The goals of pharmacotherapy are to reduce morbidity and prevent complications. Retinoids - Decreases cohesiveness of abnormal hyperproliferative keratinocytes and may reduce potential for malignant degeneration. Modulate keratinocyte differentiation. Makes keratinocytes in sebaceous follicles less adherent and easier to remove. Etretinate is main metabolite and has demonstrated clinical effects close to those seen with etretinate.
Mechanism of action is unknown.
Keratolytics - Cause cornified epithelium to swell, soften, macerate, and then desquamate. By dissolving the intercellular cement substance salicylic acid produces desquamation of the horny layer of skin, while not affecting structure of viable epidermis. There have been 7 cases reported in the literature of patients with Rothmund-Thomson who developed osteosarcoma. Patients usually have normal intelligence and a normal life span unless affected by the rare development of malignancy. Increased frequency of learning disabilities may require educational attention.