Erythroderma is a generalized exfoliative dermatitis that is characterized by erythema and scaling of larger than 85% of the body surface. It often occurs secondary as exacerbation of preexisting dermatosis, e.g. psoriasis or atopic dermatitis, or as a drug reaction. The group of rare conditions that can cause erythrodermia is very heterogenous and vary by populations and ages. Pityriasis rubra pilaris is an extremely rare skin disease. Its incidence has been reported to be 1 per 3500-5000 patients presenting to the dermatologist (16). The reddish orange colour of erythema, keratotic follicular papules, distinct areas of uninvolved skin, the so-called islands of sparing on the body and extremities and palmoplantar keratoderma can help to distinguish RPR from other diseases. None of the above signs are diagnostic and as histological features of RPR can be absent the diagnosis is always based on both clinical and morphological features.