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Caracterización de procesos

7. MÒDULO DE ORGANIZACIÒN

7.2. ENFOQUE DE GESTIÒN BASADO EN PROCESOS

7.2.2. Caracterización de procesos

Lymphocytoma cutis

ing of gold pierced earrings, medicinal leech therapy and

ing of gold pierced earrings, medicinal leech therapy and

tattoos [70–72]. One of the most common associations is

tattoos [70–72]. One of the most common associations is

found with the

found with the spirochaetespirochaeteB. burgdorferiB. burgdorferi[12,73].[12,73].

Women are affected more commonly than men. There are

Women are affected more commonly than men. There are

numerous clinical presentations of lymphocytoma cutis.

numerous clinical presentations of lymphocytoma cutis.

Frequently, a firm solitary lesion can be observed although

Frequently, a firm solitary lesion can be observed although

lesions may be clustered in a region or, rarely, be scattered

lesions may be clustered in a region or, rarely, be scattered

widely. There is usually a nodule

widely. There is usually a nodule or tumour although papulesor tumour although papules

or plaques may also be observed. The colour varies from

or plaques may also be observed. The colour varies from

reddish brown to reddish purple. Scaling and ulceration

reddish brown to reddish purple. Scaling and ulceration

are absent. Involvement of particular body sites (earlobe,

are absent. Involvement of particular body sites (earlobe,

nipple, scrotum) is almost pathognomonic of

nipple, scrotum) is almost pathognomonic of B. burgdorferiB. burgdorferi--

associated lymphocytoma cutis (Figs 20.27 & 20.28) [73].

associated lymphocytoma cutis (Figs 20.27 & 20.28) [73].

The

The B. burgdorferiB. burgdorferi-associated type of lymphocytoma cutis-associated type of lymphocytoma cutis

often occurs in children and is the

often occurs in children and is the most frequent pseudolymmost frequent pseudolym-- Fig. 20.24

Fig. 20.24 Lymphomatoid drug eruption, T-cell type. Patchy lichenoidLymphomatoid drug eruption, T-cell type. Patchy lichenoid

infiltrate of lymphocytes without epidermotropism within the

infiltrate of lymphocytes without epidermotropism within the

superficial dermis.

superficial dermis.

Fig. 20.25

Fig. 20.25 Lymphomatoid drug eruption, T-cell type. Note severalLymphomatoid drug eruption, T-cell type. Note several

atypical lymphocytes and one mitotic figure (detail of Fig. 20.24).

atypical lymphocytes and one mitotic figure (detail of Fig. 20.24).

Fig. 20.26

Fig. 20.26 Lymphomatoid drug eruption, B-cell type. NodularLymphomatoid drug eruption, B-cell type. Nodular

infiltrates of lymphocytes with reactive germinal centres. (Courtesy of

infiltrates of lymphocytes with reactive germinal centres. (Courtesy of

Dr Dieter Metze, Münster, Germany.)

Dr Dieter Metze, Münster, Germany.)

Fig. 20.23

Fig. 20.23 Lymphomatoid drug eruption. Papules, plaques andLymphomatoid drug eruption. Papules, plaques and

nodules on the back.

Pseudolymphomas of the Skin

Pseudolymphomas of the Skin 167167

phoma in this age group in regions with endemic

phoma in this age group in regions with endemicB. burgdor-B. burgdor-

 feri

 feriinfection.infection.

Histological examination shows dense, nodular, mixed-

Histological examination shows dense, nodular, mixed-

cell infiltrates, often with the formation of lymphoid follicles

cell infiltrates, often with the formation of lymphoid follicles

(Fig. 20.29). Although the infiltrates may be ‘top-heavy’,

(Fig. 20.29). Although the infiltrates may be ‘top-heavy’,

in

in B. burgdorferiB. burgdorferi-associated lymphocytoma cutis there are-associated lymphocytoma cutis there are

frequently dense diffuse lymphoid infiltrates involving the

frequently dense diffuse lymphoid infiltrates involving the

entire dermis and superficial subcutaneous fat (Fig. 20.30).

entire dermis and superficial subcutaneous fat (Fig. 20.30).

In addition, in these lesions the reactive germinal centres are

In addition, in these lesions the reactive germinal centres are

commonly devoid of mantle zones and may show confluence

commonly devoid of mantle zones and may show confluence

simulating the picture of a

simulating the picture of a large B-cell lymphoma (Fig. 20.31)large B-cell lymphoma (Fig. 20.31)

[73,74]. Plasma cells and eosinophils are found

[73,74]. Plasma cells and eosinophils are found in almost allin almost all

cases as well as a distinct population of T lymphocytes, fea-

cases as well as a distinct population of T lymphocytes, fea-

tures that represent useful clues for the differential diagnosis.

tures that represent useful clues for the differential diagnosis. Fig. 20.27

Fig. 20.27 Lymphocytoma cutis associated with infection byLymphocytoma cutis associated with infection byBorreliaBorrelia

burgdorferi 

burgdorferi . Erythematous nodule on the right earlobe.. Erythematous nodule on the right earlobe.

Fig. 20.28

Fig. 20.28 Lymphocytoma cutis associated with infection byLymphocytoma cutis associated with infection byBorreliaBorrelia

burgdorferi 

burgdorferi . Erythematous nodule on the right nipple.. Erythematous nodule on the right nipple.

Fig. 20.30

Fig. 20.30 Lymphocytoma cutis associated with infection byLymphocytoma cutis associated with infection byBorreliaBorrelia

burgdorferi 

burgdorferi . Dense diffuse lymphoid infiltrate with prominent follicular. Dense diffuse lymphoid infiltrate with prominent follicular

structures devoid of a mantle (arrows).

structures devoid of a mantle (arrows).

Fig. 20.29

Fig. 20.29 Lymphocytoma cutis. Wedge-shaped infiltrate within theLymphocytoma cutis. Wedge-shaped infiltrate within the

entire dermis. Note small regular germinal centres.

Immunohistology reveals a normal phenotype of germinal centre cells (CD10+

, Bcl-6+

, Bcl-2–), normal (high) prolifera-

tion, and polytypical expression of immunoglobulin light- chains (Figs 20.32 & 20.33). Molecular analysis of the JHgene 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

excision, and local injection of corticosteroids or interferon- αmay 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 anti- bodies 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.

Fig. 20.31 Lymphocytoma cutis associated with infection byBorrelia burgdorferi . Large blastic cells (centroblasts, large centrocytes) admixed with ‘tingible body’ macrophages characterized by large empty spaces with nests of apoptotic cells (arrow).

Fig. 20.32 Lymphocytoma cutis associated with infection byBorrelia burgdorferi . Polyclonal expression of immunoglobulin light chains kappa and lambda.

Fig. 20.33 Lymphocytoma cutis associated with infection byBorrelia 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.

Pseudolymphomas of the Skin 169

The most typical example of this group of lymphomatoid infiltrates is nodular scabies but many other arthropods can induce skin lesions that may simulate malignant lymphoma histopathologically. Clinically, in nodular scabies, elevated round or oval bright reddish papules and nodules occur most frequently on the genitalia, elbows and in the axillae (Fig. 20.34). The lesions are found in approximately 7% of patients with scabies. The nodules are very pruritic and may persist for many months.

The mite and its parts are seldom identified in the long- standing papules or nodules of scabies. The clinical differen- tial diagnosis includes prurigo nodularis and malignant

Persistent nodular arthropod