|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 1 | Page : 19-20
Giant verrucous porokeratosis with psoriasiform alteration: A rare entity with atypical clinico-histopathologic features
Anup Kumar Tiwary1, Dharmendra Kumar Mishra2
1 Department of Dermatology, Venereology and Leprosy, Government Medical College, Haldwani, Uttarakhand, India
2 Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India
|Date of Web Publication||6-Jun-2017|
Anup Kumar Tiwary
Department of Dermatology, Venereology and Leprosy, Government Medical College, Haldwani, Uttarakhand
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Tiwary AK, Mishra DK. Giant verrucous porokeratosis with psoriasiform alteration: A rare entity with atypical clinico-histopathologic features. Indian J Dermatopathol Diagn Dermatol 2017;4:19-20
|How to cite this URL:|
Tiwary AK, Mishra DK. Giant verrucous porokeratosis with psoriasiform alteration: A rare entity with atypical clinico-histopathologic features. Indian J Dermatopathol Diagn Dermatol [serial online] 2017 [cited 2020 Oct 29];4:19-20. Available from: https://www.ijdpdd.com/text.asp?2017/4/1/19/207564
Porokeratosis is an inherited, clonal disorder of epidermal keratinization, classically characterized by the development of single or multiple annular lesions with raised, sharply marginated, keratotic ridge, and central atrophy. This clinical presentation may vary with different types of porokeratosis but histopathologically unified by the consistent finding of “cornoid lamella.” Here, we document an atypical and rare case of “giant verrucous porokeratosis” which showed psoriasiform histopathological changes at the center of the lesion.
A 25-year-old male, with no premorbid illness, presented with multiple verrucous plaques over the right side of the back, left lateral aspect of chest and abdomen, nape of the neck, right arm, and abdomen for the past 8 years. The lesions were mildly pruritic and scaly. Cutaneous examination revealed a total of five lesions characterized by well-defined, whitish, scaly, verrucous plaque without any apparently raised margin. Two of such lesions were giant in size involving the whole right half of the back and left lateral aspect of trunk and one was over the nape of neck [Figure 1] and [Figure 2]. No other sites were affected including scalp, mucosa, and nails. There was no family history of psoriasis or porokeratosis, and the patient had not received any prior allopathic treatment for this condition. Routine laboratory parameters were within normal limits.
|Figure 1: Giant scaly verrucous plaque over (a) the right half of the back and (b) left lateral aspect of chest and abdomen extending to the right scapular area|
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For histopathological evaluation, two samples of punch biopsy were taken, one from the center of plaque and other from the edge. The sample taken from the edge incidentally demonstrated cornoid lamella as vertical column of poorly stained parakeratotic cells with pyknotic nuclei, running through the normally stained surrounding cells along with absence of granular layer in its floor and hypergranulosis of wall. Dyskeratotic keratinocytes in the spinous layer were also present along with perivascular patchy lichenoid lymphocytic infiltrates and focal basal vacuolar changes. All these findings were in favor of porokeratosis [Figure 3]a and [Figure 3]b. Other sample taken from the center of the lesion revealed characteristic psoriasiform changes such as orthohyperkeratosis, foci of parakeratosis, attenuated granular layer, and moderate papillomatosis. In dermis, dilated tortuous capillaries at the tip of clubbed dermal papilla and moderately dense superficial perivascular lymphocytic infiltrate were seen supporting the psoriasiform changes [Figure 3]c and [Figure 3]d.
|Figure 3: (a and b) Margin of the lesion showing cornoid lamella (H and E, ×200). (c) Center of the lesion revealing psoriasiform changes: moderate papillomatosis, moderately dense superficial perivascular lymphocytic infiltrate (H and E, ×200). (d) Focal parakeratosis, attenuated granular layer with dilated tortuous capillaries at the tip of clubbed dermal papilla (H and E, ×400)|
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The presence of typical cornoid lamella at the margin of verrucous plaque and other histopathologic findings led us to diagnose it as “verrucous porokeratosis” with psoriasiform changes. The patient was kept on oral isotretinoin (30 mg daily) and topical salicylic acid, but he did not turn up further.
There are many well-recognized clinical variants of porokeratosis: porokeratosis of Mibelli, disseminated superficial porokeratosis, disseminated superficial actinic porokeratosis (DSAP), giant porokeratosis, palmaris et plantaris disseminata, linear porokeratosis, and punctate porokeratosis. An another variant was reported by Helfman and Poulos in 1985, but first described in detail by Lucker et al. in 1995 who termed it as “porokeratosis ptychotropica” (ptyche means fold) owing to its characteristic flexural involvement in genitogluteal region. Due to its verrucous morphology, it was also known as hyperkeratotic or “verrucous porokeratosis.” Since its first description, <40 cases have been reported worldwide to the best of our knowledge. Of note, we were unable to find a single case of “verrucous porokeratosis” which affected sites other than genitogluteal region, buttocks, and extremities (as in our case).
The association of psoriasis and porokeratosis is long known, but definitely rare and their pathogenetic evolution is still an enigma. Recently, in a study, a similar pattern of upregulation of certain proteins such as keratins (keratins 16, 6A, 6B, and 17), specific calcium-binding proteins (psoriasin 1, calgranulin A, calgranulin B, and calgizarin), and gap junction proteins (connexin 26 and 30) was noted in psoriasis and porokeratosis which favors a genetic relationship between these two dermatoses. In 2006, a classification was proposed to categorize this association into three groups. The first group includes cases of porokeratosis mimicking psoriasis, wherein verrucous porokeratotic plaques are usually localized to the natal cleft and buttocks known as “porokeratosis ptychotropica.”, The second group includes patients presenting with classical clinical lesions of porokeratosis along with psoriasiform changes at the center of the porokeratotic plaque. The third group consists of patients with psoriasis and DSAP induced by therapeutic phototherapy or photochemotherapy. This classification also has therapeutic and prognostic implications as treatment approach of these two conditions differs, and unfortunately on rare occasions, porokeratosis may turn into cutaneous malignancy. Our case revealed combined features of both first and second groups, hence could not be strictly categorized under any of the above three groups. Thus, this is a unique case of giant verrucous porokeratosis with atypical sites of involvement and peculiarly associated with psoriasiform alteration at the center of the lesion which can be treated with topical 5% imiquimod, Vitamin D analogs, oral retinoic acid derivatives, or can be surgically excised if feasible.,
The authors sincerely thank Dr. Uday Khopkar for his contribution by giving valuable suggestions for the histopathological diagnosis.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]