Scleroderma of the scalp.

Scleroderma rarely affects the scalp. Among its various forms in this localization, in descending order, there are linear scleroderma of the frontoparietal region, systemic scleroderma, widespread plaque and small-focal scleroderma, or scleroatriphic lichen. Dermatosis is more common in women, and its linear form - in children. Due to the peculiarities of clinical manifestations of scleroderma and the presence of hair, the stages of erythematous spot and compacted plaque are not detected on the scalp. The lesion is detected at the final stage of the disease, when focal atrophic alopecia, or pseudopelade condition, is formed. The surface of the lesion becomes smooth, shiny, fused with the underlying tissues, completely devoid of hair.

In linear scleroderma of the frontal region, the lesion usually begins with the scalp, where it is represented by a vertically located strip of atrophic cicatricial alopecia 1-3 cm wide, descending onto the skin of the forehead, then onto the bridge of the nose, and sometimes onto the upper lip. In shape and location, the atrophic scar is very reminiscent of a mark left after a sabre blow. In some cases, stripe scleroderma of the frontoparietal region is accompanied by Romberg's hemiatrophy of the face. In this case, near the eye, in the zygomatic region or in the lower jaw area, all tissues (subcutaneous fat, muscles, cartilage and bones of the skull) atrophy in the affected areas. Hair falls out not only in the area of the affected part of the scalp, but also on the eyebrows and eyelids. The face becomes asymmetrical, the affected part is smaller than the healthy one, the skin on it is atrophic, dyschromic with numerous folds and furrows. In the EEG of such patients, a diffuse irregular rhythm of brain waves may occur on the affected side.

On the scalp, the lesion may be isolated or be one of many foci of widespread plaque scleroderma. Its foci are localized mainly on the trunk and limbs, rarely in the forehead and scalp. Thus, Saenko-Lyubarskaya V.F. (1955) out of 36 patients with various forms of scleroderma, including systemic forms, found lesions of the scalp and face in only one patient. Guseva N.G. (1975) observed lesions of the scalp in the type of discoid lupus erythematosus in 4 out of 200 patients with systemic scleroderma, manifested mainly by foci of cicatricial atrophy with alopecia. These changes preceded the development or detection of systemic scleroderma. Thus, one of these patients developed a bald spot on the scalp at the age of 19 and was diagnosed with discoid lupus erythematosus. Six years later, the patient developed two new similar spots on the scalp, and in the autumn of the same year - vasospastic phenomena on the arms, then legs, general weakness, myasthenic syndrome. Systemic scleroderma was diagnosed. Manifestations on the scalp (atrophic alopecia) were considered (most likely erroneously) as a combination of systemic scleroderma and discoid lupus erythematosus due to the great similarity of the clinical manifestations of these diseases on the scalp. This example confirms the great difficulties in diagnosing isolated scleroderma of the scalp. The results of a histological examination of the affected skin can help establish the correct diagnosis.

Histopathology

Histopathological changes largely depend on the duration of the lesion. In the initial, edematous-inflammatory stage, the spinous layer of the epidermis is little changed, vacuolar degeneration of the cells of the basal, and sometimes the spinous layer is found. In the dermis, thickened and closely adjacent collagen fibers are noted, between which there is a moderately expressed, mainly lymphocytic infiltrate, the walls of the vessels are edematous. When the subcutaneous fat layer is involved in the process, its connective tissue septa thicken due to inflammatory infiltration and neoplasm of collagen fibers, which in places completely replace it. In the late, sclerotic stage, inflammatory phenomena are weakly expressed, the epidermis is atrophic, the border between it and the dermis appears as a straight line due to the absence of the papillary layer. Collagen fibers are sclerotic, compact, there are few fibroblasts; the infiltrate is absent or remains in small quantities perivascularly. The vessel walls are thickened due to fibrosis, their lumens are narrowed. The sebaceous glands and hair follicles are atrophied. The subcutaneous fat is also thinned, partially replaced by sclerotic collagen tissue.

Diagnosis of scleroderma of the scalp

Scleroderma of the scalp is differentiated from other dermatoses, which in this localization lead to focal atrophic alopecia - pseudopelade condition. In addition to dermatoses, which most often lead to pseudopelade condition, one should also remember scleroderma-form basalioma of the scalp, scleroderma-form manifestations that occur after exposure to certain drugs and bone marrow transplantation. Metastasis of cancer of internal organs to the scalp may also resemble manifestations of scleroderma.

Sclerodermoid basalioma is one of its rare and unusual forms. It is usually localized on the skin of the forehead, but can also affect the temples, neck and scalp. It is a lesion, a thickened plaque the size of a coin in the form of a sclerotic plate with a smooth, rarely flaky surface, yellowish-waxy in color with distinct telangiectasias penetrating its surface. On the scalp, the cicatricially altered surface of the sclerodermoid basalioma is devoid of hair and can protrude somewhat above the surrounding unaffected skin surface. Unlike other flat basaliomas, with its sclerodermoid variety there is no characteristic peripheral ridge and no ulcerative decay. It is prone to long-term slow peripheral growth. Histological examination allows to verify the diagnosis. Among the powerfully developed stroma, often sclerotic and hyalinized, thin strands and complexes consisting of compactly located small dark cells are visible. The overall picture of the lesion resembles scirrhous cancer of the stomach or mammary gland.

Scleroderma-like manifestations in the skin have been described as a characteristic side effect of treatment with the antitumor antibiotic bleomycin. Against the background of its use, patients develop scleroderma-like nodules and plaques, sometimes widespread thickening of the skin. Induration often develops on the hands, which can lead to necrosis of the fingers, as in the acrosclerotic form of scleroderma. Several months after discontinuation of the drug, the disease usually regresses.

Injections of the opioid analgesic pentazocine may cause localized or generalized sclerosis of the skin in alcoholics and drug addicts. In some cases, fibrosis of the skin and muscles may be combined with calcification of the subcutaneous fat and muscle tissue, and ulcers sometimes form in the affected areas. Laboratory parameters (except for an increase in ESR) usually do not change.

In the late phase of chronic graft-versus-host disease, which occurs in some patients after allogeneic bone marrow transplantation, generalized sclerotic and atrophic lichen or scleroderma-like skin changes develop. In patients with widespread scleroderma-like skin manifestations induced by drugs or bone marrow transplantation, lesions are likely to be localized on the scalp.

Metastases of primary cancer of internal organs, which are rarely localized in the scalp, can manifest in this localization as scleroderma-like bald spots ("neoplastic alopecia"), resembling plaque scleroderma. They can occur without affecting regional lymph nodes and, unlike scleroderma, are characterized by a rapid increase in number and size and occur in individuals who have previously undergone surgical treatment for breast cancer or other localizations.

Scleroatrophic lichen of the scalp

Most authors classify primary sclerosing and atrophic lichen as a kind of small-plaque scleroderma (syn.: guttate scleroderma, white spot disease, sclerotic lichen, or white lichen of Zumbusch). It affects mainly women, may be accompanied by typical scleroderma plaques and is usually localized on the neck, upper chest, flexor surface of the limbs, abdomen, genitals, and less often in other places. In the literature, there are individual reports of sclerosing lichen affecting, in addition to favorite areas, the scalp with the formation of cicatricial atrophic alopecia. Rook A. and Dauber R. (1985) believe that sclerosing lichen of the scalp is rare. In the domestic literature, we were unable to find a description of pseudopelade caused by this dermatosis. In recent years, we have observed two elderly women with barely noticeable small-focal atrophic alopecia and widespread, long-term recurring scleroatrophic lichen on the trunk, limbs, and anogenital area. These patients have small plaques on the scalp, atrophic changes in the skin with thinning hair, not accompanied by any subjective sensations. Upon careful examination, small (3-4 mm in diameter) oval areas of skin without hair and mouths of hair follicles with a white and smooth surface were found in the frontal-parietal region. They did not have a distinct border, were at the level of the surrounding skin and smoothly merged into it. When palpating these areas, the skin "wrinkled" somewhat more than the adjacent one. Follicular keratosis was absent in these foci. In patients with lichen sclerosus, large atrophic plaques were not found on the scalp, which they had on the skin of the trunk, limbs and in the genital area. Histological examination of the lesions on the skin of the scalp in patients with lichen sclerosus was not carried out, therefore, there is no convincing evidence of a single genesis of skin lesions on the trunk and on the scalp. It is also impossible to exclude the possibility of similar changes in the scalp in elderly women with long-standing androgenic alopecia. Perhaps, with a targeted study of patients sclerosing lichen allows us to reliably prove the presence of pseudopelade caused by this dermatosis.

Treatment of patients with pseudopelade caused by scleroderma

Treatment of patients with pseudopelade caused by isolated plaque scleroderma of the scalp or as a manifestation of a widespread or systemic form of the disease is aimed at the known links in the pathogenesis of scleroderma. Therapy is based on inhibition of increased biosynthesis of abnormal collagen fibers, normalization of microcirculation in the lesions and reduction of autoimmune shifts. It is important to exclude or reduce the impact of factors in patients that provoke the development or progression of the disease and cause in some cases a scleroderma-like syndrome that is very similar to the manifestations of scleroderma (silicon dioxide, polyvinyl chloride, trichloroethylene, hexachloroethane, benzene, toluene, xylene, artificial resins, oil, diesel oil, paraffin, silicone, contaminated vegetable oil - denatured rapeseed oil, etc.). Therefore, it is also necessary to avoid exposure to certain medications (bleomycin, pentazocine), vaccines, serums, ultraviolet radiation and penetrating radiation, hypothermia, mechanical injuries, hormonal disorders, and sanitize foci of infection. Plaque scleroderma after months and years can transform into a systemic form < of the disease. In this regard, each time a patient with active foci of plaque scleroderma visits a doctor, it is necessary to conduct a clinical and immunological examination to exclude the systemic form. The main criteria for distinguishing between systemic and focal forms of scleroderma are vasospastic changes in the distal extremities, occurring as Raynaud's syndrome, damage to the musculoskeletal system and internal organs, as well as characteristic immunological disorders. During an objective examination of patients with scleroderma, a dermatologist evaluates the nature and area of skin damage, paying special attention to the patient's hands and face. Typical skin changes retain the leading diagnostic value among other clinical manifestations of systemic scleroderma and are the main ones in the diagnosis of its focal forms. The predominant localization of skin changes in systemic scleroderma are the hands, forearms and face. As the disease spreads, the skin of the chest, back (a feeling of a "corset" or "shell") is also affected, sometimes the entire surface of the trunk and limbs. In addition to dense edema, induration and atrophy of the skin, focal hyperpigmentation and multiple telangiectasias on the face, neck, chest and limbs are also of diagnostic value. Systemic scleroderma is characterized by a dark blue color of the nail bed on the fingers (less often - and feet); the nails are reduced in size and flattened, the cuticles of the nails are widened with fringing ("frayed") of the distal edges, sometimes with telangiectasias (as in lupus erythematosus and dermatomyositis). The nail plates are curved like claws, there may be small painful ulcers (partially under the crusts) or scars on the fingertips,the fingers are shortened and pointed due to lysis of part of the terminal phalanges, their skin is thickened, their claw-like bent position is characteristic. The face of patients with systemic scleroderma is amimical and gives the impression of a mask. The skin of the face is stretched, thickened, has a waxy color, sometimes pigmented, with telangiectasias. The nose is pointed, the oral opening is narrowed, the red border of the lips is thinned, atrophic, pale, radial folds are formed around the mouth ("purse-string" mouth), the tongue becomes rigid, shortens, its frenulum is thickened, sclerotic. On the scalp, the atrophic process is manifested by diffuse, less often - focal hair loss, "pseudopelade condition".

Unlike systemic scleroderma, focal forms of the disease almost never affect the hands. An exception is stripe scleroderma, in which skin lesions can be located along one limb, sometimes spreading to its distal parts. A study of the vasomotor reflex on the fingers of patients with scleroderma revealed an early disturbance of microcirculation in the systemic form of the disease, which leads to a slow recovery of the initial temperature in the finger after its dosed cooling. This does not occur in patients with focal scleroderma, with the exception of stripe scleroderma of the extremities, when a similar disturbance of microcirculation is present only on the affected hand. In addition to an objective examination of the patient by a dermatologist, consultations with a therapist, neurologist and ophthalmologist are also necessary (the last two specialists are especially important for patients with lesions localized on the scalp). A study of the organs that are most often affected by systemic scleroderma is carried out. To detect lung pathology, a chest X-ray is prescribed, esophagus - barium fluoroscopy in the supine position, heart - ECG and echocardiography, kidneys - Reberg tests, fluctuations in creatinine, urea, etc. The absence of changes in chest X-rays (diffuse pneumosclerosis with bronchiectasis and cysts in the lower lobes of the lungs - "honeycomb lungs", adhesions, pleural fibrosis, pulmonary heart), normal patency of the barium lump along the esophagus without sluggish, slow peristalsis, segmental expansions, protrusions and narrowing in its lower third, the absence of data on ECG and echocardiography for myocarditis, myocardial sclerosis, hypertrophy and dilation of the right ventricle of the heart, normal creatinine clearance and renal function - allow us to exclude systemic damage in scleroderma. Routine laboratory tests are less informative at the onset of systemic scleroderma. In a clinical blood test, attention is paid to an increase in ESR, in a proteinogram - to hyperproteinemia and hypergammaglobulinemia, in a urine test - to proteinuria and changes in sediment (casts, leached erythrocytes). The titers of antinuclear antibodies, antibodies against cytoplasmic RNA and collagen, rheumatoid factor, etc. are studied. Such examination and treatment of a patient with scleroderma is preferably carried out in a hospital setting.

In the active stage of focal scleroderma, intramuscular injections of water-soluble penicillin (sodium salt of benzylpenicillin) are prescribed daily 2,000,000-3,000,000 IU for 2-3 weeks. The basis of the therapeutic effect of penicillin in scleroderma is unknown. There is an opinion that penicillin is partially transformed in the body into D-penicillamine, which determines its effectiveness. In some cases, lesions on the extremities, similar to scleroderma, but with a more pronounced inflammatory reaction, are a manifestation of borreliosis, where the effectiveness of penicillin is well known. At the same time, penicillin is a strong allergen and can cause allergic reactions of both immediate (more often) and delayed types. The most common immediate allergic reactions include urticaria, Quincke's edema, bronchial asthma, and occasionally anaphylactic shock may develop. Therefore, before prescribing this antibiotic, patients are tested for its tolerance in previous uses. Contraindications to prescribing penicillin include a history of allergic diseases (bronchial asthma, urticaria, atopic dermatitis, hay fever), as well as hypersensitivity and unusual reactions to the use of a cephalosporin antibiotic or griseofulvin. Particular caution is required when prescribing intramuscular injections of penicillin to patients with intolerance to a number of other drugs and to women with long-standing foci of infection (trophic ulcers of the shins, chronic tonsillitis, sinusitis, frontal sinusitis, odontogenic osteomyelitis, chronic adnexitis, etc.) due to the risk of anaphylactic shock. With good tolerance and effectiveness of penicillin, it is advisable for patients with focal scleroderma to undergo preventive courses of treatment 2 times a year (in spring and autumn).

If penicillin is insufficiently effective or there are contraindications to its use, D-penicillamine therapy (cuprenil, artamin, melcaptil, bianodyne) can be administered. It is a complexing compound that binds and accelerates the removal of copper, mercury, arsenic, lead, zinc, etc. ions from the body. In addition, D-penicillamine has the ability to suppress collagen synthesis, depolymerize macroglobulin complexes, break down cross-links between newly synthesized protocollagene molecules, and is a pyridoxine antagonist.

Contraindications to the use of penicillamine include a history of hypersensitivity to penicillamine or penicillin, pregnancy and lactation. It is advisable to avoid prescribing it to people with intolerance to cephalosporin antibiotics and griseofulvin, with impaired liver function, pancreatitis, gastric ulcer, anemia, leukopenia, polyneuritis, and alcohol abusers. Before prescribing penicillamine, a hemogram, transaminases, and creatinine levels in the blood are examined. The drug is prescribed on an empty stomach 1 hour before meals or 2 hours after meals, without combining it with other medications. In focal scleroderma, there is usually no need to prescribe high daily doses of the drug. The initial dose of D-penicillamine in these cases is 150-250 mg per day (1 capsule or tablet). Large doses of the drug (over 1 g per day) used in the treatment of systemic scleroderma cause side effects in about 1/3 of patients, which leads to its forced cancellation. During treatment, the patient must be under medical supervision: once every 2 weeks, a clinical blood test (decrease in the number of platelets, hemoglobin, later - erythrocytes and leukocytes) and urine, once a month, liver function is monitored (transaminases, bilirubin, creatinine, gamma-glutamyl transferase). If penicillamine is well tolerated, control studies are carried out once every 3-6 months. Slowly increasing the dose of the drug reduces the frequency of some side effects and improves its tolerability. Nausea, anorexia, vomiting, glossitis, aphthous stomatitis, loss of taste or its distortion, reversible polyneuritis (due to vitamin B6 deficiency) are possible during treatment; Rarely, diarrhea, hepatitis, intrahepatic cholestasis, nephritis, fever, toxemia, induced lupus erythematosus syndrome have occurred during treatment; anemia, thrombocytopenia, leukopenia, agranulocytosis, eosinophilia, proteinuria, etc. are possible.

The arsenal of agents that have a therapeutic effect in patients with scleroderma also includes unithiol, which is little known in this capacity and is rarely used. Unithiol was synthesized in 1950 by V. I. Petrunkin. The drug is highly soluble in water and low in toxicity, containing 29% of free SH-groups. In terms of its action, unithiol, like penicillamine, is a complexing compound. With many divalent and trivalent metals, it forms stable, dissociating complexes that are readily soluble in water and relatively quickly excreted from the body with urine. An experiment showed that the introduction of thiol compounds, which are donors of sulfhydryl groups, significantly reduces the synthesis of insoluble collagen. Based on this, A. A. Dubinsky proposed unithiol for the treatment of rheumatoid arthritis (1967) and systemic scleroderma (1969). His student P.P. Guida used unithiol with good effect in the treatment of patients with scleroderma, including the systemic form. A decrease in the peripheral zone, density and size of lesions and their faster regression with good tolerance of the drug by patients was noted. A distinct therapeutic effect in the treatment of various forms of scleroderma with unithiol (especially after repeated courses) was also noted by other dermatologists. Its vasodilatory and antispasmodic action was also noted. Sulfhydryl donors, which include penicillamine and unithiol, break intra- and intermolecular bonds, in particular, disulfide bonds, promote the depolymerization of pathological macroglobulins and increase the proportion of soluble collagen. They have a direct effect on collagen: they inhibit its synthesis, cause dissolution of newly formed collagen, have a direct inhibitory effect on immunocompetent lymphoid-plasmacytic cells, dissociate immunoglobulins, and inactivate humoral antibodies. Unithiol is administered intramuscularly as a 5% solution, 5 ml daily, up to 15-20 injections per course. Patients usually tolerate the drug well, but in some cases undesirable side effects occur. Sometimes nausea, dizziness, and general weakness occur immediately after intramuscular injection of unithiol. These rapidly occurring reactions also quickly pass (in 10-15 minutes) and do not require special therapeutic measures. When they appear, it is rational to temporarily reduce the dose of the drug and then gradually increase it to the original. Sometimes allergic reactions are also possible, which manifest themselves as widespread spotted or occasionally bullous rashes. They can occur at the end of the 1st course of treatment, after 10 injections. Most patients with allergic reactions caused by unithiol had a history of intolerance to certain antibiotics, vitamins, and sometimes even antihistamines. Unithiol did not cause anaphylactic shock in any patient. It should be noted thatthat the results of treating patients with plaque scleroderma with unithiol are comparable to the therapeutic effect of penicillamine treatment. Unithiol is also useful in the treatment of scleroatrophic lichen, which is the most resistant to therapy. At the same time, the tolerance of patients to unithiol and penicillamine is different. Unithiol is usually well tolerated by patients and does not have the undesirable effects that occur during treatment with penicillamine. In some cases, aminoquinoline derivatives are used in the treatment of patients with plaque scleroderma, given their moderate immunosuppressant and anti-inflammatory effects. Contraindications, treatment regimens and prevention of complications when prescribing aminoquinoline drugs do not differ from those described earlier (see treatment of atrophic forms of lichen planus).

In addition to D-penicillamine and unithiol, some scientists believe that diaminodiphenylsulfone (DDS) and its derivatives also have an antifibrotic effect. In addition to the anti-leprosy effect, drugs of this series are credited with immunosuppressive and anti-inflammatory effects. They have been used with good results in various dermatoses (Duhring's dermatosis herpetiformis, cicatricial pemphigoid, gangrene pyoderma, acne conglobata, etc.). The mechanism of action of DDS and its derivatives has not been fully elucidated. One of the drugs in this group, diucifon, has been successfully used to treat patients with systemic scleroderma. Diucifon is one of the DDS derivatives and, along with the sulfone group, also contains a pyrimidine compound, 6-methyluracil. It is prescribed 0.2; 0.5; 10; 12; 13; 14; 15; 16; 18; 19; 20; 21; 22; 23; 24; 25; 26; 28; 29; 30; 31; 32; 33; 34; 35; 36; 37; 38; 39; 40; 41; 42; 43; 44; 45; 46; 47; 48; 50; 51; 52; 53; 54; 55; 56; 57; 58; 60; 62; 63; 64; 65; 66; 68; 69; 70; 71; 61; 63; 65; 67; 69; 72; 63; 65; 67; 69; 73; 69; 74; 69; 75; Diucifon was synthesized in Russia as a replacement for DDS; it is better tolerated by patients and lacks many of the negative properties of DDS, in particular, it rarely causes changes in the blood formula.

Due to vascular damage and severe microcirculation disorders in scleroderma, in addition to drugs with antifibrotic action, vasodilators, disaggregants and angioprotectors are also prescribed in the complex treatment of patients. Phenigidine (corinfar, nifedipine), xanthinol nicotinate, nicergoline (sermion), pentoxifylline (trental), dipyridamole (curantil), low-molecular dextran, rheopolyglucin, prostaglandin E-1 (vasaprostan), etc. have proven themselves to be the most effective. Corinfar at a dose of 30-50 mg per day is usually well tolerated by patients. The headache and slight hypertension that arise disappear after reducing the dose of the drug. Xanthinol nicotinate can be used 1-2 tablets (0.15-0.3 g) 3 times a day or intramuscularly 2 ml 1-3 times a day.

In focal forms of scleroderma in stages 1-2 localized in the scalp, corticosteroids are used in the form of an ointment or cream. Subsequently, intrafocal administration of a crystalline suspension of triamcinolone is possible at 0.3-0.5 ml in a 2% lidocaine solution at a rate of 5-10 mg per 1 ml. For administration, use the thinnest needles and an insulin syringe. The density of the lesion makes it difficult to administer the suspension of corticosteroids. In the presence of a large lesion, it is recommended to initially administer corticosteroids into a small area of the affected skin, gradually expanding the injection zone as the previously compacted areas soften. Injections are repeated after 2-4 weeks. If atrophy develops at the sites of infiltration of the affected skin with a suspension of triamcinolone, further intrafocal administration of corticosteroids is stopped.

Physiotherapeutic procedures significantly accelerate the regression of lesions. When scleroderma is localized on the scalp, local and indirect physiotherapeutic effects are recommended. Ultratone, d'Arsonval, massage and lymphatic drainage can be prescribed to the affected area, including the collar zone. Amplipulse, diadynamic currents or inductothermy can be used on the cervical spine in the absence of contraindications. However, regression of plaque scleroderma always ends in skin atrophy, and on the scalp - persistent focal alopecia, which can be expressed to a greater or lesser extent. Patients must be warned about this in a timely manner. A real and achievable goal is to reduce the area of cicatricial baldness and its severity, as well as to prevent new areas of alopecia.

The effectiveness of the therapy is assessed by the results of comparing repeated copies-drawings of the contours of the alopecia focus on the scalp and by the degree of regression of the rash on the skin of the trunk and limbs. At the same time, it should be borne in mind that focal forms of scleroderma are characterized by spontaneous remissions.

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