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Author: Dr Amanda Oakley, Dermatologist, Waikato Hospital, Hamilton, New Zealand, 2004.
Generalised eruptive keratoacanthomas (Grzybowski syndrome) refers to a very rare disease in which hundreds of keratoacanthoma-like papules appear.
It should be distinguished from an inherited disorder, multiple self-healing squamous epitheliomas of Ferguson-Smith, in which multiple keratoacanthomas appear in children and young adults. Witten and Zak described a similar condition in which there were giant keratoacanthomas.
Only about 30 cases of generalised eruptive keratoacanthomas have been written up in medical journals worldwide. The syndrome affects middle-aged adult males and females equally.
The cause is unknown. Because in some ways these lesions look and behave like viral warts, several investigators have looked for evidence for human papillomavirus within the lesions. The results have been inconclusive, but in most cases, no evidence of viral infection has been found.
The patient with generalised eruptive keratoacanthomas presents with multiple itchy papules and nodules, which start abruptly and increase in number over some months. The main features are:
An individual papule or nodule seen in generalised eruptive keratoacanthomas resembles a keratoacanthoma.
Skin biopsy reveals:
Most individuals with generalised eruptive keratoacanthomas are otherwise in good health, and blood test results are normal.
Treatment fails for many patients with generalised eruptive keratoacanthomas. Only one case has been reported to clear up spontaneously. In other cases, improvement or complete resolution has occurred with one of the following medications:
Symptoms can be eased with emollients and anti-itch medications.
Untreated, it is reported to persist indefinitely.
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