Lymphocytoma cutis

Several synonyms have been used for lymphocytoma cutis including lymphadenosis benigna cutis, cutaneous lympho-plasia, cutaneous lymphoid hyperplasia and pseudolymphoma of Spiegler-Fendt. Various antigenic stimuli can induce these lesions: insect bites, drugs, vaccinations, acupuncture, wear-

Fig. 20.25 Lymphomatoid drug eruption, T-cell type. Note several atypical lymphocytes and one mitotic figure (detail of Fig. 20.24).
Atypical Lymphocytic Infiltrate Skin
Fig. 20.26 Lymphomatoid drug eruption, B-cell type. Nodular infiltrates of lymphocytes with reactive germinal centres. (Courtesy of Dr Dieter Metze, Münster, Germany.)

ing of gold pierced earrings, medicinal leech therapy and tattoos [70-72]. One of the most common associations is found with the spirochaete B. burgdorferi [12,73].

Women are affected more commonly than men. There are numerous clinical presentations of lymphocytoma cutis. Frequently, a firm solitary lesion can be observed although lesions may be clustered in a region or, rarely, be scattered widely. There is usually a nodule or tumour although papules or plaques may also be observed. The colour varies from reddish brown to reddish purple. Scaling and ulceration are absent. Involvement of particular body sites (earlobe, nipple, scrotum) is almost pathognomonic of B. burgdorferi-associated lymphocytoma cutis (Figs 20.27 & 20.28) [73]. The B. burgdorferi-associated type of lymphocytoma cutis often occurs in children and is the most frequent pseudolym-

Hautver Nderung Ohrl Ppchen Borreliose

Fig. 20.27 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Erythematous nodule on the right earlobe.

Lymphoma The Skin
Fig. 20.29 Lymphocytoma cutis. Wedge-shaped infiltrate within the entire dermis. Note small regular germinal centres.

Fig. 20.27 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Erythematous nodule on the right earlobe.

Lymphadenosis Cutis Benigna Nipple

Fig. 20.28 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Erythematous nodule on the right nipple.

  1. 20.30 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Dense diffuse lymphoid infiltrate with prominent follicular structures devoid of a mantle (arrows).
  2. 20.28 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Erythematous nodule on the right nipple.
  3. 20.30 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Dense diffuse lymphoid infiltrate with prominent follicular structures devoid of a mantle (arrows).

phoma in this age group in regions with endemic B. burgdorferi infection.

Histological examination shows dense, nodular, mixed-cell infiltrates, often with the formation of lymphoid follicles (Fig. 20.29). Although the infiltrates may be 'top-heavy', in B. burgdorferi-associated lymphocytoma cutis there are frequently dense diffuse lymphoid infiltrates involving the entire dermis and superficial subcutaneous fat (Fig. 20.30). In addition, in these lesions the reactive germinal centres are commonly devoid of mantle zones and may show confluence simulating the picture of a large B-cell lymphoma (Fig. 20.31) [73,74]. Plasma cells and eosinophils are found in almost all cases as well as a distinct population of T lymphocytes, features that represent useful clues for the differential diagnosis.

Borrelia Burgdorferi Entering Body
Fig. 20.31 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Large blastic cells (centroblasts, large centrocytes) admixed with 'tingible body' macrophages characterized by large empty spaces with nests of apoptotic cells (arrow).

Immunohistology reveals a normal phenotype of germinal centre cells (CD10+, Bcl-6+, Bcl-2-), normal (high) proliferation, and polytypical expression of immunoglobulin light-chains (Figs 20.32 & 20.33). Molecular analysis of the JH gene rearrangement shows a polyclonal pattern in most (but not all) cases [12].

Lymphocytoma cutis may resolve spontaneously in several months or years. Small nodules can be removed by surgical

Fig. 20.33 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Germinal centres with normal (high) proliferation rate. Note absence of mantle and polarization of the staining reflecting the presence of normal dark and light areas within the germinal centres.

excision, and local injection of corticosteroids or interferon-a may result in regression. Cryosurgery has also been applied with success [75]. Patients with lesions of lymphocytoma cutis and evidence of B. burgdorferi (detection of serum antibodies by enzyme-linked immunosorbent assay [ELISA] or immunoblotting or of Borrelia DNA by PCR) can be treated with doxycycline or erythromycin. In refractory lesions, a very effective treatment method is radiotherapy.

Lymphomatoid Contact Dermatitis
Fig. 20.32 Lymphocytoma cutis associated with infection by Borrelia burgdorferi. Polyclonal expression of immunoglobulin light chains kappa and lambda.
Pubic Area Scabies
Fig. 20.34 Typical lesions of nodular scabies on the genital area.

Fig. 20.36 Nodular scabies. Note some atypical lymphocytes (detail of Fig. 20.35).

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