Mucha-Habermann disease is an acute benign papulosquamous variety of teardrop-shaped parapsoriasis. The clinical difference of pathology is the true polymorphism of rashes, papules, pustules, bullae, hemorrhages, heterogeneous crusts and varicell-like (hemorrhagic) elements combined with the pink teardrop-shaped papules familiar to teardrop psoriasis. The primary elements are scattered, arranged symmetrically. The disease debuts acutely and generalizes very quickly. Prodrome phenomena are possible. Capable of independent spontaneous resolution. The only reliable diagnostic method is a biopsy. If necessary, local and systemic steroids and antibiotics are prescribed.
General information
Mucha-Habermann disease is a polymorphic dermatosis of unclear etiology, which is a type of parapsoriasis, characterized by acute onset and spontaneous resolution of the process. A rare severe febrile ulcerative necrotic form of the disease is also known, requiring treatment in a hospital. Usually, smallpox-like scars remain in place of the primary varioliform elements, papules resolve with hyperpigmentation phenomena, which clinically resembles teardrop-shaped psoriasis. It is this fact that has given some dermatologists reason to consider Mucha-Habermann disease a kind of teardrop. Pathology occurs more often in men of adolescent and young age, has no racial characteristics, occurs in all ethnic groups and geographical regions, off-season. The urgency of the problem at the present stage is associated with the possibility of spontaneous transformation of Mucha-Habermann disease into malignant fungal mycosis.
Causes
The etiology of the pathological process is not clear. In modern dermatology, three possible causes of the development of Mucha-Habermann disease are considered. The infectious theory is considered to be the leading one. From this point of view, Mucha-Habermann disease is provoked by the introduction of an infectious pathogen into the skin, which triggers a protective antigen-antibody reaction. Antigenic penetration disrupts the integrity of the cells of the local and general immune system, the production of inflammatory mediators begins, which results in skin reactions in the form of a rash of primary elements.
The theory of the occurrence of Mucha-Habermann disease due to a qualitative violation of T-cell tissue is close to the infectious theory. In this case, skin inflammation is considered as a process provoked by a response (possibly to a virus) involving a complex of T-lymphocytes + C04 suppressing cytokines and producing interleukins that are involved in the proliferation of lymphoid tissue. The result is a rash on the skin of a lymphoproliferative nature, capable of transforming into a fungal mycosis at any time. The process is disseminated with the reactive involvement of lymph nodes.
The third theory suggests the occurrence of allergic vasculitis as a result of the formation of immune complexes. In this case, an allergic onset is considered as a skin-damaging factor, which, when introduced, causes sensitization of the dermis. Allergic skin becomes hypersensitive and in response to any negative impact begins to actively produce antibodies, as a result, an autoimmune process develops with vascular damage.
Symptoms
Clinical manifestations of the disease are reduced to the sudden appearance of symmetrical rashes on the flexor surfaces of the extremities, abdomen and anterior part of the chest. The primary elements are represented by brown erythemas, small (from 1 mm to 7 mm) pink convex papules and bullae with central hemorrhagic necrosis. Rashes appear against the background of a rapidly passing subjective deterioration of general well-being. Initially, the scalp, palms and soles are not affected, then the process can spread to these areas of the skin. After a few months, the rash resolves itself. It is possible to damage the mucous membranes of the oral cavity, tongue.
The appearance of elements in some cases is preceded by high fever, asthenia, a feeling of weakness, weakness, myalgia. Then there is a polymorphic rash, the elements of which merge into large foci with raised edges, ulceration and central necrosis. In 70% of cases, regional lymph nodes increase. When these symptoms appear, they speak of a severe course of the febrile ulcerative-necrotic form of the disease. Such a variety is capable of chronicling and persisting for several months or years. The course of Mucha-Habermann disease depends on the general health of the patient and his immunity.
Diagnostics
The clinical diagnosis is made by a dermatologist based on the symptoms of the disease and the results of a biopsy. In the studied fragment of skin from the lesion, pronounced hyperkeratosis, necrosis of epidermal cells and lymphocytic infiltrate are found. In the dermis, the infiltrate consists of T-cell cytotoxic lymphocytes. Mucha-Habermann disease is differentiated with chickenpox, chronic lichenoid and teardrop-shaped parapsoriasis, lymphomatoid papulosis, pustular syphilis, Gianotti-Crossti disease, erythema multiforme, allergic vasculitis, papulonecrotic tuberculosis of the skin and toxicoderma.
Treatment
The tactics of treating the disease depends on the severity of the pathological process and its prevalence. In the therapy of the pathological process, dermatologists distinguish three lines. The first line is the intake of tetracycline antibiotics or macrolides inside according to individual schemes, the use of corticosteroids and local immunomodulators in the treatment. The second line is the use of physiotherapy: PUVA therapy, UVB therapy. The third line is the appointment of cytostatics. In severe cases, mixed therapy of the pathological process is possible. In the absence of the effect of outpatient treatment or the spread of the process, the patient is hospitalized. Externally, emollients (fat-based substances evenly distributed over the skin), hormonal ointments, preparations with alklomethasone that reduces pain, itching and the severity of inflammation in the skin are prescribed. There are no methods of prevention yet. The prognosis is uncertain, while the acute form responds to treatment better than other types of the disease and is less likely to transform into fungal mycosis.