Generalized eruptive keratoacanthoma (Grzybowski variant)

Abstract

We describe a patient with generalized eruptive keratoacanthoma (KA) of Grzybowski showing the characteristic features of this extremely rare condition. Since the first description by Grzybowski in 1950, only 28 additional cases (including the present one) have been reported. This variety of KA most commonly affects patients during the fifth to seventh decade of life and appears as a generalized eruption of hundreds to thousands of follicular papules. The small pruriginous papules often have a keratotic centre and show microscopic features of KA. Marked facial involvement is characteristic and can lead to masked facies with ectropion, as in our patient. The course of the disease is chronic and the response to therapy is poor.

Generalized Eruptive Keratoacanthoma of Grzybowski: Follow-Up of the Original Description and 50-Year Retrospect

“…An analysis of 29 published cases showed patients to be middle aged or older, with an age of onset ranging from 32 to 84 years (mean age 57 years) [7, 9]. No familial pattern has been identified; cases appear to be sporadic.…”

Treatment of multiple keratoacanthomas with erlotinib

“…multiple, eruptive, and keratoacanthoma centrifugum marginatum--have been described. In the eruptive type, there is a rapid, diffuse development of dome-shaped papules with a keratatotic center [1][2][3]. Lesions may number in the tens or hundreds, and are often intensely pruritic.…”

Eruptive Keratoacanthoma of Grzybowski

“…Поскольку множественные кератоаканто-мы могут быть проявлением синдрома Мюир -Торре, при эруптивной кератоакантоме необхо-димо исключение рака внутренних органов [12].…”

“…Предполагается, что эффективность примене-ния ретиноидов при этом типе кератоакантомы связана с их способностью понижать кератини-зацию и модулировать терминальную диффе-ренцировку эпидермальных клеток, уменьшать уровень ИЛ-2 и митоген-индуцированную про-лиферацию лимфоцитов [12].…”