Cutaneous lymphomatoid granulomatosis: a rare cause of recurrent skin ulceration

We report the case of a 34-year-old male who presented with recurrent skin ulceration due to lymphomatoid granulomatosis. Monoclonal antibody studies showed a high T helper (T4):T suppressor (T8) cell ratio in both the lesions and the peripheral blood. Plasma cells in the infiltrate showed IgA lambda staining but as yet no monoclonal immunoglobulin has been identified in the blood or urine. Lymphocytes bearing markers of immature cells (T10) were observed in the tissue and blood.     

Cutaneous lesions of lymphomatoid granulomatosis

Lymphomatoid granulomatosis is a rare multisystem disease involving the lungs, skin, and nervous system. The cutaneous disease may be the presenting complaint. A case with nodular and annular infiltrated lesions is reported to encourage the inclusion of lymphomatoid granulomatosis in the differential diagnosis of similar lesions. Prompt diagnosis and early long-term treatment with cyclophosphamide and prednisone can lead to prolonged remissions of this disease. If relapse occurs, prompt treatment with the same regimen may again induce a remission.     

Cutaneous signs of lymphomatoid granulomatosis

A retrospective review of 47 cases of lymphomatoid granulomatosis encountered between 1977 and 1990 revealed that 24 patients had skin involvement. Clinical and histologic features were noted. The dominant and most characteristic cutaneous finding was scattered nodules. Other cutaneous changes included eroded and crusted lesions, facial edema, papules, and folliculitis-like eruptions. The nodular skin lesions most often had an angiocentric and angiodestructive dense, polymorphous, lymphohistiocytic infiltrate in the dermis and fat. A minority of the cases had a lymphoma cutis type of infiltrate. Immunophenotypic analysis showed a mixed population of T cells, with helper T cells (CD4+) predominant. Our results emphasize the usefulness of the clinical and histologic examination of the skin in lymphomatoid granulomatosis. The histologic pattern of lymphomatoid granulomatosis was seen in the skin in more than half of the biopsy specimens.     

Successive legionellosis in the course of lymphomatoid granulomatosis

A 75-year-old male presented with typical cutaneous lesions and pulmonary involvement due to lymphomatoid granulomatosis. A legionellosis was associated with this condition. Though the radiological findings in lymphomatoid granulomatosis are suggestive, they are not specific, and set a difficult problem in distinguishing the two conditions. Monoclonal antibody studies indicated the predominance of CD8 T lymphocytes in the infiltrate of the skin, as well as in the bronchoalveolar fluid.     

Cartilage hair hypoplasia with cutaneous lymphomatoid granulomatosis

Cartilage–hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short‐stature, sparse hair and impaired cellular immunity. We describe a young girl who was diagnosed with CHH based on the findings of recurrent infections, short stature with metaphyseal chondrodysplasia, and a confirmed bi‐allelic RMRP gene mutation. At 13 years, the patient developed an Epstein–Barr virus (EBV)‐driven lymphoproliferative disorder involving the lung, which responded partially to chemotherapy. Simultaneously, she developed multiple indurated plaques involving her face, which had histological findings of granulomatous inflammation and EBV‐associated low‐grade lymphomatoid granulomatosis. The patient received a matched unrelated peripheral blood stem cell transplant at 15 years of age, and her immunological parameters and skin lesions improved. Lymphomatoid forms of granulomatosis and cutaneous EBV‐associated malignancies have not been described previously in CHH. This case highlights the possibility of EBV‐associated cutaneous malignancy in CHH.     

Primary and isolated cutaneous lymphomatoid granulomatosis following heart-lung transplantation

BACKGROUND: Lymphomatoid granulomatosis is an Epstein-Barr virus-associated B-cell lymphoproliferative disease. It is angiocentric and angiodestructive and involves the lungs, central nervous system and skin. Exclusive cutaneous involvement is rare and may be associated with a better outcome. Contrarily to the extra-cutaneous forms of lymphomatoid granulomatosis, it is difficult or impossible to detect Epstein-Barr virus DNA sequences in primary and isolated cutaneous lymphomatoid granulomatosis. CASE REPORT: A 54-year-old woman developed erythemato-violaceous lesions on both legs 3 years after a heart-lung transplantation. The diagnosis of erythema multiforme and of drug-induced vasculitis were first made. Because of fever and of the rapid extension of the lesions, the patient was hospitalized. The histologic examination of the first lesions showed a perivascular infiltrate, without epidermotropism, composed of histiocytes, lymphocytes and plasma cells. Immunohistochemistry revealed the presence of a predominantly T-cell infiltrate with some large B cells. Subsequent biopsies were diagnosed as high grade B-cell lymphoma. Polymerase chain reaction analysis as well as in situ hybridation study showed the presence of Epstein-Barr virus load in the lesions. There was however no serologic evidence of viral reactivation. Extensive systemic evaluation revealed no visceral or bone marrow involvement. Despite antiviral treatment and CHOP polychemotherapy, the patient died 3 months after her admission. DISCUSSION: This observation of lymphomatoid granulomatosis is particular because of its exclusive cutaneous involvement associated with a fulminant evolution to high grade B lymphoma. The presence of a context of iatrogenic immunosuppression underlies the role of altered immune cellular functions in the initiation and/or progression of lymphomatoid granulomatosis and strengthens the role of a viral agent in its pathogenesis. We suggest that the presence of Epstein-Barr virus, which is generally not associated with the isolated cutaneous forms of lymphomatoid granulomatosis, may have played a role in this fulminant evolution to high grade B lymphoma.     

Posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis in a lung transplant patient

A 58-year-old lung transplant patient developed worsening shortness of breath and indurated erythematous plaques on the lower left leg. A skin biopsy specimen revealed a dense angiocentric and angioinvasive infiltrate in the mid to reticular dermis and panniculus containing large, atypical lymphocytes with convoluted nuclei and prominent nucleoli. Immunohistochemical stains showed that the atypical cells were of B-cell origin, and that Epstein-Barr virus was present. Molecular studies demonstrated B-cell clonality. The patient was successfully treated with rituximab. The clinical and pathologic features in this case represent posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis. The case also highlights the importance of clinical and pathologic examination of cutaneous lesions in the evaluation of lymphoproliferative disorders for patients undergoing transplantation.