2005
DOI: 10.1111/j.1440-0960.2005.00157.x
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Grzybowski's generalized eruptive keratoacanthoma: Remission with cyclophosphamide

Abstract: A 57-year-old woman presented with intensely pruritic generalized eruptive keratoacanthomas affecting cutaneous and mucosal surfaces (Grzybowski syndrome). She derived marginal benefit from anti-pruritic agents, acitretin and methotrexate. However, cyclophosphamide 100 mg daily for 1 month followed by 3 months at 200 mg daily resulted in remarkable improvement and eventual remission without further treatment. The disease resulted in severe ectropion of upper and lower eyelids bilaterally. Two years after the o… Show more

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Cited by 24 publications
(39 citation statements)
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“…108,109 In three patients unresponsive to acitretin, complete remission was observed after six cycles of monthly CPT (1 g) in two patients who had disease durations of 5-7 years and were also unresponsive to MTX. 110 …”
Section: Generalised Eruptive Keratoacanthoma (Grzybowski Variant)mentioning
confidence: 97%
“…108,109 In three patients unresponsive to acitretin, complete remission was observed after six cycles of monthly CPT (1 g) in two patients who had disease durations of 5-7 years and were also unresponsive to MTX. 110 …”
Section: Generalised Eruptive Keratoacanthoma (Grzybowski Variant)mentioning
confidence: 97%
“…Hier sind systemische Therapien mit Acitretin (Neotigason) ini− tial 0,5 ± 1 mg/kg KG/Tag mit Reduktion auf eine Erhaltungsdosis mit Erfolg eingesetzt worden [8,9] ± im Gegensatz zu der kleine− ren follikulären Variante der KA, die auf die systemische Reti− noidtherapie nicht ansprachen [7]. Des Weiteren werden in der Literatur Remissionen der Erkrankung unter Methotrexat und auch Fallbeispiele mit einer Remission der Erkrankung unter Cy− clophosphamid [3] …”
Section: Apparative Diagnostikunclassified
“…Eine Zunahme der Häufigkeit bei immun− supprimierten Patienten sowie eine Assoziation zu Neoplasien innerer Organe (insbesondere des Intestinaltraktes) wurden ebenfalls beschrieben. Extrem selten ist das disseminiert−exanthemati− sche Auftreten von KA, bei dem verschiedene Va− rianten beschrieben wurden [3,4] Prädilektionsstellen sind hier Nase, Augenlider und insbesonde− re der Handrücken. Ferguson−Smith beschrieb 1934 erstmalig die familiären KA, auf dem Boden autosomal−dominanter Vererbung (Mutation des Gens MSSE ± multiple self−healing squamous epithelioma) kar− tiert auf Genlokus 9q31 [4] mit Vorkommen häufig an chronisch lichtexponierten Arealen [4].…”
unclassified
“…However, his main contribution to international dermatology was the first description in the medical literature of a specific variant of keratoacanthoma (Fig. 5) 27 which has since then been called Grzybowski's eruptive keratoacanthoma or generalized eruptive keratoacanthoma – Grzybowski's variant 28–34 …”
Section: Prominent Polish Dermatologists In the Years 1850–1950mentioning
confidence: 99%