. The size of papules varies from miliary to nummulyarny. Sharp-pointed papules or with papillomatous growths are often observed. Локализуясь in large folds and in the field of generative organs, they hypertrophy, eroded, reminding flat condylomas at patients with syphilis. Especially often spotty and squamous and papular rashes are located on palms and soles where are formed the expressed hyperkeratosis, cracks, painful erosion, the crateriform deepenings with a dry bottom and the subdug edges. On a surface of cracks and erosion papillomatous outgrowths with purulent separated, containing a large number of activators are formed. The Giperkeratotichesky stratifications alternating painful cracks and erosion on soles complicate the movements that changes gait of the patient because of what this form of a disease received the name "crab yaws" ("crab-frambeziya").

Damage of bones and joints in an early stage of a frambeziya is shown by a painful swelling, puffiness of soft tissues over the struck bone sites. Phenomena of a periostitis, osteitis or osteoperiostitis of long tubular bones (tibial, beam, etc.) are short-term, are not followed by an ulceration and a necrosis and in 2-3 weeks completely disappear. At the expense of a hypertrophic osteoperiostitis of tibial and beam bones acinaciform shins and the arc-shaped curvature of a forearm are quite often formed. Further, in later period of an early stage of a frambeziya, there are hydrarthroses, a tendovaginitis, synovites. At children phalanx osteoperiostites of brushes (polydactylites) with the puffiness and sharp morbidity complicating the movements are especially often observed. In the same period a peculiar damage of bones of a nose – Gounda forms. Owing to an osteoperiostitis of bones of a nose and an upper jaw with the subsequent edematous infiltration of skin dense roundish tumorous ledges on both sides of a nose, on cheeks, a forehead are formed. Tumorous educations deform nose bones, a hard palate, burgeon in an eye-socket, complicate nasal breath. Skin over a tumor is not soldered to the subject fabrics, is not ulcerated, painless. The disease is followed by an intensive headache and a sanious purulent discharge from a nose.

The late frambezida of skin presented by deep gummous tumorous nodes and diffusion infiltration are exposed to expression, destruction, scarring. Gummous язвен-ные the centers are characterized by the pasty density, the bluish-brown or brown-red coloring expressed by morbidity and red papillomatous granulations. Ulcers with accurate, crateriform edges are followed by pain.

Bottom their uneven with scanty separated among papillomatous granulations. Ulcers cicatrize with formation of rough keloid ledges and contractures.

Damage of bones in a late stage of a frambeziya consists in development of gummous periostites, osteitises and osteoperiostites with a necrosis and destruction of a bone tissue, spontaneous changes, nearthroses and an invalidizapiya of patients. More often bones of shins, forearms, brushes are surprised. However quite often edges, a breast are involved in process. Damage of joints proceeds in the form of gummous arthritises with defeat of an intra joint part of an epiphysis, cartilages, the joint capsule and formation of hydrarthroses, synovites, bursitis with deformations, ankiloza. At distribution of gummous process on periartikulyarny fabric there are deeply getting, is long not healing ulcers leading to an invalidism of patients. The mutiliruyushchy nasopharyngitis – a gangosa (gangosa), representing gummous defeat of nasopharyngeal structures with partial or final fracture of cartilages and bones of a nose, a hard and soft palate, adjacent soft tissues of the person is characteristic of this stage of a frambeziya. The gangosa is followed by pains in frontal area and the sanious allocations from a nose containing inclusions of the destroyed bone parts. Gummous infiltration extends to wings of a nose, a cheek, an upper lip where the deforming infiltrates and ulcerations are visible. Process comes to an end with scarring and the spoiling destructive destructions of a soft and hard palate, nose, a frontal bone with formation of brain hernia. Also fibrous circumarticular nodosity which is characterized by the expressed density and painlessness is characteristic of a late stage of a frambeziya.

Complications
Without treatment approximately at 10% of the people suffering from a frambeziya in 5 years the complications leading to a disfiguration and disability as the disease can cause considerable destructions of skin and bones develop. It can lead to deformation of legs, a nose, the sky and an upper jaw also.

Frambeziya's reasons:

The infestant of Treponema pertenue Castellani, (Treponema Variabilis), found in 1905, represents a spiral-shaped treponema from 8 to 20 microns long and with a diameter from 0,2 to 0,4 microns, very similar on the causative agent of syphilis. Given N. M. Ovchinnikov and V. V. Delektorsky (1974), Treponema pertenue Castellani differs from Treponema pallidum only in existence of a two-layer cover, a difficult, but single-layer cytoplasmic membrane and cysts with a single-layer cover (the pale treponema has a three-layered cover, a two-layer cytoplasmic membrane and cysts with a three-layered cover).

Lack of a multilayer cytoplasmic membrane at Treponema pertenue and at its cysts promotes more effective influence of antibiotics on it. Antigenic Treponema pertenue and Treponema pallidum properties are very similar, especially on concentration of the lipidic, immunofluorescent and immobilizing antibodies. Therefore Wassermann reaction, the REEF and RIBT at a frambeziya positive though the caption of lipidic antibodies, as a rule, at a frambeziya is slightly lower, than at syphilis. The antigenic community of both treponemas causes a cross immunity between syphilis and a frambeziya.

Frambeziya's treatment:

In an early stage of a frambeziya appoint penicillin drugs (Bicillinums, PAM, экстенциллин) in number of 1200000-2 400 000 PIECES to 1-2 injections. At late forms enter 4800000 PIECES in the form of 2-4 bucketed injections in 5-10 days. Already in 24-28 h the disease-producing factor disappears, but permission of manifestations occurs in 1-1,5 months. Late forms are also exposed to regress, but not earlier than 1,5-3 months. Apply also tetracyclines, levomycetinum, cephalosporin, erythromycin on 1 g a day within 14 days. For precautionary treatment, in case of contact with patients, 1 injection of dyurantny drug of penicillin is carried out (the adult in number of 600 000 PIECES, to children to 15 years – 300 000 PIECES).