Lymphocytic papulosis: causes, symptoms, diagnosis, treatment

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

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

Pathomorphology of lymphocytic papulosis. R. Willemse et al. (1982) identified two histological types, A and B, depending on the nature of the cells that make up the infiltrate. Type A is characterized by the presence of large atypical cells with vesicular nuclei of non-lymphoid origin; type B contains 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 are classified as histological type A, plaque elements - as type B. In some cases, there is a transitional picture between types A and B. In addition, the histological picture depends on the stage of development of the element, which is especially well seen 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 of small lymphocytes, mononuclear cells with cerebriform nuclei, histiocytes with an admixture of neutrophilic and eosinophilic granulocytes. The number of large atypical cells with massive cytoplasm and a split nucleus is insignificant. The 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, mitotic figures, vessels with swelling and proliferation of the endothelium may be observed, extravasates of erythrocytes, as well as neutrophilic and eosinophilic granulocytes are noted. The third stage of a fully developed element is characterized by diffuse infiltration with penetration of 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. A large number of mitotic figures are noted. Small lymphocytes and mononuclear cells with cerebriform nuclei are located only on the periphery of the lesion. There are foci of necrosis, and in necrotic papules - total destruction of the epidermis with ulceration and crusts. Blood vessels sometimes with fibrinoid changes in the walls, accompanied by extravasates of erythrocytes, especially in the papillary layer of the dermis. The fourth stage of regression of the element is distinguished by superficial, mainly perivascular infiltrates consisting of lymphocytes and histiocytes. Mononuclear cells with cerebriform nuclei, neutrophilic and eosinophilic granulocytes are present in small quantities. Large atypical cells of non-lymphoid origin are single or completely absent.

Type B differs from type A by the absence of parallelism in the histological and clinical pictures. Even in the clinically expressed form, the infiltrate is not diffuse. A characteristic feature of this type is the 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 also found in perivascular infiltrates, in which neutrophilic and sometimes eosinophilic granulocytes are detected in large quantities.

A.V. Ackerman (1997) also distinguishes 2 types of lymphomatoid papulosis - a type similar to mycosis fungoides and a type similar to Hodgkin's disease, and considers 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 atypical lymphocytes with cerebriform nuclei, and the second - a monomorphic infiltrate with the presence of many atypical binuclear and even multinuclear lymphocytes.

G. Burg et al. (2000) believe that since small and large pleomorphic cells and all transitional forms can be detected in the same patient at the same time, but in elements of different periods of existence, there is no point in dividing into A- and B-types.

Gene rearrangement studies indicate the possibility of Hodgkin's disease, lymphomatoid papulosis, and cutaneous T-cell lymphoma arising from a single T-cell clone.

Lymphomatoid papulosis is differentiated from the plaque stage of mycosis fungoides; Hodgkin's disease; insect bites; and Mucha-Gobermann parapsoriasis.

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