Miliary and agminated-type primary cutaneous follicle center lymphoma: Report of 18 cases

Background: Primary cutaneous follicle center lymphoma (PCFCL) presents usually with reddish nodules, plaques, and tumors on the head and neck area, particularly the scalp, and on the back. Objective: We sought to describe a peculiar clinical variant of PCFCL. Methods: We report a series of 18 patients (male:female = 7:11; median age 52 years; mean age 50.8 years; age range 27-75 years) with a clinical variant of PCFCL characterized clinically by multiple, miliary, or agminated papules predominantly on the head and neck. Results: All patients presented with multiple erythematous, firm papules arranged in a manner that resembled millet seeds or collected together in small clusters. Lesions were located on the entire face in one patient (5.6%), the forehead in 8 (44.4%), the cheeks in 3 (16.7%), the preauricular region in two (11.1%), and multiple regions on the head and neck area in 3 (16.7%). The last patient had miliary papules on the entire face, back, upper aspect of arms, and scattered on the front of the chest. The initial diagnosis was never cutaneous lymphoma, and all patients had been treated unsuccessfully for different skin conditions including mainly rosacea, lupus miliaris disseminatus faciei, and persistent arthropod bite reaction. Microscopic examination confirmed the diagnosis of PCFCL in all patients. Limitations: Small number of cases and retrospective study are limitations. Conclusions: This peculiar clinical presentation of PCFCL is unusual and represents a pitfall in the clinical diagnosis. Dermatologists should be aware of this variant of PCFCL so as to treat patients timely and properly. (J Am Acad Dermatol 2011;65:749-55.)