Generalized eruptive keratoacanthoma: proposed diagnostic criteria and therapeutic evaluation

Abstract

Generalized eruptive keratoacanthoma (GEKA) of Grzybowski is a sporadically occurring, extremely rare variant of keratoacanthoma characterized clinically by severely pruritic, generalized eruption of numerous small follicular papules, often with a central keratotic plug, and histologically by typical features of solitary keratoacanthoma. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is not yet determined. Herein, we review the different aspects of this rare entity, including pathogenesis, clinical and histopathological features, differential diagnosis, course and prognosis. Different therapeutic approaches and their impact on the course and prognosis of the disease are also evaluated and presented. We propose two sets of diagnostic criteria to define the disease more precisely and to avoid overlapping and confusion with other types of multiple keratoacanthoma. The first set comprises constant clinical and histopathological features that almost always present in every case and the second set includes variable features that were reported in some patients, and to which any emerging finding could be added to avoid missing cases. Although different therapeutic options have been used, either as single agents or in combinations, there is no standard therapy for GEKA and the disease still represents a therapeutic challenge.

Generalized eruptive keratoacanthoma with vitiligo followed by the development of prurigo nodularis: A case report and published work review

“…No family history of multiple KA was found. All the features were consistent with the diagnostic criteria proposed by Nofal et al . and modified by Kwiek et al .…”

“…In the present case, oral acitretin was dramatically effective, while the mechanism was still unclear. Retinoic acid seems to inhibit keratinization, regulate terminal differentiation of epidermal cells and increase both IL‐2 production and mitogen‐induced lymphocyte proliferation …”

Intralesional therapy for the treatment of keratoacanthoma

“…Lastly, it should be emphasized that MTX provides minimal to almost no response when used for treatment of variants of multiple KA, such as GEKA (Nofal et al, 2014;Nofal, Assaf, Ghonemy, Nofal, & Yosef, 2015;Oakley & Ng, 2005). Therefore, in those cases, a more suitable therapeutic option should be considered.…”

Keratoacanthoma

“…These papules can appear anywhere on the body, even on the tongue, oral mucosa, or larynx. They are usually distributed most heavily in sun-exposed areas such as the face, where they can lead to ectropion (Nofal, Assaf, Nofal, & Alradi, 2014).…”