By Gary M. White, MD

Progressive Symmetric Erythrokeratoderma (PSE) or Gottron's syndrome is a rare disorder of well-demarcated erythematous and hyperkeratotic plaques.


There is the onset in early childhood of symmetrically distributed erythematous and hyperkeratotic plaques on the head, extremities, and buttocks.

Differential Diagnosis

Similar entities include erythrokeratodermia variabilis, psoriasis, and pityriasis rubra pilaris.


An 18-year-old man presented with mildly itchy, red, raised scaly lesions over face, neck, trunk, and limbs since he was one year old. Lesions started from trunk and gradually increased in size and number to involve face and limbs till the time of puberty, after which the disease stabilized. The lesions were persistent and non-migratory. There was no seasonal variation and no other family member was involved. Indian J Dermatol 2014;59:317

A 15-year-old boy born of second-degree consanguineous marriage presented with asymptomatic scaly skin lesions on the extremities since 5 years of age. Lesions initially started on dorsum of feet, and gradually progressed to involve knees and hands. These lesions were persistent and nonmigratory. There was no history of similar skin eruptions in the family or siblings. Indian Dermatology Online Journal 2013 : 4 : 347-349


Homepage | FAQs | Use of Images | Contact Dr. White

It is not the intention of to provide specific medical advice, diagnosis or treatment. only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.