Two Case Reports of Columnar Dyskeratosis, an Unusual Keratinisation Disorder

Abstract

Keratinisation disorders with distinctive histopathological patterns are few in number. We describe two men with unusual dermatosis, characterised by a distinctive pattern of focal dyskeratosis. Both men suffered from generalised dermatosis formed by verrucous red-brown plaques. Repeated skin biopsies showed the same histopathological pattern with foci of vertically oriented dyskeratotic cells. The dyskeratotic cells on the level of the stratum spinosum and granulosum were positive for AE1/AE3, CK-HW, CK-LS and CK116 immunostaining. PCR for HPV was negative. The similar clinical appearance of skin lesions in both patients together with their identical histopathological pictures seems to represent a unique clinicopathological condition that we believe is best described by the term ‘columnar dyskeratosis’.

Human polyomavirus 6 and 7 are associated with pruritic and dyskeratotic dermatoses

“…We identify HPyV6 and 7 infection in three additional patients with pruritic dermatoses and provide evidence for the involvement of these viruses in the pathogenesis of the eruptions 12, 13 . Our work expands the spectrum of skin diseases associated with HPyV6 and 7 and yields novel insights into the biology of these ubiquitous skin polyomaviruses.…”

“…Importantly, all cases were first identified based on biopsies that showed a characteristic histologic pattern—scattered dyskeratotic cells throughout the epidermis along with irregular columns of parakeratosis in the stratum corneum (Fig 1B). This pattern has been described in the literature as “columnar dyskeratosis,” “tiered parakeratosis with dyskeratosis,” or “peacock plumage” 9, 12, 13 . FFPE lesional skin biopsy samples from these patients were screened for known human polyomaviruses by endpoint PCR.…”

“…The clinical characteristics of the patients, which have been previously reported, are summarized in Table I 12, 13 . All three patients presented with generalized, scaly, hyperpigmented papules coalescing into plaques, which had all been present for at least 12 months.…”

Florid cutaneous papillomatosis

“…2). Similarly, there is no dyskeratosis, spike-like orthoparakeratosis or other findings suggestive of a variety of other hyperkeratotic entities [27]. Verrucous mucocutaneous nodulation of the lips and buccal mucosa may represent incipient FCP in a patient with malignant acanthosis nigricans.…”

The Biology and Clinical Features of Cutaneous Polyomaviruses

“…Immunosuppressive medicines in these organ transplant recipients included prednisone, azathioprine, sirolimus, tacrolimus, everolimus, and mycophenolate (Canavan et al, 2017;Ho et al, 2015;Smith et al, 2018). Histopathology reveals epidermal papillomatosis, dyskeratotic keratinocytes in the epidermis, and irregular columns of parakeratosis, the latter referred to as "peacock plumage," "columnar dyskeratosis," or "tiered parakeratosis with dyskeratosis" (Figure 1d) (Canavan et al, 2018;Champagne et al, 2015;Ho et al, 2015;Nguyen et al, 2017;Pock and Stork, 2010). In the upper dermis, there may be sparse, perivascular, lymphocytic infiltrates (Ho et al, 2015;Pock and Stork, 2010).…”