INTRODUCTION:Lymphomatoid granulomatosis (LGM) is a rare diagnosis which often presents with a variety of seemingly unrelated symptoms such as multiple pulmonary nodules, subcutaneous skin nodules which erupt and necrose, and central nervous system (CNS) involvement. Its protean manifestation can delay diagnosis or result in misdiagnosis, and confound clinicians.
CASE PRESENTATION:We present a case of a 29 year old male who presented to our institution with a chief complaint of post-prandial left upper quadrant pain, new dyspnea on exertion, and multiple skin lesions which had erupted over a period of weeks on his legs and face. Our patient is a fit, otherwise healthy male with no prior medical history and taking no medications, whose symptoms originated roughly two weeks prior to presentation. On review of systems the patient denied fevers, chills and nightsweats, but noted an 8 lb weight loss over two months. Exam was notable for numerous crusted erythematous papules on his scalp, chin, left pinna, chest, abdomen and thighs.Except for mildly elevated ESR and LDH (23 and 247, respectively), labs were unremarkable. Diffuse bilateral lung zone opacities and bihilar adenopathy were noted on chest radiograph. PET-CT showed widely diffuse areas of metabolic uptake to include the bilateral lung parenchyma, left hilum, liver, spleen, and subcutaneous tissues of the face and extremities. Biopsies of the lesions of the lower extremities on two occasions failed to yield a definitive diagnosis.We opted to refer our patient to cardiothoracic surgery for wedge biopsy to secure a diagnosis. A biopsy of the lingula yielded the diagnosis of lymphomatoid granulomatosis.
DISCUSSIONS:First described by Leibow in 1972, LGM is a rare diagnosis with roughly 500-600 case reports in the literature. It is a lymphoproliferative disorder of B-cell lineage with a predilection for males (3:1), typically presenting in ages 30-50. Histopathologically the disorder presents itself as an infiltrative disease of multiple organ systems (skin, lung, CNS, and renal primarily) with atypical lymphocytoid and plasmacytoid cells in conjunction with granulomatous inflammation. It takes its unwieldy name because of its characteristics of both a granulomatous process and lymphoproliferative disorder. The diagnosis of LGM requires the histopathologic demonstration of polymorphic lymphoid infiltrates with focal areas of necrosis and transmural vascular infiltration by pathologic B cells and reactive T cells. From a pulmonary perspective, the disorder presents with multiple bilateral nodules with a predilection for the mid and lower lung zones, generally without hilar or mediastinal adenopathy. Symptoms would include insidious dyspnea and concomitant constitutional symptoms such as weight loss. Treatment for this disorder is based on corticosteroids and cyclophosphamide, with rituximab and interferon alfa sometimes added. The overall prognosis is poor, with a median survival of fourteen months.
CONCLUSION:Lymphomatoid granulomatosis is a disorder that clinically and radiographically takes the form of numerous other pulmonary disease processes such as sarcoidosis, other infectious granulomatous diseases, vasculitides, and malignant disorders such as lymphoma. The differential diagnosis can be large and difficult to pare down in spite of extensive work ups. Because of its protean manifestation and ability to foil the diagnostician, the disease requires a high degree of suspicion and a fair amount of inquisitive tenacity to make the diagnosis and not fall into the trap of misdiagnosis.
DISCLOSURE:Timothy Quast, No Financial Disclosure Information; No Product/Research Disclosure Information