Scleroderma of the scalp

Scleroderma rarely affects the skin of the scalp. Among its different forms, in this localization, linear scleroderma of the frontal-parietal region, systemic scleroderma, widespread plaque and small-scleroderma scleroderma, or sclerotriphytic lichen arise according to the degree of decrease. Dermatosis is more common in women, and its linear form is in children. On the scalp due to the peculiarities of the clinical manifestations of scleroderma and the presence of hair, the stages of the erythematous macula and the compacted plaque are not detected. The lesion is detected at the final stage of the disease, when focal atrophic alopecia is formed, or the condition of the pseudo-phelala. The surface of the lesion becomes smooth, shiny, soldered to the underlying tissues, completely devoid of hair.

With linear scleroderma of the frontal region, the lesion usually begins with the scalp, where it is represented by a vertical strip of atrophic cicatricial alopecia 1-3 cm wide, descending to the forehead, later on the back of the nose, sometimes on the upper lip. In shape and location, the atrophic scar is quite reminiscent of the footprint remaining after the impact of the saber. In some cases, the ribbon-like scleroderma of the frontal-parietal region is accompanied by the hemiatrophy of Romberg's face. In this case, all tissues (subcutaneous fatty tissue, muscles, cartilage and skull bones) are atrophied at the sites of the lesion near the eye, in the zygomatic area or in the region of the lower jaw. 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 less healthy, the skin on it is atrophic, dyschromic with numerous folds and furrows. On EEG in such patients on the side of the lesion, a diffuse irregular rhythm of brain waves can arise.

On the scalp, the lesion may be located in isolation or be one of many foci of common plaque scleroderma. Its foci are localized mainly on the trunk and extremities, rarely - in the forehead and scalp. Thus, Sayenko-Lyubarskaya VF (1955) of 36 patients with different forms of scleroderma, including systemic forms, only one patient had a lesion of the scalp and face. Guseva N.G. (1975) in 4 out of 200 patients with systemic scleroderma observed lesion of the skin of the scalp as a type of discoid lupus erythematosus, manifested mainly as foci of cicatricial atrophy with alopecia. These changes preceded the development or detection of systemic scleroderma. So, one of these patients at the age of 19 had a foci of baldness on the scalp and discoid lupus erythematosus was diagnosed. Six years later, the patient had two new similar foci on the scalp, and in the autumn of the same year - vasospastic phenomena on the hands, 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 on the scalp of the clinical manifestations of these diseases. This example confirms great difficulties in the diagnosis of isolated scleroderma of the scalp. The results of a histological examination of the affected skin can help to make the correct diagnosis.

Histopathology

Histopathological changes largely depend on the duration of the lesion. In the initial, edematous-inflammatory stage, the thorny layer of the epidermis is changed little, and there is a vacuolar degeneration of the cells of the basal, sometimes spiny layer. In the dermis, thickened and closely-fitting collagen fibers are noted, between which is a moderately expressed, predominantly lymphocytic infiltrate, the walls of the vessels are swollen. When involved in the process of the subcutaneous fat layer, its connective tissue septum thickens due to inflammatory infiltration and neoplasm of collagen fibers, which in places completely replace it. In the late, sclerotic stage inflammatory phenomena are poorly expressed, the epidermis is atrophic, the border between it and the dermis is represented as a straight line due to the absence of the papillary layer. Collagen fibers are sclerosed, compact, fibroblasts are few; infiltrate is absent, or persists in a small amount of perivascular. The walls of the vessels are thickened due to fibrosis, their lumens narrowed. Sebaceous glands and hair follicles are atrophied. Thinning and subcutaneous fatty tissue, which is partially replaced by a sclerotic collagen tissue.

Diagnosis of scleroderma of the scalp

Scleroderma of the scalp is differentiated with other dermatoses, which in this localization lead to focal atrophic alopecia - the condition of the pseudo-peloid. In addition to dermatoses, which, most often lead to a pseudo-peloid condition, one should also remember the sclerodermiform basalioma of the scalp, sclerodermiform manifestations that occur after exposure to some drugs and bone marrow transplantation. The metastasis of the cancer of internal organs in the skin of the scalp may also resemble the manifestations of scleroderma.

Sclerodermiform basalioma is one of the rare and unusual forms of it. It is usually localized on the skin of the forehead, but it can also affect the region of the temples, neck and scalp. It is a hearth, the size of a coin thickened plaque in the form of a sclerosed plate with a smooth, rarely - peeling surface, a yellowish waxy color with distinct telangiectasias that penetrate its surface. On the scalp, the cicatricial altered surface of the sclerodermoform basal cell is devoid of hair and can stand a little above the uninjured skin surface. Unlike other flat basal forms, with a sclerodermiform its variety there is no characteristic peripheral cushion and there is no ulcerous decay. It is prone to prolonged slow peripheral growth. Histological examination allows verifying the diagnosis. Among the powerfully developed stroma, often sclerotized and hyalineized, thin strands and complexes consisting of compactly located shallow dark cells are seen. The general picture of the lesion resembles a scirrhous cancer of the stomach or breast.

Scleroderm-like manifestations in the skin are described as a characteristic side effect when treated with an antineoplastic antibiotic bleomycin. Against the background of its use in patients appear scleroderm-like nodules and plaques, sometimes - widespread densification of the skin. Often, induration develops on the hands, which can lead to necrosis of the fingers, as in the acrosclerotic form of scleroderma. A few months after the withdrawal of the drug, the disease usually regresses.

Injections of opioid analgesic pentazocine can cause localized or generalized skin sclerosis in alcoholics and drug addicts. In some cases, fibrosis of the skin and muscles can be combined with calcification of subcutaneous fat and muscle tissue, and ulceration in the lesions is sometimes also formed. Laboratory indicators (other than an increase in ESR) usually do not change.

In the late phase of the chronic "graft-versus-host disease", which occurs in some patients after allogeneic bone marrow transplantation, generalized sclerotic and atrophic lichen or scleroderm-like skin changes develop. In patients with common scleroderm-like manifestations on the skin, induced by drugs or bone marrow transplantation, lesions can probably be located on the scalp.

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

Scleroatrophic lichen of the scalp

Most authors consider primary sclerosing and atrophic lichen to a kind of small-scleroderma scleroderma (syn: scleroderma teardrop, white spot disease, sclerotic lichen, or Tsumbusha white diarrhea). It affects mainly women, can be accompanied by typical plaques of scleroderma and is usually localized on the neck, upper chest, flexor surface of the extremities, abdomen, genital organs, less often elsewhere. In the literature there are separate reports on the defeat of sclerotrophic lichen, in addition to the beloved sites, and the scalp with the formation of cicatricial atrophic alopecia. Ruk A. And Dauber R. (1985) consider that sclerotrophic lichen of the scalp is rare. In the domestic literature, we could not find a description of the pseudo-peloid caused by this dermatosis. In recent years, we have observed two elderly women with an unobtrusive melkochagovym atrophic alopecia and a common, long-lasting recurrent scleroatrophic lichen on the trunk, extremities, anogenital region. In these patients on the scalp, the plaques are small, atrophic skin changes with hair thinning, not accompanied by any subjective sensations. A careful examination in the fronto-parietal region revealed small (3-4 mm in diameter oval areas of the skin without hair and estuaries of hair follicles with a white and smooth surface, they did not have a distinct border, were at the level of the surrounding skin and smoothly passed into it. Palpation of these areas, the skin "wrinkled" somewhat more than the one located nearby, there was no follicular keratosis in these foci .. In patients with sclerotrophic lichen, no large atrophic plaques were found on the scalp, which they would have whether on the skin of the trunk, extremities, or in the genital area.There has not been a histological study of lesions on the skin of the scalp in patients with sclerotrophic deprivation, so there is no convincing evidence of a single genesis of skin lesions on the trunk and on the scalp, and it is impossible to exclude the possibility of such scalp changes In elderly women with long-standing androgenic alopecia, it is possible, with a purposeful study of patients with sclerotrophic lichen, to be reliably proven the pseudo-peloid caused by this dermatosis.

Treatment of patients with pseudocolel caused by scleroderma

Treatment of patients in whom the condition of the pseudo-pellet is caused by isolated plaque scleroderma of the scalp or is one of the manifestations of a common or systemic form of the disease is directed at known links in the pathogenesis of scleroderma. Therapy is based on inhibition of enhanced biosynthesis of abnormal collagen fibers, normalization of microcirculation in lesion sites and reduction of autoimmune shifts. It is important to exclude or weaken the influence of the factors that provoke the development or progression of the disease in patients and cause in some cases a scleroderm-like syndrome very similar to the manifestations of scleroderma (silicon dioxide, polyvinyl chloride, trichlorethylene, hexachloroethane, benzene, toluene, xylene, artificial resins, oil, diesel oil , paraffin, silicone, contaminated vegetable oil - denatured rapeseed oil, etc.). Therefore, the effects of certain drugs (bleomycin, pentazocine), vaccines, serums, ultraviolet radiation and penetrating radiation, hypothermia, mechanical trauma, hormonal disorders, sanitation of foci of infection should also be avoided. Plaque scleroderma after months and years can be transformed into a systemic form of disease. In this connection, every time a doctor visits a patient with active foci of plaque scleroderma, a clinical and immunological examination should be performed to exclude the systemic form. The main criteria for distinguishing the systemic and focal forms of scleroderma are vasospastic changes in the distal parts of the extremities, proceeding according to the type of Raynaud's syndrome, the defeat of the musculoskeletal system and internal organs, as well as the characteristic immunological disorders. In an objective study of patients with scleroderma, the dermatologist evaluates the nature and extent of skin lesions, paying special attention to the patient's hands and face. A typical skin change retains the leading diagnostic value among other clinical manifestations of systemic scleroderma and is the main one in the diagnosis of its focal forms. The predominant localization of skin changes in systemic scleroderma are the hands, forearms and face. When the disease spreads, the skin of the chest, back (the "corset" of the "shell"), and sometimes the entire surface of the trunk and extremities, is also affected. In addition to dense edema, induration and atrophy of the skin, focal hyperpigmentation and multiple telangiectasias on the face, neck, chest and extremities are also of diagnostic importance. Systemic scleroderma is characterized by a dark blue color of the nail bed on the fingers of the hands (rarely - and stop); The nails are reduced in size and flattened, the cuticles of the nails are expanded with fringe ("frayed") distal margins, sometimes with telangiectasias (like in lupus erythematosus and dermatomyositis). Nail plates bent clawlike, on the tips of the fingers may be minor painful ulceration (partially under crusts) or scars, fingers of the hands shortened and sharpened due to lysis of part of the terminal phalanges, their skin is compacted, characteristically clawed their position. The face of patients with systemic scleroderma is amygous and gives the impression of a mask. The facial skin is stretched, compacted, waxy in color, sometimes pigmented, with telangiectasias. The nose is pointed, the oral opening is narrowed, the red border of the lips is thinned, atrophic, pale, the radial folds ("kisetoobrazny" mouth) form around the mouth, the tongue becomes rigid, shortens, its bridle thickened, sclerotized. On the scalp, the atrophic process is manifested by diffuse, less often - focal hair loss, "pseudo-pellet condition".

Unlike systemic scleroderma, focal forms of the disease almost never affect the brush. The exception is the band-shaped scleroderma, in which the lesion of the skin can be located along one limb, spreading sometimes to its distal sections. The study of the vasomotor reflex on the fingers of the hands in patients with scleroderma revealed an early disturbance of microcirculation in the systemic form of the disease, which leads to a slow restoration of the initial temperature in the finger after its dosed cooling. This does not occur in patients with focal scleroderma, except for striated scleroderma of the extremities, when a microcirculation disturbance is found only on the affected arm. In addition to an objective study of the patient with a dermatologist, consultations of a therapist, neurologist and ophthalmologist are also necessary (the last two specialists are especially important for patients with localization of lesions on the scalp). Investigate the organs that are most often affected by systemic scleroderma. For the detection of lung pathology, an x-ray of the chest, esophagus - fluoroscopy with barium in the lying position, cardiac ECG and echocardiography, kidneys - Reberg samples, fluctuations in creatinine, urea, etc. Are prescribed. Lack of changes on chest radiographs (diffuse pneumosclerosis with bronchiectasises and cysts in the lower lobes of the lungs - "cellular lungs", adhesions, fibrosis of the pleura, pulmonary heart), the normal patency of the barium lump in the esophagus without sluggish, delayed peristalsis, segmental extensions, protrusions and narrowing in its lower third, the absence of ECG and echocardiography data for myocarditis, myocardiosclerosis, hypertrophy and dilatation of the right ventricle of the heart, normal creatinine clearance, and renal function - allow to exclude systemic damage in scleroderma. Routine laboratory studies in the onset of systemic scleroderma are less informative. In the clinical analysis of blood, attention is drawn to the increase in ESR, in the proteinogram - to hyperproteinemia and hypergammaglobulinemia, in urine analysis to proteinuria and changes in sediment (cylinders, leached red blood cells). The titres of antinuclear antibodies, antibodies against cytoplasmic RNA and collagen, rheumatoid factor, etc. Are investigated. Such examination and treatment of a patient with scleroderma is preferably performed in a hospital setting.

In the active stage of focal scleroderma, intramuscular injections of water-soluble penicillin (sodium salt of benzylpenicillin) daily 2,000,000-3,000,000 units are prescribed for 2-3 weeks. What is the reason for the therapeutic effect of penicillin in scleroderma - is not known exactly. There is an opinion that penicillin is partially transformed in the body into D-penicillamine, which determines its effectiveness. In some cases, foci on the extremities, like scleroderma, but with a more pronounced inflammatory response, are a manifestation of borreliosis, where the effectiveness of penicillin is well known. Simultaneously penicillin is a strong allergen and can cause allergic reactions both immediate (more often) and delayed type. The most frequent allergic reactions of the immediate type include urticaria, Quincke edema, bronchial asthma, and occasionally anaphylactic shock can develop. Therefore, before the appointment of this antibiotic in patients, determine its tolerability in previous applications. Contraindications to the appointment of penicillin are allergic diseases in the anamnesis (bronchial asthma, urticaria, atopic dermatitis, hay fever), as well as hypersensitivity and unusual reactions when using antibiotic cephalosporin series or griseofulvin. Particular caution is needed when administering intramuscular penicillin injections to patients with intolerance to a number of other drugs and women with long-term foci of infection (trophic ulcers of the shins, chronic tonsillitis, maxillary sinusitis, frontal sinusitis, odontogenic osteomyelitis, chronic adnexitis, etc.) because of the risk of anaphylactic shock. If penicillin is well tolerated and effective, patients with focal scleroderma should make preventive treatment courses 2 times a year (in spring and in autumn).

If penicillin is ineffective or if there are contraindications to its use, D-penicillamine (custenyl, artamine, melkaptil, bianodine) can be used. In addition, D-penicillamine has the ability to suppress collagen synthesis, to depolymerize macroglobulin complexes, to split the cross bonds between the newly synthesized protolagene molecules, and is an antagonist. It is a complex-forming compound that binds and accelerates the excretion of copper, mercury, arsenic, lead, zinc ions from the body. Pyridoxine.

Contraindications to the appointment of penicillamine are hypersensitivity in the anamnesis to penicillamine or penicillin pregnancy and lactation. It is advisable to avoid its appointment to persons with intolerance to antibiotics of cephalosporin series and griseofulvin, with impaired liver function, pancreatitis, stomach ulcer, anemia, leukopenia, polyneuritis, alcohol abusers. Before the appointment of penicillamine, examine the hemogram, transaminases, the level of creatinine in the blood. The drug is prescribed on an empty stomach 1 hour before meals or 2 hours after eating, without combining with the reception of other medications. In focal scleroderma there is usually no need to prescribe high daily dosages 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 (more than 1 g per day), used in the treatment of systemic scleroderma, in about 1/3 of patients cause side effects, which leads to its forced cancellation. During the treatment for the patient, medical control is necessary: once in 2 weeks, a clinical blood test (decrease in the number of platelets, hemoglobin, later - red blood cells and leukocytes) and urine, once a month, monitor liver function (transaminases, bilirubin, creatinine, gamma- glutamyl-transferase). With good tolerability of penicillamine, control studies are performed every 3-6 months. A slow increase in the dose reduces the incidence of certain adverse reactions and improves its tolerability. During treatment, nausea, anorexia, vomiting, glossitis, aphthous stomatitis, loss of taste sensations or their distortion, reversible polyneuritis (due to a deficiency of vitamin B6) are possible; rarely on the background of treatment there was diarrhea, hepatitis, intrahepatic cholestasis, nephritis, fever, toxemia, induced lupus erythematosus syndrome; possible anemia, thrombocytopenia, leukopenia, agranulocytosis, eosinophilia, proteinuria, etc.

In the arsenal of drugs that have a therapeutic effect in patients with scleroderma, also includes unitiol, which in this capacity is known little and rarely used. Uniothiol was synthesized in 1950 by VI Petrunkin. The drug is highly soluble in water and is slightly toxic, contains 29% of free SH-groups. According to the action, unitiol, like penicillamine, refers to complexing compounds. With many divalent and trivalent metals, it forms persistent, dissociating complexes that are easily soluble in water and relatively quickly eliminated from the body with urine. In the experiment it was shown that the introduction of thiol compounds related to donators of sulfhydryl groups significantly reduces the synthesis of insoluble collagen. Proceeding from this, Dubinsky AA suggested unitiol for the treatment of rheumatoid arthritis (1967) and systemic scleroderma (1969). His pupil Guida P.P. With good effect used unitiol in the treatment of patients with scleroderma, including the systemic form. There was a decrease in the peripheral zone, density and size of the foci and their faster regression with a high tolerance of the drug to patients. A distinct therapeutic effect in the treatment with Unithiol of different forms of scleroderma (especially after repeated courses) was noted by other dermatologists. His vasodilating and spasmolytic effect was also noted. Donators of sulfhydryl groups, such as penicillamine and unitiol, rupturing intra- and intermolecular bonds, in particular, disulfide bonds, contribute to the depolymerization of pathological macroglobulins and increase the fraction of soluble collagen. They have a direct effect on collagen: inhibit its synthesis, cause dissolution of newly formed collagen, exert a direct inhibitory effect on immunocompetent lymphoid-plasmocyte cells, dissociate immunoglobulins, inactivate humoral antibodies. Uniotiol is administered intramuscularly in the form of a 5% solution of 5 ml daily to 15-20 injections per course. Usually patients tolerate the drug well, but in some cases, undesirable side effects occur. Sometimes immediately after intramuscular injection unitiol occurs nausea, dizziness, general weakness. These rapidly occurring reactions also quickly pass (in 10-15 minutes) and do not require special therapeutic measures. When they appear rationally reduce the dose of the drug and then gradually increase it to the original. Sometimes allergic reactions are possible, which are manifested by widespread spotted or occasionally bullous eruptions. They can occur at the end of 1 course of treatment, after 10 injections. In the majority of patients with allergic reactions caused by Uniotiol, there was a history of intolerance to certain antibiotics, vitamins, and sometimes antihistamines. None of the uniothiol patients caused anaphylactic shock. It should be noted that the results of treatment with patients with plaque scleroderma with unithiol are comparable with the therapeutic effect of treatment with penicillamine. Uniotiol is useful in the treatment of sclerotrophic lichen, which is the most resistant to therapy. At the same time, the tolerability of patients with unithiol and penicillamine is different. Uniothiol is usually well tolerated by patients and does not have those undesirable effects that occur when treated with penicillamine. In some cases, in the treatment of patients with plaque scleroderma, aminoquinoline derivatives are used, given their moderate immunosuppressive and anti-inflammatory effect. Contraindications, treatment regimens and prevention of complications when prescribing aminoquinoline preparations do not differ from those described earlier (see treatment of atrophic forms of red flat lichen).

In addition to D-penicillamine and unithiol, according to some scientists, diaminodiphenylsulfone (DDS) and its derivatives are antifibrotic. In addition to anti-leukemia, the drugs of this series are attributed to immunosuppressive and anti-inflammatory effects. They were used for various dermatoses with good effect (herpetiform dermatosis of Dühring, cicatricial pemphigoid, gangrenous pyoderma, conglobata acne, etc.). Finally, the mechanism of action of DDS and its derivatives is not clear. One of the drugs of this group - diucifon was successfully used in the treatment of patients with systemic scleroderma. Dyuziphone is one of the derivatives of DDS and along with the sulfonic group also contains pyrimidine compound - 6-methyluracil. It is assigned to 0.2; once a day (0.01 g / kg body weight). Dyuzifon was synthesized in Russia as a replacement for DDS; it is better tolerated by patients and devoid of many negative properties of DDS, in particular, rarely causes a change in the blood formula.

In connection with the defeat of blood vessels and severe microcirculation disorders in scleroderma in the complex treatment of patients in addition to drugs with antifibrotic effect, prescribe vasodilating agents, disaggregants and angioprotectors. The most justified itself is phenygidine (corinfar, nifedipine), xanthinal nicotinate, nicergoline (sermion), pentoxifylline (trental), dipyridamole (curantyl), low molecular weight dextran, rheopolyglucin, prostaglandin E-1 (vasaprostan), etc. Corinfar in a dose of 30-50 mg per day is usually well tolerated by patients. The resulting headache and slight hypertension passes after a reduction in the dose of the drug. Xanthinal nicotinate can be used for 1-2 tablets (0.15-0.3 g) 3 ra per day or intramuscularly 2 ml 1-3 times a day.

In focal forms of scleroderma in the 1-2 stage with localization in the scalp, corticosteroids are used in the form of an ointment or cream. In the future it is possible to intracutaneously introduce a crystalline suspension of triamcinolone in 0,3-0,5 ml in a 2% solution of lidocaine from the calculation of 5-10 mg per 1 ml. For the introduction, use as thin a needle and an insulin syringe. The density of the focus makes it difficult to administer a suspension of corticosteroids. In the presence of a large focus, it is recommended first to inject corticosteroids into a small area of the affected skin, gradually widening the zone of drug administration as the previously softened areas are softened. Injections are repeated after 2-4 weeks. With the development of atrophy at the sites of infiltration of the affected skin by cyclic triamcinolone, further intraocular administration of corticosteroids ceases.

Physiotherapeutic procedures significantly accelerate the regression of lesions. When localizing scleroderma on the scalp, local and indirect physiotherapeutic effects are recommended. In the affected area, you can appoint an ultrasound, D'Arsonval, to carry out massage and lymphatic drainage, including the collar zone. In the cervical spine, in the absence of contraindications, you can apply amplipulse, diadynamic currents, or inductothermy. However, regression of the focus of plaque scleroderma always ends with skin atrophy, and on the scalp with persistent alopecia areata, which can be expressed to a greater or lesser degree. It is necessary to warn patients in a timely manner. The real and achievable goal is to reduce the area of cicatrical alopecia and its severity, as well as prevent new areas of alopecia.

The effectiveness of the therapy is evaluated by comparing the duplicate images of the contours of the alopecia areata on the scalp and the degree of regression of the rashes on the skin of the trunk and extremities. At the same time, it should be borne in mind that for focal forms of scleroderma, spontaneous remissions are characteristic.

[1], [2], [3], [4], [5], [6], [7], [8], [9]