Translate this page into:
Primary cutaneous b-cell lymphoma
V R Janaki
I-D, Lakshmi Apartments, 122, Fourth Street, Abhiramapuram, Chennai-600 018
|How to cite this article:
Bhat P I, Janaki C, Sentamilselvi G, Janaki V R. Primary cutaneous b-cell lymphoma. Indian J Dermatol Venereol Leprol 2003;69:22-23
AbstractA 54-year-old man, presented with leonine facies and multiple tumours more over the exposed parts of the body. On investigation, it turned out to be a case of primary cultaneous B-cell lymphoma with a distrinctive histopathology. Chemotherapy was given with a good therapeutic response.
Cutaneous lymphomas, arising from T or B lymphocytes, constitute an uncommon but nevertheless important entity with the so called Skin Associated Lymphoid Tissue (SALT) being either the origin (primary) or the target (secondary) in the neoplastic process. The primary cutaneous lymphomas, by definition, present clinically on the skin with no extracutaneous disease at the time of diagnosis or for 6 months thereafter.2 Of these, primary cutaneous lymphomas of B cell origin are less common, their incidence being one third that of their T-cell counterpart. Although several classifications like Kiel, Lukes and Collins, Rappaport and Revised European and American Lymphoma (REAL) classification have been used, the one proposed by the European Organisation for Research and Treatment of Cancer (EORTC) is the latest and being followed widely. The follicular centre cell type described in the EORTC classification is the commonest type of primary cutaneous B cell lymphoma and shows a characteristic histopathology5 and expression of cell surface markers like CD 19, CD 20, CD 79a, one such case is described below.
A 54-year-old man who been a road layer for 30 years presented with one year history of progressively enlarging swellings over the face, chest and forearms. Lesions were mildly photosensitive.
On examination, multiple tumour masses of firm consistency were found infiltrating the forehead and malar areas to produce a leonine appearance [Figure - 1]. Overlying skin was hyperpigmented with peu-de-orange appearance. Similar lesions were found on the neck, upper back and dorsal aspect of forearms. No significant lymphadenopathy was detected clinically.
Histopathology of involved skin showed a normal epidermis with absence of epidermotropism. A clear subepidermal cell free Grenz zone was seen in the papillary dermis. The mid and lower dermis were diffusely infiltrated by small, round lymphocytes in a bottom heavy pattern [Figure - 2]. Radiological and bone marrow aspiration studies revealed no extracutaneous pathology.
Immuno histochemical techniques for cell markers was done. A final diagnosis of primary cutaneous B cell lymphoma of follicular centre cell type was made.
Chemotherapy with use of cyclophosphamide, adriamycin, oncovin and prednisolone was started. An excellent response with near total resolution of lesions was seen [Figure - 3]. Patient is being followed up at regular intervals.
The follicular centre cell type of primary cutaneous B cell lymphoma with its classical histopathology and cell markers, as seen in this case has a favourable prognosis with good therapeutic response, a fact well documented in literature., Interestingly, with the patient′s history of life long exposure to coal tar and sunlight, and with the distribution of lesions being limited to the exposed areas of the body, the possibility of these environmental factors playing a carcinogenic role in primary cutaneous B cell lymphoma, although hitherto unreported, needs be further explored.
Giannotti B, Santucci M. Skin associated lymphoid tissue (SALT) related B cell lyphoma (primary cutaneous B cell lymphoma) : A concept and clinicopathological entity. Arch Dermatol 1993; 129:353-355.[Google Scholar]
Sterry W, John S. Other systemic lymphomas with skin infiltrates. In: Freeedberg IM, Fitzpatrick TB, et al, edrs. Dermatology in General Medicine. 5th edn. New York: Mc Graw Hill, 1999 : 1250-1258.[Google Scholar]
Willemze R, Kerl H, Sterry W, et al. EORTC classification of primary cutaneous lymphomas : A proposal from the cutaneous lymphoma study group of the European Organization for Research and Treatment of Cancer. Blood 1997; 90:354-371.[Google Scholar]
Harris NL, Jaffe ES et al. A revised European - American Classification of lymphoid neoplasms : A proposal from the International Lymphoma Study Group. Blood 1994; 84:1361.[Google Scholar]
Mackie RM. Cutaneous lymphomas and lymphocytic infiltrates. In : Champion RH, Burton JL, et al, edrs. Textbook of Dermatology. 6th edn. London : Blackwell Science Ltd, 1998 : 2393-2395.[Google Scholar]
Willemze R. Sentis HJ, Et al. Primary cutaneous large cell lymphoma of follicular centre cell origin. J Am Acad Dermatol 1987; 16L518-526.[Google Scholar]
Rijlaarsdam JV et al. : Treatment of Primary cutaneous B Cell Lymphoma of follicular centre cell origin - A clinical follow-up of 55 patients treated with radiotherapy of polychemotherapy. Journal of Clinical Oncology 1996; 14:549.[Google Scholar]