Lepra (leprosy) - Symptoms

Leprosy has an extremely long and uncertain incubation period (from several months to 20 years or more; on average 3-7 years); symptoms of leprosy are very diverse. Leprosy has no clear signs of progression.

According to the modern Ridley-Jopling classification, there are lepromatous (LL), tuberculoid (TT) and three borderline types of the disease: borderline leprosy (BL), borderline lepromatosis (BL) and borderline tuberculoid (BT). According to this classification, four main diagnostic criteria are taken into account when diagnosing leprosy: symptoms of leprosy, bacterioscopic index indicating the amount of the pathogen in the body, the patient's immunological reactivity assessed using the lepromin test and histological examination data.

Prodromal symptoms of leprosy are signs of peripheral autonomic failure and reflex-vascular disorders (marbling of the skin, cyanosis, swelling of the hands and feet, impaired sweating and sebum secretion), as well as weakness, body aches, paresthesia, and nosebleeds. At the onset of the development of manifest forms of the disease, diffuse changes in the color of the skin, single or multiple spotted rashes varying in location, shape, size, and color (erythematous, hypopigmented, hyperpigmented, cyanotic, with a brownish tint), and increasing paresthesia are usually noted. In later stages of the disease, there are limited or diffuse skin infiltrates, papules, tubercles, nodules, rhinitis and rashes on the mucous membranes of the upper respiratory tract, leprosy pemphigus, loss of eyebrows and eyelashes (madarosis), amyotrophy, brittle nails, ichthyosiform signs, ulcers and other trophic disorders, and gross disturbances of superficial types of sensitivity.

Skin symptoms of tuberculoid leprosy (TT leprosy), depending on the stage of the disease, have the form of single or multiple spots with clear edges, as well as papular elements, plaques, sarcoid and ring-shaped border elements with a clearly defined raised edge.

A characteristic sign of tuberculoid leprosy skin rashes is an early detectable decrease in pain, temperature and tactile sensitivity on them, usually extending 0.5 cm beyond the visible boundaries of the lesions, impaired sweating and loss of vellus hair. When tuberculoid leprosy rashes regress, hypopigmented spots remain in their place, and in the case of deeper infiltration - skin atrophy.

[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ]

Lepromatous type

The most severe and contagious (multibacillary) type of the disease. It is characterized by the prevalence of the pathological process. Skin symptoms of leprosy are localized on the face, back of the hands, shins and are represented by limited tubercles (lepromas) and diffuse infiltrates. The color of lepromas varies from pink to bluish-red, their surface is smooth and shiny, sometimes covered with bran-like scales. Lepromas rise hemispherically above the surface of the skin and, closely adjacent to each other, often form continuous tuberculous infiltrates. When the face is affected, lepromas greatly disfigure it, giving the appearance of a lion's muzzle - facies leonine. The course of lepromas is torpid, sometimes they can disintegrate, forming ulcers.

Along with skin changes, lesions of the mucous membranes of the nose, oral cavity, and larynx are often observed. Patients complain of dry mouth, nose, bloody discharge, frequent nosebleeds, difficulty breathing, and hoarseness. Ulceration of the leprosy and infiltrates of the cartilaginous nasal septum leads to its perforation with subsequent deformation of the nose.

Eye damage is observed in the form of keratitis, iridocyclitis, episcleritis, which can lead to visual impairment and even complete blindness.

Lymph nodes (femoral, inguinal, axillary, elbow, etc.) enlarge and can melt during an exacerbation of the disease. Mycobacterium leprae is found in their punctures. The liver, spleen, lungs, kidneys, etc. are affected among the internal organs. Periostitis and leproma develop in the skeletal system.

As a result of nerve damage, sharp pains appear along their course, all types of skin sensitivity (temperature, pain and tactile) are impaired, especially on the hands and feet. Patients, not feeling heat and cold, not feeling pain, are often subject to severe burns, injuries, in some patients, due to nerve damage, the muscles of the face and limbs are weakened and depleted, and sometimes destruction and rejection of fingers and toes (mutilations) are observed, deep ulcers appear on the feet, which are difficult to treat. All this leads patients to severe disability.

Usually, the sluggish, chronic course of this type of leprosy can occasionally be replaced by periods of exacerbation, the so-called leprosy reactions, during which the general condition of patients sharply worsens, existing lesions become aggravated, and new rashes appear. Old leprosy melts and ulcerates, neuritis, lesions of the eyes, lymph nodes, and internal organs become aggravated, and symptoms of "leprosy nodosum erythema" develop.

The initial rash in lepromatous leprosy (LL leprosy) most often has the form of numerous erythematous, erythematous-pigmented or erythematous-hypopigmented spots, the characteristic features of which are small size, symmetrical arrangement and lack of clear boundaries. Most often, spots appear on the face, extensor surfaces of the hands and forearms, shins and buttocks. Their surface is usually smooth and shiny. Over time, the initial red color of the spots acquires a brown or yellowish tint. Sensitivity and sweating within the spots are not impaired, vellus hair is preserved. Subsequently, skin infiltration increases, accompanied by increased function of the sebaceous glands, and the skin in the area of the spots becomes shiny and glossy. Enlarged vellus hair follicles give the skin an "orange peel" appearance. With increasing infiltration of the facial skin, natural wrinkles and folds deepen, the superciliary arches protrude sharply, the bridge of the nose widens, the cheeks, chin, and often the lips have a lobed appearance (lion's face - facies leonina). Infiltration of the facial skin in the lepromatous type of leprosy, as a rule, does not extend to the scalp. In most patients, infiltrates are also absent on the skin of the elbow and popliteal flexor surfaces, armpits and along the spine (immune zones).

Often, already in the early stages of the disease, single or multiple tubercles and nodes (lepromas) appear in the area of infiltrates, the sizes of which vary from a millet grain to 2-3 cm in diameter. Lepromas are sharply demarcated from the surrounding skin and are painless. The most common outcome of their development, if anti-leprosy treatment is not carried out, is ulceration. In all cases of the lepromatous type of leprosy, lesions of the mucous membrane of the nasopharynx are noted. It looks hyperemic, juicy, dotted with small erosions. Later, lepromas and infiltration appear here, massive crusts are formed, which greatly impede breathing. The decay of lepromas on the cartilaginous nasal septum leads to its perforation and deformation of the nose (a "collapsed" bridge of the nose). In very advanced cases of the disease, ulceration of the lepromas on the mucous membrane of the palate may be observed. Lesions of the vocal cords can cause a sharp narrowing of the glottis, aphonia. If the generalization of the lepromatous type of leprosy occurs before puberty, then underdevelopment of secondary sexual characteristics is often observed, in men - gynecomastia. The peripheral nervous system is involved in the process in the late stages of the development of the lepromatous type of leprosy, therefore, skin sensitivity disorders may be absent for a long time.

In patients with lepromatous leprosy, the lepromin test (Mitsuda reaction) is usually negative, which indicates the absence of any ability of the body to recognize and resist the pathogen. From a pathogenetic point of view, this explains the entire severity and unfavorable prognosis of the clinical manifestations of lepromatous leprosy.

The bacterioscopic index in lepromatous patients is usually high, and this is called multibacillary. Due to this, patients with the lepromatous type are the main source of infection and spread of leprosy and, with prolonged contact, pose a great danger to others.

Tuberculoid leprosy

It is polar to lepromatous leprosy and is characterized by a more benign course and is more amenable to treatment. The main elements of the skin rash in the tuberculoid type of leprosy are small reddish-bluish flat polygonal papules. They often merge to form figured elements in the form of disks, rings, and semirings. A ridge surrounding the tuberculoids is very characteristic: their outer edge is somewhat raised, clearly outlined, and stands out with a reddish-bluish color and doughy consistency; the inner edge, on the contrary, is blurred and imperceptibly passes into the central pale part of the lesion. With regression of the rash, depigmentation or atrophy of the skin remains. Due to the dominant lesion of the peripheral nerve trunks, a violation of skin sensitivity and sweating in the lesion foci is typical. Specific leprosy damage to the peripheral nerves leads to a decrease in the area of their innervation of superficial cutaneous sensitivity - first temperature, then pain and, finally, tactile, with the inevitable subsequent formation of severe trophic disorders in the form of myatrophy, mutilation and trophic ulcers.

In the tuberculoid type of leprosy, mycobacteria are rarely found in scrapings of the mucous membrane, and are present in very small quantities in the lesions. In this regard, patients with tuberculoid leprosy are usually called paucibacillary. The chronic course of tuberculoid leprosy can also be interrupted by exacerbations, during which mycobacteria leprae are often found in the lesions.

Borderline types of leprosy

They combine the symptoms of polar types of leprosy, lepromatous and tuberculoid leprosy, with lesser severity, and can be transitional. They are characterized mainly by neurological symptoms and erythematous hyperpigmented and depigmented spots with clear boundaries, of varying size and shape. Hair falls out in the affected areas, sensitivity gradually disappears, and sweating is impaired. After several months and even years, muscle atrophy develops, leading to deformations of the upper and lower extremities. The face becomes mask-like, facial expressions disappear, and incomplete closure of the eyelids (lagophthalmos) appears. Paralysis and paresis, contractures, and possible mutilations develop. Deep ulcers form on the soles of the feet, which are difficult to treat. Trophic disorders can be varied: hair loss, acrocyanosis, nail atrophy, etc.

Undifferentiated leprosy is a separate diagnosis, but not an independent type. The possibility of making such a diagnosis appears when a patient with leprosy seeks medical attention early and the earliest, debut manifestations of the leprosy process on the skin are recognized in a timely manner, which at this initial stage have not yet formed into any independent type of leprosy. The debut symptoms of undifferentiated leprosy are of little specificity and are often represented by barely noticeable hypopigmented spots of initially small sizes, within which skin sensitivity disappears rather quickly, which can serve as the basis for early and timely diagnosis of leprosy.

Symptoms of other types of leprosy are expressed in different proportions (borderline conditions).

Lesions of the peripheral nervous system are sooner or later detected in all forms of leprosy. The peculiarity of leprosy neuritis is its ascending nature and insular (ramuscular) type of sensitivity disorder, which is caused by the destruction of nerve endings in the foci of rashes, damage to cutaneous branches of nerves and individual nerve trunks. Most often, it is the damage to the peripheral nervous system that leads to disability (absence of cutaneous types of sensitivity, amyotrophy, contractures, paralysis, neurotrophic ulcers, osteomyelitis, mutilations, lagophthalmos).

After the introduction of effective anti-leprosy drugs into practice, leprosy ceased to be a cause of death, and the average life expectancy of patients with leprosy exceeds that of the rest of the population.

[ 6 ], [ 7 ], [ 8 ], [ 9 ]