Erythrodermic psoriasis is the most severe, life–threatening type of psoriasis, characterized by the severity and prevalence of the process, requiring immediate medical intervention. It is manifested by a chaotic rash of psoriatic spots, which form foci resembling a burn. Gradually, plaques, large-plate peeling, itching, skin soreness appear. It is diagnosed clinically, taking into account the anamnesis and the presence of a symptomatic triad (stearin stain, terminal film, spot bleeding). Sometimes histology is connected. Treatment is carried out in a hospital, provides detoxification, cytostatics, retinoids, hormones and physiotherapy.
Erythrodermic psoriasis (erythrodermic psoriasis) is a chronic exfoliative dermatitis characterized by secondary generalization of the process or skin manifestations of a systemic disease as a result of the loss of protective functions of the skin. In 52% of cases, the pathological process is preceded by skin pathology. Most often, pustular psoriasis is transformed into erythroderma. In 20% of cases, it is not possible to establish a precursor, such forms are called idiopathic. In 4%, erythrodermic psoriasis occurs against the background of past infections and diseases of immune genesis, 24% are drug provocations.
Erythrodermic psoriasis is rare, it is noted only in 1-2% of patients suffering from psoriasis. Men over 50 years of age of any race are more often ill, the disease debuts at any time of the year, worsens in autumn and winter, does not have endemicity. The severity of the process and the severity of the course require hospitalization due to intoxication, which poses a danger to the lives of patients. The urgency of the problem is related to the data that erythrodermic psoriasis has a tendency to transform into fungal mycosis or Cesari syndrome, as well as the fact that the pathological process significantly violates the quality of life of patients.
The exact causes of the pathological process are unknown. Nowadays, erythrodermic psoriasis is considered as a heterogeneous pathology resulting from the effects of genetic, immune and environmental triggers. Sometimes a hereditary predisposition is revealed (in the presence of family cases of psoriasis). Erythroderma can occur against the background of vulgar lichen or unchanged skin, it develops in sufferers of spongiotic dermatitis, scabies, photodermatitis and drug hypersensitivity. Provoking moments are stress, bad habits, uncontrolled medication, abrupt withdrawal of hormones or cytostatics, weakening of immunity (including HIV infection), auto-intoxication, injury to the skin, hyperinsolation.
The mechanism of development of erythrodermic psoriasis is due to the pathogenesis of the underlying pathological process. In fact, it is a reactive innate immune cytokine response of the skin to the effect of a pathological antigen. Immune disorders are reduced to a decrease in the number of T-lymphocytes with a disproportion towards an increase in T-helper cells and a decrease in T-cytotoxic lymphocytes that restrain the immune response. At the humoral level, dysglobulinemia is observed, there is an activation of chemokines – plasma proteins with pleiotropic activity, which, combined with the loss of protective functions of the skin, leads to acute inflammation and the spread of the pathological process over the entire surface of the skin.
Psoriatic erythrodermic erythema, which transforms into a papule or plaque, is formed due to a violation of the nutrition of the dermis and a spasm of the regional arteriole. The skin in the area of the primary element loses its barrier function, becomes vulnerable to penetration deep into any pathogenic origin. There is chronic inflammation with autoimmune conflict, which supports inflammation and triggers increased cell proliferation (in the erythema region, the intensity of division increases 200 times). The nutrition of this area of the dermis is disrupted, new cells die as quickly as they appear.
Visually, the formation of a new hyperemic spot, plaque is visible. At the same time, another “vicious circle” of psoriasis closes, which is the basis of the chronic course of the pathological process: vessel spasm in combination with a violation of the barrier function leads to the penetration of antigens into the skin, the development of inflammation, autoimmune reaction and active proliferation; some cells die, some form erythema and plaques, after which everything repeats. The pathological process depletes the adrenal glands, which are involved in the production of anti-inflammatory hormones.
In modern dermatology, it is customary to distinguish between primary (manifests spontaneously, against the background of complete health) and secondary (develops against the background of already existing pathology) erythroderma and three main varieties of the pathological process:
- Generalized erythroderma with almost total lesion of the skin. The primary element is a flaky drain bright red edematous erythema.
- Hyperergic erythroderma, initially forming single foci of the inflammatory pathological process, accompanied by itching and a burning sensation of the skin, which tend to constantly spread and disrupt the general condition of the body.
- Suppurating erythroderma is a transitional form between pustular psoriasis of Tsumbush and true erythroderma, characterized by the addition of pustular rashes, the formation of suppurated areas of the skin and mucous membranes.
Generalization of the psoriatic process in the vast majority of cases occurs secondarily, against the background of a long-existing precursor disease. In the initial stage, erythrodermic psoriasis is visually manifested by scarlet erythema. The acute onset is accompanied by an exacerbation of the underlying pathology, a significant increase in body temperature and a violation of the general condition of the patient. Requires hospitalization. Lymph nodes are involved in the process, hair loss and nail stratification begin, hyperhidrosis joins, chills appear.
The foci of hyperemia resemble a burn, spread over the entire surface of the skin, new plaques appear on their surface, which begin to peel off. The scales peel off in large plates, the surface under them sometimes bleeds due to the subcutaneous location of arterioles involved in the formation of primary elements. Dryness and soreness of the skin are noted, itching is added. The lack of protective functions of the dermis makes a patient suffering from erythrodermic psoriasis a target for any infection.
With a generalized form that occurs gradually, as a result of peripheral growth of primary elements, individual spots merge, first forming large diffuse areas with fine peeling, and then affecting the entire skin. Papules and plaques are indistinguishable in them, the skin is tense, infiltrated, looks like a shell. Hair and nails are not involved in the process. With the development of erythroderma, as a continuation of pustular psoriasis, a rash of pustules joins other manifestations.
Purulent bulls dry up with the formation of crusts, disappear and reappear. The rash spreads to the mucous membranes of the mouth and tongue. At the same time, the general condition may deteriorate sharply, which necessitates hospitalization. All forms of the pathological process are complicated by heart failure, edema, infectious diseases, thermoregulation disorders and protein deficiency. Psoriatic arthritis often develops. Erythrodermic psoriasis can cause disability or death.
Diagnosis and treatment
The clinical diagnosis is made by a dermatologist based on symptoms, anamnesis of the disease and the presence of a diagnostic triad (stearin stain, terminal film, bleeding by the type of dew drops), in doubtful cases, histology results are used. Erythrodermic psoriasis is differentiated with lymphoproliferative processes, toxicoderma, scabies, dry eczema, dry epidermitis, malignant neoplasms, borreliosis, dermatomycosis, bullous dermatitis, lichen planus, neurodermatitis and Zhiber’s lichen.
Symptomatic treatment of erythrodermic psoriasis is ineffective, since the pathological process is a combination of systemic problems in combination with an infectious component. Recently, the method of PRM – physiological regulatory medicine has become widespread, which is a comprehensive therapy program for erythrodermic psoriasis, designed for 21 days of inpatient treatment. In the optimal variant, the patient is provided with a separate room with a special microclimate, if there is none, regular quartz treatment is required, maintaining a certain humidity and temperature in the room.
The course of treatment includes detoxification, antioxidant and antiparasitic therapy, correction of the adrenal glands, vitamin therapy and the appointment of trace elements (selenium, zinc, silicon). The program also provides for hydration of the body, immunomodulation, corticosteroid treatment followed by foreign protein therapy, PUVA therapy for psoriasis, transfusion of one’s own quantum photomodified blood, plasmapheresis, hemosorption, lymphocytopheresis and correction of the nutrition profile. If necessary, external therapy with emollients and cooling dressings is connected.
Prevention of erythrodermic psoriasis consists in regular preventive examinations, medical examinations, maintenance therapy courses and diet. With timely diagnosis and treatment, the prognosis is usually favorable, in the absence of therapy, various complications and even death are possible.