Buschke’s scleredema is a rare scleroderm–like disease characterized by indurative-fibrous changes in the skin. Clinically manifested by thickening, compaction of the skin of the face, neck, upper torso, leading to limited movement. The diagnosis is made on the basis of histological examination of the affected area of the skin. In some cases, treatment is not required, scleredema regresses independently. With prolonged persistent course, different methods of therapy are used – glucocorticosteroids, antifibrotic agents, phototherapy. The use of blood clotting factors and radiation therapy is also effective.
Meaning
Scleredema Buschke (BS, syn. ‒ edematous scleroderma, progressive benign subcutaneous induration, adult scleredema) is a pathology similar in symptoms to focal scleroderma and cutaneous manifestations of systemic scleroderma. For the first time this disease was described by the German dermatologist A. Buschke in 1902. There are 2 age peaks of morbidity – from 1 year to 20 years, from 41 to 50 years. Women suffer more often than men. Scleredema is often associated with other autoimmune rheumatic diseases (rheumatoid arthritis, ankylosing spondylitis, Sjogren’s syndrome), malignant neoplasms, especially in the field of hematology.
Causes
To date, the exact cause of Buschke’s scleredema is unknown. However, there is a clear link between the development of scleredema and a streptococcal infection of the upper respiratory tract, viral infections (rubella, mumps, chickenpox). There is information about the occurrence of scleredema after tuberculous lymphadenitis. Endocrine diseases – diabetes mellitus, thyrotoxicosis, insulinoma – also serve as a background for the appearance of BS. The role of hereditary predisposition is assumed. Family cases of BS were described.
Pathogenesis
The mechanism of pathological processes in Buschke scleredema remains not fully understood. Some authors claim that under the influence of various triggers, the autonomic innervation of the dermis is disrupted, inflammatory mediators are produced. As a result, excessive accumulation of mucoid-like substances (hyaluronic acid, mucopolysaccharides), proliferation of collagen and elastic fibers occurs in the dermis and subcutaneous tissue.
Scleredema after streptococcal angina is caused by molecular mimicry, i.e. the similarity of the antigenic structure of streptococcus and dermal cells, which leads to the production of antibodies and a cross-immune reaction. In diabetes mellitus, long-term hyperglycemia and insulin resistance are important, stimulating the synthesis of collagen and its non-enzymatic glycosylation, which makes collagen fibers less elastic and more rigid.
Classification
In clinical rheumatology, there is a classification that distinguishes three types of Buschke scleredema (depending on the previous disease), differing in course, prognosis and required treatment methods:
- Type I (classical, postinfectious BS). Occurs after suffering streptococcal tonsillopharyngitis, as well as viral infections. The most frequent and favorable option.
- Type II (BS associated with paraproteinemia). It develops in diseases in which pathological proteins-paraproteins – are found in the blood – hemoblastosis, multiple myeloma, Waldenstrom’s macroglobulinemia. The most severe type of flow.
- Type III (diabetic BS). This variety is typical for people with poorly controlled type 2 diabetes mellitus. It is more common in men.
Symptoms
The onset is usually acute, sudden. Small areas of compaction appear on the face and neck. Skin folds and facial wrinkles are smoothed out. The process spreads very quickly to the upper chest and back. The skin of the arms, legs, and lower back may be affected, but the hands and heels always remain intact. The skin acquires a pale or slightly bluish hue. In terms of density and tension, the skin can be compared with paraffin, it cannot be gathered into a fold. The defeat is always symmetrical.
The patient feels constriction, stiffness and difficulty in movement. Facial expressions are violated. At the same time, the patient does not experience pain, itching or tingling. Sometimes there is an increase in the tongue and dryness of the mucous membrane of the eyes (xerophthalmia). The regulation of sweating may suffer, both upward and downward. There is no atrophy characteristic of systemic and focal scleroderma. As a result of a strong compaction of the skin of the chest, the respiratory excursion of the chest is limited – it becomes difficult for the patient to breathe. Possible pericarditis, accompanied by squeezing pain in the heart, swelling of the legs and heaviness in the right hypochondrium.
Complications
Buschke’s scleredema rarely causes serious complications. Only isolated cases of disability of patients due to pronounced difficulty in movement, which reduces performance and deprives self-service opportunities, are described. Xerophthalmia can lead to keratitis, ulceration and perforation of the cornea. With pericarditis, there is a risk of life-threatening cardiac arrhythmias and severe heart failure. Basically, the adverse consequences that pose a danger to the patient are associated with the pathology against which BS has developed – diabetes mellitus, neoplasms.
Diagnostics
Dermatologists and rheumatologists are engaged in the supervision of patients with BS. Anamnestic data, namely, transferred and concomitant diseases, play an important role in the diagnosis. On examination, the skin is smooth, shiny and dense. With pericarditis, deaf tones are heard in the heart, signs of circulatory insufficiency are also noted – an increase in the liver, swelling of the jugular (cervical) veins. The following types of examination are carried out:
- Laboratory and instrumental diagnostics. In general and biochemical blood test, markers of inflammation are detected – an increase in white blood cells, an accelerated rate of erythrocyte sedimentation, an increased concentration of C-reactive protein. Echocardiography reveals thickening of the pericardial leaves, effusion into the pericardial cavity.
- Skin biopsy. Histological examination of the compacted area of the skin is the main diagnostic method that reliably confirms Buschke’s scleredema. The following changes are noted – diffuse sclerosis, hyalinosis of the dermis, atrophy of hair follicles, sebaceous, sweat glands, accumulation of a large amount of mucin-like substance. In the subcutaneous tissue ‒ the growth of elastic, collagen fibers.
In addition to the diagnosis of BS, the examination should be aimed at finding a disease that could serve as a trigger factor. This is especially true of types II and III. To do this, the content of antistreptolysin-O, glycated hemoglobin, and paraproteins in the blood is determined. In the urine, the level of total protein, the presence of Bence-Jones protein is examined. If necessary, immunophenotyping of blood cells is performed, trepanobiopsy – taking a bone marrow sample for microscopy.
BS first of all needs to be differentiated with focal and systemic scleroderma. For this purpose, an analysis is performed for specific antibodies – antinuclear factor, antibodies to topoisomerase, anti-centromeric antibodies. Scleredema should also be distinguished from diffuse eosinophilic fasciitis, late cutaneous porphyria, Werner syndrome (adult progeria).
Treatment
Patients are subject to mandatory hospitalization in the rheumatology or dermatology department. There is no etiotropic therapy. However, most often, for example, in many cases of type I scleredema, no treatment is required. The disease regresses on its own after a few weeks. It is often enough to carry out therapy of causal pathology (antibiotics, hypoglycemic drugs, chemotherapy, etc.) that provoked the BS. In severe and persistent scleredema, various methods of pathogenetic exposure are used:
- Medications. Antifibrotic drugs (penicillamine, colchicine) are used to slow the growth of connective tissue in the skin and subcutaneous fat. Injections of the enzyme hyaluronidase are sometimes prescribed. Medications that suppress the inflammatory process are also used – topical and systemic glucocorticosteroids (prednisone), cytostatics (methotrexate).
- Non-medicated. Non-medicinal methods include PUVA therapy, which consists in the external or oral administration of a drug that increases sensitivity to ultraviolet (UV), followed by irradiation of the skin with long-spectrum UV waves. There are data on the effectiveness of local radiation therapy, intravenous transfusion of blood clotting factor XIII.
Prognosis and prevention
Buschke’s scleredema is a benign disease that very rarely leads to serious complications. Approximately half of all patients experience an independent regression of symptoms. In isolated cases, pronounced movement difficulties are possible. The prognosis is mainly determined by the disease that served as the background for scleredema. As a preventive measure, competent glycemic control can be recommended for people with diabetes mellitus, early diagnosis of malignant neoplasms, general measures to strengthen immunity to prevent viral infections and streptococcal angina – regular exercise, balanced nutrition, hardening, etc.
