Integrum lupus erythematosus is a type of erythematosis with skin manifestations and photosensitivity. Clinically characterized by a rash of spots with further transformation into atrophic scarring through the stage of hyperkeratosis. The provoking factor is insolation. The integrative form of the disease is diagnosed on the basis of anamnesis and a typical clinical picture. To confirm the diagnosis, microscopy of scraping from the element, histology, and immunological blood testing are used. Therapy includes correction of concomitant pathology, a complex of anti-inflammatory drugs, vitamin therapy. Patients need lifelong medical supervision.
Integrum lupus erythematosus is a local type of skin lesion in systemic collagenosis (SLE), known to dermatologists for more than a century. In 1828, the French physician Biette described it under the guise of centrifugal erythema, but his student Cazenave published this information in 1833. The Austrian dermatologist Gebra in 1845 noted that most congestive (stagnant) dermatoses on the face have the shape of a “butterfly”, and in 1851 Kazenava proposed to introduce the term “lupus erythematosus” – “lupus erythematosus” to distinguish skin tuberculosis and manifestations of collagenosis. The pathology got its name due to the fact that the rashes on the skin, according to experts, resembled the bites of hungry wolves.
In 1872, Hungarian dermatologist Kaposi linked skin rashes with lesions of internal organs, dividing all manifestations of SLE into discoid and disseminated. In the next 20 years (from 1875 to 1895), one after another, dermatologists Kaposi, Hutchinson and Osler proposed theories of the etiopathogenesis of integrum lupus erythematosus, pointing to insolation as the main cause of the pathology. The urgency of the problem is due to the ability of skin lesions to transform into a systemic type of collagenosis with the possibility of a fatal outcome.
The causes of the pathological process have not been fully studied. Dermatology considers the virus to be the trigger of integrum lupus erythematosus. Indirectly, this proves the presence of formations containing virus-like DNA in the scrapings from the affected skin. Focal infection foci, hyperinsolation, temperature changes, skin traumatization, irrational medication intake are called provoking moments. Some dermatologists consider integrated lupus erythematosus as a family pathology (more than 1% of cases have a family history of this disease). Even a gene of hereditary predisposition – HLA BD/DR, capable of provoking the disease, has been identified, but there is no reliable confirmation of this theory yet. The recognized mechanism of development of integrum lupus erythematosus today is considered autoimmune. In response to the introduction of a damaging antigen into the skin, the body’s immune system is activated. A distinctive feature of the immune response in integrum lupus erythematosus is its autoimmune nature.
For reasons that are unclear to the end, some of the foreign antigens are introduced into the connective tissue structures, causing the production of special autoantibodies, sensitizing the dermis. Autoantibodies are mostly antinuclear, associated with nucleic acids and proteins in the cell nucleus, which is used for the accurate diagnosis of integrum lupus erythematosus. In parallel, keratinocytes and lymphocytes producing biologically active inflammatory mediators are damaged, circulating immune complexes are formed. They damage the membranes of skin cells, increasing inflammation. Cellular immunity is disrupted, there is a disproportion between T and B lymphocytes. The number of T-killers and T-suppressors is decreasing, the population of B-lymphocytes is rapidly increasing. Immunoglobulin LE appears in the blood, followed by LE cells specific to pathology. A bright pink erythematous rash appears on the skin.
Most often, cutaneous erythematosis is divided into several variants according to the nature of the primary elements. Distinguish:
1. Discoid erythematosis is the most common type of integumentary lupus erythematosus, characterized by local changes on the skin associated with climatic conditions. It includes:
- erythematous form, the distinctive feature of which is a rash of pale pink spots on the background of edematous skin with a tendency to peripheral growth;
- infiltrative (hyperkeratotic) form, manifested by the formation of flaky foci of infiltration and hyperkeratosis with a positive Meshchersky phenomenon;
- atrophic form characterized by central alabaster dystrophy with scar formation.
2. Centrifugal erythema Bietta, which is manifested by the formation of a superficial bright spot on the skin of the face.
3. Disseminated erythematosis, a distinctive feature of which is the multiplicity of foci with a tendency to spread throughout the skin.
4. Kaposi-Irgang erythematosis is the rarest deep variety of integrum lupus erythematosus, characterized by the formation of specific mobile formations (lupus–panniculitis) and deprived of the possibility of transformation into a systemic process.
Currently, there is another classification of integrum lupus erythematosus in dermatology. According to atypical clinical manifestations , there are:
- Pigmented integumentary lupus erythematosus, in which brown erythemas appear around the formed focus.
- Hyperkeratotic integumentary lupus erythematosus with abundant (“calcareous”) peeling.
- Tumor integrum lupus erythematosus, in which purple edematous plaques appear in the focus of hyperkeratosis.
- Seborrheic integumentary lupus erythematosus, characterized by the appearance of primary elements against the background of seborrheic skin lesions.
- Mutating integrum lupus erythematosus, in which atrophic “dissolution” of the connective tissue of the tip of the nose and auricle occurs.
- Pemphigoid integrum lupus erythematosus, characterized by the formation of bullae in the foci of inflammation.
Erythematosis debuts with rashes of pink spots with clear borders on the background of slightly edematous dermis. Almost immediately, whitish scales with spikes on the inside appear in the center of the erythema, growing into the hair follicles and creating hyperkeratosis phenomena. An attempt to remove the scales causes pain (Meshchersky’s symptom). Over time, white skin atrophy begins from the center. A typical discoid focus is formed – an asbestos atrophic center with a shiny scar and a shaft of deep hyperkeratosis against the background of infiltration with a scarlet rim along the periphery. The localization is specific, the shape resembles a butterfly that has opened its wings from the back of the nose to the cheeks. Integrum lupus erythematosus spreads over time to the decollete area, head, neck, mucous membranes. Erosions appear on the red border of the lips.
Less often, erythema occurs, characterized only by hyperemia, spread over the face in the shape of a butterfly. The dermis does not infiltrate, does not atrophy, the foci do not tend to spread (erythema Bietta). Reaching a certain size, the erythema stabilizes, while the transformation of the lesion into a systemic type of lupus erythematosus is possible. If foci of lupus hyperemia or lupus discs multiply, begin to spread throughout the skin, then the disease transforms into disseminated cutaneous lupus.
Rarely, mainly after injury to the skin, cases of a deep form of integumentary lupus erythematosus (Kaposi-Irgang erythematosis) are recorded. In this form, along with all the typical symptoms, the formation of nodes under the dermis (lupus-panniculitis) is revealed. The nodes inside ulcerate and atrophy, dense, separated from the surrounding tissues. A distinctive feature of this type of integrum lupus erythematosus is its inability to degenerate into systemic lupus erythematosus with a pronounced tendency to malignancy. It is localized mainly on the upper extremities and head.
Diagnosis and treatment
The clinical diagnosis of pathology is usually not difficult. Dermatologists use the results of microscopy and histology, immune testing (ELISA). To determine the stability and severity of inflammation, the epidermis scarification is microscopically examined. Electron microscopy reveals virus-like inclusions confirming the viral nature of integrum lupus erythematosus. Specific immunoglobulins M and G are detected by ELISA as part of autoimmune complexes. ELISA is also used to determine anti-nuclear antibodies. Differentiation of integrum lupus erythematosus is carried out with seborrhea, psoriasis, acne, dry eczema.
The therapy of the disease is complex. They begin with the correction of concomitant pathology, connect quinolines, vitamins, anti-inflammatory drugs. Externally, hormonal ointments are indicated. In case of resistance, the destruction of nodes with liquid nitrogen is used. Suspicion of transformation of the process is an indication for hospitalization of the patient. Sunscreens are required in spring and summer. Medical examination is carried out for life. The prognosis with timely adequate therapy and no signs of transformation into a systemic form of pathology is favorable for life. When symptoms of systemic lupus erythematosus appear, the prognosis worsens.