Scleroderma

SCLERODERMA (sclerodermia, seleroderma, sclerema, dermato-sclerosis, hardening of the skin), a chronic or subacute disease of the skin, characterized by a peculiar woody thickening and hardening of the connective tissue elements of the skin and subcutaneous tissue, as well as underlying tissues, which in the further course of the disease leads to atrophy or ends with complete resolution and recovery. Two forms are distinguished: a) generalized diffuse, or diffuse, and b) limited, also known under the name morphoea (E. Wilson). The first form in turn is usually subdivided into widespread and progressive symmetrical S. a) Generalized diffuse S. In the development and course of the disease, 3 stages are distinguished: stage of edema (stadium oedematosum), stage of induration, or sclerosis (stadium indurativum), and stage of atrophy (stadium atrophicum). In the initial period, S. is manifested by the appearance in the skin of a slight edema of doughy consistency, extending into the subcutaneous tissue, and hyperemia. In other cases, the development of S. is often preceded by various and highly variable in character and course prodromal symptoms, which in essence do not present anything characteristic. Often shortly before the development of scleroderma changes, angioneurotic phenomena appear in the form of rapidly transient erythema on the extremities, accompanied by local asphyxia. From the very beginning of its appearance, the edema acquires a bright red color, as in erysipelas, or a bluish tint. In other cases, the color of the skin at the site of edema is normal. The affected skin appears smooth, shiny, and tense due to the deep spread of edema into the skin itself and subcutaneous tissue. When pressing with a finger on affected areas, a pit usually does not remain. Following the edema, which lasts for several weeks or quickly passes into the next stage of sclerosis or induration, the typical clinical picture of S. develops. The skin acquires the consistency of wood or cartilage. Apparently, the phenomena of sclerosis, i.e., an increase in connective tissue, already exist in the first stage. The normal skin pattern disappears. On palpation of the affected focus, a dense infiltrate with indistinct or sharp borders is noted. The skin over it does not gather into a fold, is unyielding, does not shift from the underlying tissue, is cold to the touch, stiffened, "like a frozen corpse," and hard as a board. When pressed with a fingernail or probe, a depressed line that does not disappear for a long time is formed. The surface of the sclerosed foci becomes shiny, smooth, hard, and rough. At the same time, significant changes occur in the color, which appears either dull or brilliantly white, waxy-yellow, or marble-like, rarely reddish-brown. The affected foci lie either at the level of the surrounding skin or rise sharply above it. If the disease affects the skin over the joints, voluntary movements encounter an obstacle, and in severe cases, complete extension of the corresponding joints becomes impossible (ankylosis). In the later atrophic stage, the skin may be so stretched over bony prominences that it leads to its ulceration. In its further development, the process can spread to the mucous membranes (mouth, nasopharynx, larynx, esophagus, vagina) and extend to the fasciae, periosteum, muscular and bone systems, and sometimes even to internal organs (cirrhoses), resulting in various severe functional disorders. When the face is affected by the disease, its corpse-like immobility is strikingly conspicuous. With localization on the face, facial expression disappears, wrinkles and skin folds are smoothed out, as a result of which the face acquires a mask-like appearance (masque sclerodermique). Disease of the larynx, soft palate, and tongue causes disturbance of speech (quiet voice, slow speech), and with diffuse sclerotic induration of the chest wall, respiratory movements are also limited. Patients eventually die from general exhaustion or some intercurrent disease. In the case of progressive symmetrical S., the process develops slowly, with the first manifestations most often appearing on the extremities, mainly on the hands, and then showing an irresistible tendency to further spread, involving the face and over several months or years affecting the entire body. With general spread of the process throughout the trunk (sclerema universalis) and extremities, the entire human body is surrounded by hard skin, like a shell, which gives the patient a peculiar appearance of a "walking statue" (homme moraie). Usually the induration passes into atrophy with a sharp shortening and tension of the skin. As the sclerosis disappears, the skin gradually thins and pales, turning into parchment-like atrophied skin of scar-white color, often dotted with more or less abundant pigment spots. At the same time, muscles and fatty tissue atrophy, and the skin fuses with the underlying tissues. In places where the skin directly adheres to bones or is fused with joints, bedsores and ulcers easily form under the influence of traumatic injuries or strokes. S. is often combined with progressive idiopathic atrophoderma. The atrophied skin remaining after the resolution of the indurative process resembles crumpled cigarette paper (a characteristic sign of idiopathic skin atrophy). According to Meshchersky, S., beginning with a spot, and idiopathic atrophy are pathogenetically the same dermatosis. Diffuse progressive S. is often combined with the so-called sclerodactyly (sclero-dactylia), which usually begins with local asphyxia of the extremities and local cyanosis and edematous swelling of the fingers, accompanied by various prodromal phenomena (rheumatoid symptoms, paresthesias, neuralgias, etc.). In case of hand involvement, it becomes cold to the touch, rigid, and hard, taking on a bluish-red color. The fingers are spindle-shaped and pointed, tightly stretched by sclerosed skin, and are in a semi-flexed position. After several years, the skin becomes thin, immobile, as if glued to the fingers of the hands and feet (Wolters), the bluish color disappears, giving way to a yellowish-white. The thinned skin is easily ulcerated; sometimes actual necrosis (gangrene) occurs. The process can lead to shortening of the phalanges, atrophy, and complete resorption of their bony substance without necrosis. The nails are thickened in a ridge-like manner and are covered with grooves or become brittle and fragile and thin. The cessation of nail growth and their almost complete disappearance, along with the shortening of the skin and atrophy of the bones, lead to various degrees of disfigurement of the fingers of the hands and feet. Hair sometimes turns gray and falls out. Sebaceous and sweat secretion, especially in the atrophic stage, are reduced. Sensitivity is usually normal, sometimes deviations toward reduction are noted. The temperature at the sites of scleroderma foci is reduced by 1-2°. Path-histological studies in S. give a varied picture, depending on the stage of development of the process at which the biopsy was performed. In the stage of edema, an inflammatory infiltrate develops around slightly dilated blood vessels. Subsequently, the collagen connective tissue fibers swell and thicken (intrafibrillar edema). The elastic fibers remain and show no signs of degeneration, and with prolonged existence of the process they appear sparse or in fragments. In the sclerotic stage, there is increased proliferation of connective tissue, the elements of which, densely packed into one mass, transform it into dense sclerosed tissue, infiltration of connective tissue fibers, and at the same time changes in the walls of small-caliber vessels, namely, endo- and mesoarteritis with subsequent narrowing and obliteration of the vessel lumen. The terminal stage shows gradual wrinkling of the swollen collagen masses with subsequent formation of scar tissue. The papillary and subpapillary layers and subcutaneous tissue almost completely disappear. The epidermis and sweat glands atrophy. In the skin, mainly near blood vessels, abundant accumulation of pigment in the form of clumps is found. Lymphatic spaces are stretched in places. In the subcutaneous tissue, muscles, bones, and in some internal organs, similar sclerotic changes are often found. In differential diagnosis, S. can sometimes be confused with leprosy and syringomyelia. But the disturbance of skin sensitivity in S. never reaches the degree of complete loss. Limited, or partial, S. (sclerodermia circumscripta, s. partialis). The sclerotic changes may be limited exclusively to the involvement of small areas of skin in the form of clearly defined round, oval, or irregularly shaped plaques (s. en plaques) [see separate table (art. 407-408), fig. 3] with convex or wavy edges, called morphoea, or bands and cords (s. en bandes), located over a greater or lesser extent, thin strips (s. striata) or in the form of rings, encompassing a finger or an entire extremity.

Localized S. en plaques is characterized by the appearance on separate areas of skin thickening in the form of single or numerous limited, slightly raised, hard-as-cardboard disks, aptly compared to pieces of hard skin embedded in the thickness of the skin, ranging in size from 1 to 20 cm and larger, of pale-red or violet-red color. They are usually located asymmetrically and show a tendency to slow progressive growth and increase. After several months or years, the plaques begin to fade in the center, taking on a yellowish-white coloration, and undergo regression and may even disappear without a trace, but more often they transition to a state of atrophy. Often on such areas, a disturbance of pigmentation can be detected in the form of either solid pigmentation or individual densely pigmented spots, sometimes surprisingly alternating with normally colored areas, resembling a chessboard (Meshchersky). Pigmentation from affected areas can also spread to healthy parts. Sometimes on the sites of limited plaques with a smooth, waxy, and shiny surface, lamellar scaling forms.--In many cases, the affected foci are bordered by a peculiar bluish or lilac ring of dilated capillaries (lilac ring of Anglo-American dermatologists), imperceptibly transitioning into normal skin; this ring usually accompanies the spread of the process and disappears with its regression. This is a very characteristic sign indicating further spread of the lesion. Superficial vascular ectasias often appear. In short, localized S. exhibits all the characteristic features inherent in widespread and progressive-symmetrical S. (preliminary edema, woody hardening, thickening and infiltration of the skin and the final outcome in atrophy). Sometimes sharply protruding above the level of the surrounding skin, isolated disks show a great resemblance to keloids. On areas subject to constant friction and irritation, stubbornly healing ulcers with very sluggish course may form. J The localized form of S. has no definite localization. It can appear on any area of the skin cover, often on the face, neck, extensor surfaces of the upper limbs, as well as on the chest and abdomen. Even the mucous membrane of the oral cavity can be affected by S., taking the form of white, hard, and tightened spots. When the process is located on the hairy scalp, it often leads to hair loss. Sweating and sensitivity in the affected foci may be preserved, however in a number of cases sweating is absent and sensitivity is reduced depending on the degree of sclerosing. Sometimes moderate itching, slight burning, tingling, and a sensation of crawling ants are noted. The following forms of localized S. are distinguished. 1. Band-like, or ribbon-like, S. (sclerodermia en bandes). Sclerotic bands with irregular sometimes outlines, 2 to 5 cm wide, are either flat or convex and rising above the level of the skin or depressed in the form of a groove. They are often observed on the limbs, especially in children, and can sometimes hinder movement and cause sharp pain. In some cases, the arrangement of bands is observed in the form of a ring, as if encircling the limb (s. annularis). In the band-like form, the color of the bands resembles old ivory. The purple border around them is relatively weakly expressed. Sometimes the lesion penetrates deep, involving not only the skin but also the fasciae and muscles. 2. Superficial limited S. (sclerodermia circumscripta superficialis en plaques). A relatively rare form of S., characterized by the rapid formation of sclerotic atrophy. In these cases, the transition from inflammatory infiltration to skin atrophy occurs imperceptibly. The localized form of S. has a benign course, although the process sometimes drags on for many years. Only in rare cases does diffuse universal S. develop from limited plaques by the spread and fusion of individual elements. Etiology of S. is not yet fully clarified. S. is a disease of middle age, although it also occurs in children; women are more predisposed to the disease than men. As possible predisposing factors for the development of S., many authors cite sharp changes in temperature (cold, rapid cooling, frostbite), trauma, various acute infectious diseases (rheumatism, diphtheria, typhoid, erysipelas, pneumonia, scarlet fever), chronic infectious diseases (tuberculosis, syphilis, malaria), lead poisoning, alcoholism, severe nervous shocks or excitement, overwork, etc. In a number of cases of localized S., it may be a matter of edemas of the most diverse, often mechanical origin, which subsequently underwent sclerotic induration. Observations by Spiegler and others speak in favor of this, where S. was strictly localized between the edge of a stiff hat and the edge of a collar on a shirt or exactly corresponded to the boundaries of a hunting boot or glove. The appearance of the disease on the chest in women was often attributed to irritation caused by a corset.--Based on sharp changes in the innervation of blood- (587 б»8) vessel vessels, a number of authors are inclined to consider S. as an angiotrophoneurosis. According to Audry and Chatellier, S. is a lesion of the skin in which syphilis plays a prominent role. They call it an endocrinide, in particular an angiotrophoneurotic endocrinide of syphilitic origin. The most plausible and scientifically substantiated hypothesis is that of endocrine-vegetative disorders, which is confirmed by clinical observations, patho-anatomical data and a number of facts, namely: 1) the frequent coincidence of S. with various organic changes in the endocrine glands, 2) the almost constant existence of clinical signs of imbalance of the endocrine apparatus and parasympathetic hypertension (Hofmann) in S., 3) the successful treatment of S. with organotherapeutic means, 4) the favorable influence in some cases of S. on specific therapy, mainly when combined with luetic infection, and 5) the beneficial effect of X-ray irradiation of the thyroid and thymus glands (in irritating doses) in some cases of S.--The prognosis for significantly widespread forms of S. quoad vitam is not always favorable due to frequent complications. Nevertheless, complete recovery can occur even with the long existence of the disease. A less favorable prognosis is given for sclerodactyly, which is distinguished by its extraordinary stubbornness and progressive course.--Treatment consists in the long and persistent application of appropriate or pluriglandular organotherapy depending on the dysfunction of the endocrine apparatus and salicylic acid or its derivatives. Antisyphilitic treatment should be carried out only in the presence of luetic infection. It is extremely useful to prescribe resolving agents (heat, hot, sulfur, saline, mud baths, systematic massage). Sometimes diathermy gives a good effect. Some authors recommend the use of X-rays and a quartz lamp. For localized S., constant or faradic currents should be tried, iontophoresis with potassium iodide solution by Burgignon's method, bipolar electrolysis according to Brocq. Scleroedema adultorum (Busch-ke) [Sclereme aponevrotique benin (Audry et Gadrat), benign scleredema of adults (Nobl), edematous scleroderma of Hardy]. S. is expressed in the development of a completely painless dense edematous infiltration, involving not only the deep layers of the proper skin and subcutaneous tissue, but also often extending to the fasciae and musculature (Busch-ke, Beck, Sellei). It usually begins with the occiput and neck and quickly spreads to the face, back, chest, abdomen, and limbs, while the fingers of the hands and feet, as a rule, remain free from lesions or appear painful and cyanotic. Sometimes the process is limited to small areas of skin. Initially, the sclerotic edema of the skin has a lilac-rose coloration, and as it further develops and transitions to atrophic edematous sclerosis, it acquires a waxy tint and becomes exactly impregnated with paraffin. When pressed, the finger does not leave a dent. It is impossible to pinch the skin into a fold. Individual parts of the limb appear uniformly thickened and have the appearance of smooth cylinders. All skin folds and muscle outlines are smoothed out. S. not only causes a sensation of tension and rigidity but also significantly limits the movements of the eyelids, lips, and forehead skin. The face seems as if petrified (mask-like face of French authors). The tight mobility of the neck and chest cavity hinders chewing, swallowing, speech, and in rare cases even breathing. The mucous membranes are also sometimes involved in the process. With scarifications or pricks, no fluid is excreted from the affected tissue. Usually pigmentation is absent; hair is preserved in the affected area; sensitivity is not impaired. No disorders of the sweat and sebaceous glands can be noted. Patients always complain of chilliness and a constant sensation of cold.

Darrieu saw on the prominent parts of the extremities bands of warty hyperkeratosis. In the blood, a lymphocytosis is sometimes noted. Histologically, the most significant changes come down to a significant loosening of the deep layers of the cutis and subcutis, to the spreading and edematous swelling of the collagen bundles with the formation of slits and empty cavities around the vessels and epithelial appendages. The elastica is unchanged. The disease usually affects adults in young years and youths, predominantly the female sex, and more rarely occurs in childhood. It usually develops acutely over several days or weeks and continues for months or even years. Despite the protracted nature, the condition has a benign course. Gradual recovery occurs (usually within less than a year). An acute form has also been described, leading to a fatal outcome within several weeks or months. Often, prodromal phenomena in the form of joint diseases, fever, and secretory disorders precede the onset of the disease. -Etiology is unknown. The disease often appears after a severe injury, emotional experiences, moral shock, strong excitement, or fright. Acute infections (influenza, scarlet fever, nephritis, whooping cough, mumps, sore throat, pneumonia, encephalitis, pernicious anemia, etc.) often precede it. Many authors express the hypothesis of a connection between the disease and the endocrine glands and the autonomic nervous system. Darrieu is inclined to associate this condition with myxedema. -Treatment: bed rest, salicylate preparations, warmth, massage with 1-2% salicylic ointment; fibrinolysin, injections of 10% sodium chloride (Sellei), hormone therapy. Scleroedema neonatorum (oedema neonatorum, scleredema of newborns). The disease develops soon after birth, no later than the 6th day of life, but most often in a retarded and premature child, very rarely in mature children, due to cardiac dysfunction. It begins in most cases with the lower extremities, the area of the feet and calves, and gradually spreads upward in the form of an extremely soft, edematous swelling of the skin and subcutaneous tissue with induration. The parts of the body affected by edema appear pale, cold to the touch, and have a dusky color. When pressed with a finger, a deep pit remains on the skin, which slowly disappears. Rarely does the edema spread to the trunk, upper extremities, and face. Heart activity and respiration are usually slowed. Urination is decreased. Body temperature falls below normal. When the face is affected, food intake is difficult and the ability to suck is lost. The child's general condition is very severe. Gradually, death occurs with signs of cyanosis and relentlessly increasing weakness. Scleredema of newborns in many respects bears great resemblance to sclerema. The two forms differ from each other not so much in their course as in the fact that in edematous sclerema there is a pronounced edema of the subcutaneous tissue, as a result of which the skin here is more tense, immobile, and more shiny and takes on a pale-yellowish or marbled color. Sometimes the affected skin is covered with ecchymoses (purpura, disseminated gangrene according to Demme). Soltmann points to complications with pneumonia, jaundice, pemphigus, erysipelas, furunculosis, polyarthritis, peritonitis, umbilical arteritis. In two cases, he saw the development of scleredema in children with congenital syphilis. The cause is unknown. Some express the hypothesis that the disease owes its origin to congenital general weakness or depends on a disorder of the circulation, resp. respiratory apparatus, and the development of these phenomena is favored by incomplete, not yet completed tissue development and underdevelopment of the vessels. L. Dumas considers the disease as analogous to phlegmasia alba dolens and reports a case with bilateral femoral thrombosis. The hypothesis of abnormal permeability of the vascular walls hardly deserves attention. - Path-anatomically: swelling and edema of connective tissue, hardening of the skin and subcutaneous fat, massive edema of the subcutaneous fatty tissue, which on section appears yellowish or brownish, loose or granular, according to Bednar, sometimes turns into yellow-brown dense masses. During the incision, abundant transparent serous fluid escapes, partly mixed with blood. The prognosis is poor; according to Soltmann, death occurs in 80-90% after 5-8 days. Only very mild degrees of scleredema may end with complete recovery. The treatment is the same as for sclerema. Sclerema neonatorum [scleroder-mia neonatorum; cutis rigor, disease of Chaussier and Underwood; oedema neonatorum; sclerysma (Hennig); induratio telae cellularis neonatorum (Henke, Baumgarten); algidite progressive (Hervieux); sclerema, or hardening of the skin of newborns]. An extremely rare disease, characterized by thickening and progressive hardening of the skin. Usually present in the child at birth or develops in the first months of its life, more rarely at the age of 1 to 2 years. It affects predominantly premature, poorly developed, and severely exhausted infants or mature children after severe illnesses (e.g. pneumonia), quite often after gastrointestinal disorders (especially infantile cholera). The favorite localization is the lower extremities. With signs of a decrease in general body temperature, often reaching very low degrees (24°C), the skin of the feet and calves becomes cold, as if frozen; these phenomena are accompanied by edema and hardening of the subcutaneous tissue, acquiring a dirty-yellow-brown, red-brown, or whitish-shiny color. Within a few hours or days, the process spreads to the thighs, lower abdomen, upper extremities, more rarely to the trunk and face. In sclerema, the skin is very hard and cannot be pinched into a fold with the fingers. Just as in S, the skin tightly covers the underlying tissues, so that almost the entire body is surrounded by hardened and rigid skin, like a shell. Only those places where the subcutaneous fat layer is absent or very weakly expressed remain free from the lesion (e.g. scrotum, penis, soles and palms). Food intake is greatly hindered, and breast sucking becomes impossible due to the rigidity of the oral fissure. Due to the sharp decrease or complete loss of mobility, the face appears waxy, as if frozen, and often wrinkled like an old person. This pathological condition causes a rapid loss of body weight, weakening of heart activity, slowing of respiration, and barely perceptible pulse. Often diarrhea is added. Urination decreases; the fontanelle is often found sunken. Sick children lie in a state of apathy, without any movements. In most cases, the fatal outcome occurs by the end of the first week of the disease with clouding of consciousness _g continuous decrease in body temperature or in an attack of convulsions. In a very small percentage of cases (Garrod), the induration of the skin gradually disappears and there is a partial or complete return to normal. Inflammatory phenomena very rarely develop in the sclerotic skin. According to Genoux, Parrot, Soltmann, and others (Henoch, Parrot_rSoltmann), two kinds of varieties of sclerema of newborns should be distinguished: true, or fatty (sclerema adiposum), and edematous (sclerema oedematosum). Sometimes a combination of both forms is observed. Some authors believe that sclerema of newborns has many clinical features in common with sclerema of adults, and tend to identify both processes, citing as the triggering factor the same, as yet unknown cause, which in adults produces a chronic course due to the considerable resistance of the body, and in weakened and cachectic infants causes an acute course. According to Darrieu, this disease, which runs the course of an infectious process of unknown character, in which some authors saw congenital syphilis as a possible cause, differs sharply from the curable skin induration of Marfan (induration cutanee curable du nouveau-né), which arises from obstetric trauma. Thanks to the detailed works of Lieberthal (Chicago), Bernheim-Karrer (Zurich), and others, it is known that in the latter disease, it is a matter of necrosis of the subcutaneous tissue, apparently of traumatic origin, closely related to cysteonerosis of adults. Fatty sclerema usually develops after strong losses of water by tissue juices due to profuse diarrhea, transudations in serous cavities, or internal hemorrhages. The essence of the disease, as anatomical studies show, consists in the solidification and hardening of the subcutaneous fat, arising due to external cooling, a drop in body temperature of the exhausted child, and insufficient food intake. This is explained by the fact that in newborns and infants in the first months of life, the fat of the subcutaneous tissue, unlike the fat of adults, contains an increased amount of free fatty acids (57% instead of 35% in adults, according to Tanger). Smith (C.S.

Smith) comes to the conclusion that the changes in the fatty tissue in sclerema show great similarity to fat necrosis and do not depend on the absence, resp. insignificant content, of oleic acid in the fatty tissue of children. In other cases, serous fluid is found in the loops of the cellular tissue. Some authors point to a sharp narrowing of the blood vessels (M. Joseph) and the focal development of cellular infiltration. Apparently, a deep disorder of blood circulation in the skin and a decrease in nutrition play a major role. On a section, the fatty tissue has a white color (stearin degeneration). Recognition is not difficult. The prognosis is extremely unfavorable; usually children die within a few days or hours, however, the possibility of recovery is not completely excluded. Treatment consists of artificial and uniform warming of the patient's body (placement in an incubator, wrapping with hot bottles, warm wrapping), rubbing and systematic massage of the skin with fats, resp. camphor oil, raising cardiac activity (stimulants), and forced and rational feeding (through a tube). Mustard and hot baths, galvanization of the sympathetic nerve (Kaposi) and subcutaneous injections of artificial serum (Natr. chlorat. 4.0; Natr. bicarbonici 3.0; Aq. dest. 1,000.0, 3 times a day at 10 g and more) are recommended.