Case Reports

Regional Lymphomatoid Papulosis of the Breast Restricted to an Area of Prior Radiotherapy

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We report the case of a 59-year-old woman with type A regional lymphomatoid papulosis (LyP) that was localized to the left breast, a cutaneous area that had received radiotherapy for treatment of a carcinoma of the breast 5 years prior. This report is a rare example of regional LyP with all lesions located in an area of prior radiotherapy.

Practice Points

  • Cutaneous lesions of lymphomatoid papulosis (LyP) sometimes are confined to only one area of the skin, which is known as regional LyP.
  • Patients with regional LyP have the same prognosis as those with widespread LyP, and no specific association has been reported with this clinical variant.
  • Lesions of regional LyP respond to the same treatments as widespread LyP.


 

References

Lymphomatoid papulosis (LyP) is a clinicopathologic variant of CD30+ primary cutaneous T-cell lymphoproliferative disorder characterized by a chronic, recurrent, self-healing eruption of papules and small nodules. From a clinical point of view, LyP is not considered a malignant disorder despite demonstration of clonality in most cases.1 From a histopathologic point of view, there are 5 types of LyP: (1) type A, the most common type, which is characterized by a wedge-shaped infiltrate composed of clustered large atypical cells admixed with neutrophils, eosinophils, histiocytes, and small lymphocytes; (2) type B, a rare variant characterized by a bandlike infiltrate of small- to medium-sized pleomorphic and hyperchromatic lymphocytes involving the superficial dermis with epidermotropism; (3) type C, which consists of a nodular infiltrate of large atypical cells with a cohesive arrangement closely similar to anaplastic large-cell lymphoma; (4) type D, a variant with histopathologic features that resemble primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, but neoplastic cells express CD30 and a T-cell cytotoxic phenotype (βF1+, CD3+, CD4, CD8+), and follow-up usually does not reveal development of systemic involvement or signs of other cutaneous lymphomas2; and (5) type E, which is characterized by oligolesional papules that rapidly ulcerate and evolve into large, necrotic, escharlike lesions with a diameter of 1 to 4 cm and an angiocentric and angiodestructive infiltrate of small- to medium-sized atypical lymphocytes expressing CD30 and frequently CD8.3

The clinical appearance of LyP usually is polymorphic, with lesions in different stages of evolution scattered all over the skin; however, the lesions are occasionally localized only to one area of the skin, the so-called regional or agminated LyP.4-14 We report a case of regional LyP that exclusively involved the skin of the left breast, which had previously received radiotherapy for treatment of breast carcinoma. Lymphomatoid papulosis with cutaneous lesions involving only an area of irradiated skin is rare.

Case Report

A 59-year-old woman presented with new-onset cutaneous lesions on the left breast. The patient had a history of invasive ductal carcinoma of the left breast, which had been treated 5 years prior with a partial mastectomy and radiotherapy (10 Gy per week for 5 consecutive weeks [50 Gy total]). Physical examination revealed a large nodular lesion with a necrotic surface on the upper half of the left breast as well as 3 small papular lesions with eroded surfaces on the lower half of the breast (Figure 1). A clinical diagnosis of cutaneous metastases from breast carcinoma was suspected.

Figure 1. Three small papular lesions on the left breast (A). Close-up view of a large nodular lesion with a necrotic surface on the upper half of the breast (B).

Biopsies from one small papule and the large nodular lesion showed similar findings consisting of a necrotic epidermis covered by crusts and a wedge-shaped infiltrate involving the superficial dermis (Figure 2A). The infiltrate was mostly composed of large atypical mononuclear cells with oval to kidney-shaped nuclei, prominent nucleoli, and ample basophilic cytoplasm. Many mitotic figures were seen within the infiltrate (Figure 2B). The infiltrate of atypical cells was admixed with small lymphocytes, histiocytes, and some eosinophils. Immunohistochemically, the large atypical cells expressed CD2, CD3, CD4, CD45, CD30, and epithelial membrane antigen (Figures 2C and 2D). A few atypical cells also expressed CD8 and T-cell intracellular antigen 1. Approximately 60% of the nuclei of the atypical cells showed MIB-1 positivity, while CD20, CD56, AE1/AE3, S-100 protein, CD34, and CD31 were negative. The anaplastic lymphoma kinase was not expressed in atypical cells. Monoclonal rearrangement of the γ T-cell receptor was demonstrated on polymerase chain reaction. Physical examination showed no lymphadenopathy in any lymph node chains. Computed tomography of the chest and abdomen failed to demonstrate systemic involvement. On the basis of these clinical, histologic, immunohistochemical, and molecular results, a diagnosis of type A regional LyP was established.

Figure 2. Biopsy demonstrated an ulcerated epidermis and a wedge-shaped infiltrate involving the superficial dermis (A)(H&E, original magnification ×10). Higher magnification demonstrated atypical mononuclear cells with frequent mitotic figures (B)(H&E, original magnification ×400). Immunohistochemical staining of the same biopsy was positive for CD30 (C)(original magnification ×10). Almost all cells of the infiltrate expressed CD30 immunoreactivity (D)(original magnification ×400).

The patient was treated with 2 daily applications of clobetasol propionate cream 0.5 mg/g and 10 mg of oral methotrexate per week for 4 weeks. After 4 weeks of treatment, the lesions on the left breast had resolved leaving slightly atrophic scars. Six months later, an episode of recurrent papular lesions occurred in the same area and responded to the same treatment, but no systemic involvement had been found.

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