Lymphomatoid granulomatosis C83.8

Very rare, granulomatous systemic disease that belongs to the B-cell proliferative diseases with varying malignant potential. The disease is considered an angiocentric and destructive lymphoproliferative disease. Histopathologically, there is an EBV association (LMP1) and expression of the surface markers CD20 and CD30. The most common organ manifestations are lung, nervous system, kidney, liver and skin (Roschewski M et al. 2012).

Usually occurs after the 50th LJ. Men are affected about twice as often as women. Childhood cases have been reported.

The clinical picture of the skin lesions is uncharacteristic. Skin lesions occur in about 45% of patients: mostly painless reddish-brown spots, papules, plaques or nodules. Rarely erythema nodosum-like nodules with tendency to ulceration.

Extracutaneous manifestations:

Pulmonary: cough, shortness of breath, chest pain, fever, weight loss, CNS involvement (in 26% of pat.) headache, ataxia, hemiplegia, convulsions.

Other organ involvement: liver and kidney

The lungs and skin show angiocentric and angiodestructive granulomatosis with vascular proliferates, furthermore atypical lymphocytes, plasma cells and histiocytes; infiltration of skin appendages and nerves. No epidermotropism.

Variable course, spontaneous remissions possible. Often development of an aggressive B-cell lymphoma. Death usually due to respiratory insufficiency.

Lymphomatoid granulomatosis is a disease in which the skin alone provides monitoring signs of the granulomatous systemic disease.

The signs of systemic disease (lung, kidney, CNS, liver) must be detected early in order to provide adequate therapy.

Skin biopsy will be able to confirm granulomatous lymphocytic systemic disease.

A 62-year-old female patient came for clarification of a non-painful reddish-brownish plaque on the inside of the left knee. The lesion had existed for about 8 months with a tendency to grow in size. At the same time, persistent coughing, recurrent fever, weight loss, and significantly increased inflammatory parameters indicated a systemic disease. EBV-titer serologically positive.

During the internal medical examination, an unclear pulmonary mass was admitted to hospital. The transbronchial biopsy did not yield any trend-setting findings. As a result, a surgical intervention was performed under the mainly bronchial carcinoma. The histopathological analysis of the resectate finally showed evidence of a lymphomatoid granulomatosis in stage 1-2. Molecularly, an EBV infection could be confirmed.

The histological comparison of skin and lung biopsy revealed a comparable tissue pattern.

1. Beaty MW et al (2001) Cutaneous lymphomatoid granulomatosis: correlation of clinical and biologic features. Am J Surg Pathol 25: 1111-1120
2. Cachat F et al (2003) Lymphomatoid granulomatosis in a renal transplant patient. Pediatr Nephrol 18: 838-842
3. Caussade P et al (1985) Lymphomatoid granulomatosis (lymphomatoid vasculitis) with onset in the skin. Phleb. Proct. 14: 121-128
4. Dunleavy K et al (2012) Lymphomatoid granulomatosis and other Epstein-Barr virus associated lymphoproliferative processes. Curr Hematol Malig Rep 7:208-215
5. Gangar P et al (2015) Granulomatous lymphoproliferative disorders: granulomatous slack skin and lymphomatoid granulomatosis. Dermatol Clin 33:489-496
6. Jenkins TR et al (1989) Lymphoid granulomatosis: a case for aggressive therapy. Cancer 64: 1362-1365
7. Liebow AA, Carrington CR, Friedman PJ (1972) Lymphomatoid granulomatosis. Hum Pathol 3: 457-558
8. Roschewski M et al (2012) Lymphomatoid granulomatosis. Cancer J 18:469-474.
   Sebire NJ et al (2003) Isolated EBV lymphoproliferative disease in a child with Wiskott-Aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy. J Clin Pathol 56: 555-557
9. Tacke ZC et al (2014) Childhood lymphomatoid granulomatosis: a report of 2 cases and review of the literature. J Pediatr Hematol Oncol 36:e416-422