Lymphocytic papulosis: causes, symptoms, diagnosis, treatment

The first description of the disease of lymphocytic papulosis belongs to A. Dupont (1965). In 1968, WL Macauly introduced the term "lymphomatoid papulosis" for long-term, benign, self-healing papular rashes, histologically having a malignant appearance.

Clinically, the initial changes are characterized by erythematous spots or reddish-brown papules. Then they become hemorrhagic or necrotic, can spontaneously disappear within 3-6 weeks, and in some cases only after a few months, leaving hyperpigmentation or scars. The lesions are located on the trunk and extremities, occasionally on the face. There may be eczematoid changes. The general condition of the patients is not broken, the lymph nodes are not changed.

Pathomorphology of lymphocytic papulosis. R. Willemse et al. (1982), depending on the nature of the cells constituting the infiltrate, two histological types were identified: A and B. Type A is characterized by the presence of large atypical cells with bubble-shaped nuclei of non-lymphoid origin; at type B, there are predominantly atypical mononuclear cells with cerebriform nuclei that tend to penetrate the basal and suprabasal layers of the epidermis, and a large number of large atypical non-lymphoid cells.

This histological picture, according to the authors, correlates with clinical manifestations. Thus, papular and nodular elements belong to histological type A, plaque elements to type B. In some cases, there is a transitional pattern between types A and B. In addition, the histological picture depends on the stage of development of the element, which is especially well traced in lymphomatoid papulosis type AAR Willemse et al. (1982) subdivide the histological evolution of the element into four stages: the first stage of early changes is characterized by the presence of a superficial perivascular infiltrate from small lymphocytes, mononuclear cells with cerebral nuclei, histiocytes with an admixture of neutrophilic and eosinophilic granulocytes. The number of large atypical cells with a massive cytoplasm and a split nucleus is insignificant. Infiltrate tends to be located between bundles of collagen fibers; changes in the vessels are not detected. The second stage of the developing element is characterized by a more diffuse infiltrate penetrating into the deeper layers of the dermis and even into the subcutaneous adipose tissue. The number of large, atypical cells increases, mitosis patterns, vessels with swelling and proliferation of the endothelium can be observed, extravases of erythrocytes, as well as neutrophilic and eosinophilic granulocytes are noted. The third stage of the fully developed element is characterized by diffuse infiltration with penetration of the infiltrate cells into the epidermis and into the deep layers of the dermis up to the subcutaneous adipose tissue. The infiltrate consists of a large number of large atypical cells of non-lymphoid origin, histiocytes, neutrophilic, and sometimes eosinophilic granulocytes. There is a large number of mitosis figures. Small lymphocytes and mononuclear cells with cerebral nuclei are located only on the periphery of the focus. There are foci of necrosis, and in necrotic papules - total destruction of the epidermis with ulceration and crusts. Blood vessels sometimes with fibrinoid wall changes, accompanied by extravasation of erythrocytes, especially in the papillary layer of the dermis. The fourth stage of regression of the element is characterized by superficial, mainly perivascular infiltrates, consisting of lymphocytes and histiocytes. Mononuclear cells with cerebral nuclei, neutrophilic and eosinophilic granulocytes are present in a small amount. Large atypical cells of non-lymphoid origin are single or completely absent.

Type B differs from type A in the absence of parallelism in histological and clinical patterns. Even with a clinically pronounced form, the infiltrate is not diffuse. A characteristic feature of this type is invasion of the basal and suprabasal layers of the epidermis by a large number of mononuclear elements with hyperchromic and cerebriform nuclei. Similar cells are found in perivascular infiltrates, in which neutrophilic and sometimes eosinophilic granulocytes are detected in large numbers.

A.V. Ackerman (1997) also distinguishes 2 types of lymphomatoid papulez - a type similar to mushroom-like mycosis and a type similar to Hodgkin's disease, and treats lymphomatoid papulosis as CD30 + lymphoma, believing that the clinical manifestations of both variants are identical. Histologically, the first variant is characterized by the presence of a mixed infiltrate with the presence of atypical lymphocytes with cerebriform nuclei, and for the second one - a monomorphic infiltrate with the presence of many atypical binuclear and even multinucleated lymphocytes.

G. Burg et al. (2000) believe that since small and large pleomorphic cells and all transitional forms can be found in the same patient at the same time, but in elements of different lifetimes, it makes no sense to divide into A- and B- types.

Studies on the rearrangement of the gene show the likelihood of the appearance of lymphogranulomatosis, lymphomatoid papulosis and T-cell lymphoma of the skin from a single clone of T lymphocytes.

Differentiate lymphomatoid papulosis from the plaque stage of fungal mycosis; Hodgkin's disease; insect bites; parapsoriasis of the Fly - Gobermann.

[1], [2], [3], [4], [5], [6]