Lymphomatoid papulosis is a benign disease of the lymphatic system with recurrent polymorphic skin manifestations. Clinically accompanied by papular eruptions of various sizes, which then transform into bulls, open, forming erosions, crusts, scars. The rash can be located on any part of the skin, there are no subjective sensations. The diagnosis is made on the basis of anamnesis, clinical data, histological and immunohistochemical studies. As part of the treatment course of this disease, glucocorticosteroids, antibiotics, cytostatics, PUVA therapy are used, but all measures give only a short-term effect.
ICD 10
L41.2 Lymphomatoid papulosis
General information
Lymphomatoid papulosis is a nodular lymphoid tumor of the skin of a benign nature, which tends to slow growth. It can be represented both by a single formation and by multiple elements. The disease has no gender coloring, seasonality, endemicity, hereditary predisposition. The average age of manifestation of pathology is 40 years. The prevalence in the population is 1-2 cases per 1 million people. In representatives of the Negroid race, lymphomatoid papulosis practically does not occur. In an independent nosological unit , the disease was isolated by V. Macaulay in 1968, hence another name for lymphomatoid papulosis – Macaulay’s disease.
Currently, lymphomatoid papulosis is usually attributed to the group of primary CD30+ lymphoproliferative skin pathologies, along with such processes as pseudolymphoma, lymphogranulomatosis, atypical regressing histiocytosis, anaplastic large-cell skin lymphoma. With the listed diseases, lymphomatoid papulosis brings together not only the common origin, but also the frequent simultaneous or sequential development of several pathologies in one patient. The increased alertness of dermatologists and oncologists regarding lymphomatoid papulosis is explained by the possibility of its transformation into malignant T-cell lymphoma (in 10-20% of cases).
Causes
The etiology of the disease is unknown. Pathogenesis is also the subject of controversy and discussion. Most dermatologists, based on the histological picture of lymphomatoid papulosis, which looks like a true lymphoma, consider the disease a lymphocytic skin tumor of low malignancy. Others consider lymphomatoid papulosis as a limited benign lymphoplasia that occurs with the participation of CD4 lymphocytes, which regulate the strength of the immune response. In all likelihood, the truth is somewhere in the middle.
In the development of lymphomatoid papulosis, a violation in the differentiation of T-lymphocytes plays a role when, as a result of latent autoimmune and systemic diseases, congenital or acquired immunodeficiency, ionizing radiation, they begin to mutate, provoking increased proliferation of epidermal cells, both healthy and genetically destroyed. In parallel, the internal functions of epidermal cells change, they begin to produce cytokines and mediators that trigger the mechanism of inflammation, enhance the proliferation of cells of the affected skin. This is how benign lymphoid formations of the dermis appear.
The expression of CD30-antigen by atypical lymphoid cells in combination with the possibility of spontaneous degeneration of lymphomatoid papulosis into malignant lymphoma indicate in favor of the point of view of those dermatologists who consider lymphomatoid papulosis to be a type of tumor with a small degree of malignancy. On the other hand, the rarity of such a transformation speaks in favor of the theory of lymphoplasia. Thus, at the moment there is no consensus. It is only known for sure that if the proliferation of lymphocytes ceases to obey the control of the immune system, malignant lymphoma will develop. This indicates a close connection of immune and oncological processes in the patient’s body.
Classification
In modern dermatology, lymphomatoid papulosis is classified according to the histological picture, which helps in making a diagnosis. There are four types of lymphomatoid papulosis:
- Type 1 A – in the histological picture there are different types of cells: anaplastic lymphoid cells, lymphocytes, eosinophils, neutrophils, histiocytes; mixed-cell process;
- Type 2 B – resembles fungal mycosis, single atypical anaplastic cells of malignant lymphoma are present in the histological preparation, it is rare;
- Type 3 C – histologically similar to large-cell lymphoma, the drug is dominated by atypical lymphoid cells;
- Type 4 D – resembles pagetoid reticulosis, the most aggressive of all types.
Symptoms
The disease debuts with a rash on the skin (less often – on the mucous membranes) of asymptomatic or slightly itchy brown spots up to 3 cm in diameter, on which nodules, bullae, convex neoplasms covered with scales appear for several weeks. The rash does not have favorite localization sites, it appears on the face, scalp, extremities, less often – on the mucous membranes. Elements of a rash of lymphomatoid papulosis are poured in waves, are capable of self-resolution and new rashes, are prone to grouping, especially often around hair follicles.
The full cycle of the existence of primary elements in lymphomatoid papulosis is about 2-8 weeks. During this time, the bulls are opened with the formation of erosions, which are covered with hemorrhagic crusts; combs, the addition of secondary infection are possible. Sometimes papules begin to peel off – all this creates a picture of false polymorphism. Each element of the rash can involute at any stage of development. Usually, the resolution of the primary elements begins from the center, ends with the formation of a scar, leaving behind post-inflammatory hyper- or hypopigmentation. Subjectively, the patient’s condition is not disturbed, in rare cases, prodromal phenomena, itching are possible.
Diagnosis and treatment
Lymphomatoid papulosis is diagnosed on the basis of anamnesis, clinic, skin biopsy data, histological and immunohistochemical studies. Sometimes a biopsy of the affected lymph node located nearby is performed. In doubtful cases, cytogenetic and molecular studies are performed. It should be noted that there are no reliable criteria capable of predicting the malignant transformation of lymphomatoid papulosis today. Differentiate the disease with parapsoriasis, lymphomas, urticaria, drug toxidermy, scabies, Cesari syndrome, lymphogranulomatosis.
There are no radical methods of therapy for lymphomatoid papulosis. Large doses of tetracyclines are used in combination with corticosteroids, sulfones (dapsone) and cytostatics (carmustine). Sometimes retinoids and interferons are used. With disseminated rashes, electron beam irradiation and PUVA therapy are performed. However, none of the methods of therapy gives a long-term effect. The prognosis depends on the course of lymphomatoid papulosis, the number of relapses. Regular follow-up by a dermatologist is necessary.