Angioinvasive (type E) lymphomatoid papulosis (LyP) is a recently described subtype of LyP presenting with eschar-like lesions that can be mistaken for aggressive forms of angiocentric cutaneous T-cell lymphoma. None of the cases of angioinvasive LyP described thus far have been associated with mycosis fungoides (MF). Herein, we describe a case of angioinvasive LyP type E coexisting with MF. The patient presented with an eschar on his chest and over time developed new nodules and large plaques with eschar formation, all of which resolved spontaneously over a period of a few weeks without intentional therapy. Biopsy revealed a CD30+ atypical inflammatory cell infiltrate with marked angiocentricity. Later, he developed erythematous annular scaly patches histologically consistent with MF. Our patient's clinical course confirms the indolent behavior characteristic of LyP despite the aggressive clinical and histologic appearance of lesions. The co-occurrence of angioinvasive LyP and MF in our patient highlights the propensity for LyP type E to coexist with MF, as is characteristic of other LyP subtypes, and supports the theory that LyP and MF are related T-cell lymphoproliferative disorders. Patients with LyP can present with large lesions exhibiting eschar formation and an atypical angiocentric/angiodestructive lymphoid infiltrate and should be spared overtreatment.
Extramammary Paget disease (EMPD) is a rare intraepithelial carcinoma and an uncommon variant of Paget disease affecting areas of the apocrine-rich skin of the perineum, vulva, and less commonly, axilla. Women in their sixth to eighth decades are commonly affected. It is exceedingly rare for EMPD to present on the face, chest, abdomen, or other nonapocrine sites and even more unusual for EMPD to present as a pigmented lesion. The relationship between Paget cells in pigmented extramammary Paget disease (PEMPD) and reactive proliferation and colonization by melanocytes has been poorly explored. The relevance of this rare entity resides in its potential to be misdiagnosed clinically and histopathologically as malignant melanoma in situ. Therefore, application of a panel of immunostains and careful analysis and interpretation of these findings are essential to arrive at the correct diagnosis. We report a new case of PEMPD on a nonapocrine site. The specimen was examined by routine microscopy including hematoxylin and eosin stain as well as immunostains. Histologic examination revealed characteristic features of PEMPD confirmed with immunohistochemical stains.
Exaggerated insect bite reactions present as exuberant papular and vesiculobullous lesions in patients with hematologic malignant neoplasms. The most commonly reported hematological malignancy with exaggerated insect bite reactions is chronic lymphocytic leukemia. Other reported reactions have been seen in patients with mantle cell lymphoma. Clinical presentation may vary with many patients unaware of a previous insect bite. Therefore, the terms “exaggerated bite-like reaction” and “eosinophilic dermatosis of myeloproliferative disease” have been proposed. The lesions may wax and wane or persist for many years, and they seem to be unrelated to the underlying hematologic disorder course or therapy. In some circumstances, the lesions may actually precede the diagnosis of the hematological malignancy. In this study, we describe a case of “eosinophilic eruption of myeloproliferative disease” associated with diffuse large B-cell lymphoma in a young adult. To the best of our knowledge, this is the first case reported in association with diffuse large B-cell lymphoma.
Langerhans cell histiocytosis (LCH) is an uncommon disorder characterized by proliferation of abnormal LCs usually affecting children and adolescents. LCH in adults first presenting in the skin is rare. Although LCH and even LCH with a second malignancy may be more common in children, cutaneous LCH with a second hematologic malignancy has been more commonly identified in adults. The authors report 2 new cases of LCH in adult patients with underlying myelodysplasia and follicular lymphoma. The specimens were examined by routine microscopy and immunohistochemical stains for S100 protein and CD1a. Patients were elderly men with established diagnoses of follicular lymphoma and myelodysplasia, presented with follicular lesions and erythematous plaques involving intertriginous areas. Histologic examination revealed collections of mononuclear cells in upper dermis, which demonstrated strong positivity for S100 and CD1a, confirming their identity as LCs. BRAF analysis returned negative for detection of BRAF V600E mutation in both patients. The authors have recently encountered 2 cases of adult patients with skin-limited LCH predated by other lymphoproliferative disorders. The association between LCH and hematopoietic disorders may be explained by a common bone marrow precursor that is differentiating along different cell lines. Cutaneous LCH may be associated with underlying lymphoproliferative disorders and should be considered in the differential diagnosis of cutaneous eruptions in patients with hematopoietic disorders. Clinical follow-up evaluation of patients diagnosed with LCH for peripheral blood abnormalities and lymphadenopathy or "B symptoms" may be prudent in patients not already carrying a diagnosis of an underlying hematologic disorder.
A 66-year-old man presented with a 1.5-cm subcutaneous blue nodule on the vertex of his scalp gradually increasing in size over several years (Fig 1). Excisional biopsy found dilated duct-like structures forming intraluminal papillation of variable complexity with loss of myoepithelial layers. Lumina were filled with amorphic eosinophilic material and histiocytes. The lining cells were atypical with prominent nucleoli (Figs 2 and 3). By immunohistochemistry, the cells were positive for AE1/AE3, GATA-3, p63, cytokeratin 7, EMA, and CEA. Estrogen receptor, progesterone receptor, CD117, CK5/6, and TTF-1 immunostains were negative.
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