A case of lymphomatoid papulosis type E in a young adult: An uncommon entity.

Lymphomatoid papulosis (LyP) type E is a rare variant of the primary cutaneous CD30+ lymphoproliferative disorders, characterized clinically by large necrotic eschar-like lesions and histopathologically by angiodestructive and angioinvasive infiltrates of CD30+ lymphocytes. As in other forms of lymphomatoid papulosis, type E lesions may undergo spontaneous regression after weeks, with frequent recurrences. We report a 21-year old male with an angiodestructive infiltrate of CD30+ lymphocytes manifesting as a papular eruption rather than ulceration, and suggest that this clinical phenotype might be related to the presence of CD4+ lymphocytes in the inflammatory cell infiltrate.

Lymphomatoid granulomatosis: A case series from South India.

CONTEXT: Lymphomatoid granulomatosis (LYG) is a rare B-lymphoproliferative disorder characterised by an angiocentric and angiodestructive pattern along with Epstein - Barr virus (EBV) association. It is one of the diagnostic challenges in lymphoma pathology. Deregulation of EBV immune surveillance is one of the narrated hypotheses in the literature. Extrapulmonary manifestations are rare with LYG. Morphological grading is done based on the number of EBV-positive B cells, which is useful to strategize treatment protocol. AIMS: We report here a series of nine cases of LYG to discuss the clinical, histological, and immunohistochemistry findings. SETTINGS AND DESIGN: This is the first case series from India in published literature. SUBJECTS AND METHODS: We reviewed cases of LYG diagnosed at our center for the past 11 years (2006-2016). A total of nine cases were included in this study. Histomorphology was studied in conjunction with immunohistochemistry and clinical details. Cases without classical morphology and negative for EBV immunostain were excluded from the study. RESULTS: There were nine patients in our study (7 males and 2 female; M:F ratio 3.5:1). The age of these patients ranged from 4 years to 57 years (mean age: 30 years). The most common site involved was the lung (4, 44%), followed by the skin (2, 22%), central nervous system (2, 22%) and lymph node (1, 11%). One patient had primary immunodeficiency. Another patient had undergone renal transplant 11 years before the development of the lesion. Angiocentricity and angioinvasion were appreciated in all nine cases (9/9) with necrosis in four cases (44%) and ill-defined histiocytic aggregates in three cases (33%). The histological features were as follows: Grade 1(4 cases, 44%), Grade 2(2 cases, 22%), and Grade 3(3 cases, 33%). CONCLUSION: LYG is a rare EBV driven angiodestructive disease with predominantly lung involvement as well as isolated extrapulmonary sites as seen in our study. It is often progressive and ultimately fatal in the absence of appropriate treatment. Grading of the lesion helps to initiate the appropriate treatment of choice.

An unusual case of lymphomatoid papulosis type E with extensive necrosis.

Lymphomatoid papulosis type E (LyP) is a recently described subtype of LyP characterized by an angioinvasive infiltrate of atypical lymphocytes expressing CD30. We present a case of type E LyP with extensive cutaneous necrosis in the histopathological evaluation which was misdiagnosed as an ulcerative form of bacterial skin infection. The remarkable cutaneous necrosis showed in our case might be related to the angiodestructive infiltrate that was present in this circumstance.

Extranodal NK/T-cell lymphoma, nasal type (angiocentric T-cell lymphoma): A review about the terminology.

Extranodal NK/T-cell lymphoma, nasal type (ENKL) is a rare lymphoid neoplasm, which in the past has been grouped with a variety of granulomatous diseases. It is an aggressive non-Hodgkin's type characterized clinically by aggressive, nonrelenting destruction of the midline structures of the palate and nasal fossa. Despite the malignant clinical course, histological diagnosis can be difficult because of extensive tissue necrosis and multiple biopsies that are often required and has an ominous prognosis, as the average survival rate is between 6 and 25 months as reported with a large number of Asian studies. Several American and European studies have shown similar results. This is the case report of a 60-year-old male patient who presented with nasal obstruction and foul smelling, ulcerative lesion over the palate of 6 months duration, which had been treated with antibiotics and anti-inflammatories without success. After performing a number of diagnostic tests, it was found histologically and confirmed by immunohistochemical analysis that the patient had an ENKL, nasal type (also known as angiocentric T-cell lymphoma).