Porokeratosis ptychotropica: a rare and evolving variant of porokeratosis

Abstract

Porokeratosis ptychotropica represents a rare and under-recognized variant of porokeratosis. There are also alternative descriptions for this disorder in the literature. Since its original description in 1995, additional characteristic features have been showed in case reports published in the literature. These cumulative reports, although still limited in numbers, have helped to further shape and define this entity. A case report and review of published literature on this unusual entity are presented. The specific combination of clinical, morphological and histopathological characteristics that can facilitate recognition of the disorder is discussed. There has been a call for uniformity in terminology and a suggestion for alternative terminology has been made. However, we discuss why the earlier term, porokeratosis ptychotropica, is still preferred.

Keratotic papules and plaques in the genitogluteal region

“…Die Retinoide haben zu einer Linderung bei symptomatischen Läsionen geführt; auf die Läsion selbst zeigten sie jedoch einen geringen Effekt. Erfolgreicher waren chirurgische Interventionen, einschließlich der Dermabrasion und CO 2 -Laser-Ablation, mit Latenzzeiten von bis zu neun Jahren bis zum Wiederauftreten der Läsionen nach der Intervention [9]. Kürzlich wurde auch über eine Therapieoption mit Ingenolmebutat bei der Porokeratosis Mibelli berichtet [10].…”

Porokeratosis ptychotropica involving the glans penis: a unique case of this rare condition

“…Porokeratosis ptychotropica was first described by Lucker et al in 1995 [2]. The term "ptychotropica" comes from the Greek word ptyche (fold), and describes the flexural affinity of the disease, with a predilection for the anal cleft [3,4]. Clinically, PP presents with pruritic, symmetric, redbrown, verrucous plaques in the genitogluteal region and surrounding satellite lesions.…”

“…Histologically, it is characterized by multiple, often concentrically arranged cornoid lamellae [1,5,6]. Although many clinical variants of porokeratosis have been described, the occurrence of multiple cornoid lamellae appears to be typical of PP [1,4]. Some case reports have also described subepidermal amyloid deposits [1,7].…”

Porokeratosis ptychotropica responding to photodynamic therapy: An alternative treatment for a refractory disease

“…Porokeratoses are a group of hereditary cutaneous disorders of uncertain origin characterized by abnormalities in keratinization, resulting in the formation of keratotic papules and plaques with a well‐defined high edge and centrifugal growth, which histologically corresponds to a parakeratotic column named cornoid lamellae . Porokeratosis ptychotropica (PP) is a rare variant with a special predisposition to affect body folds, particularly the intergluteal cleft …”

“…Porokeratosis ptychotropica (PP) is a rare variant with a special predisposition to affect body folds, particularly the intergluteal cleft. 1…”