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  Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 6  |  Issue : 5  |  Page : 339-341  

Penoscrotal porokeratosis: A distinct entity


1 Department of Dermatology, PD Hinduja Hospital and Medical Research Centre, Mumbai, Maharashtra, India
2 Department of Dermatology, SPRINGS, Swar Vihar Griha Sankul, Chiplun, Maharashtra, India

Date of Web Publication4-Sep-2015

Correspondence Address:
Rajiv Joshi
14 Jay Mahal, A Road, Churchgate, Mumbai - 400 020, Maharashtra
India
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Source of Support: Nil, Conflict of Interest: None declared.


DOI: 10.4103/2229-5178.164487

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   Abstract 

A 26-year-old man presented with five months history of redness associated with itching and burning over the scrotum and shaft of the penis with a persistent rash on those sites. There had been no response to topical steroid and antifungal creams. Clinical examination revealed a large well-circumscribed erythematous plaque with a thready raised border with a tiny groove at its summit that involved almost two-thirds of the ventral part of the shaft of the penis. Ill-defined erythema with a granular surface was seen over the anterior scrotal skin. A 4 mm punch biopsy of the plaque on the penile shaft revealed multiple cornoid lamellae located adjacent to one another. The patient was treated with topical emollients. Follow up after four months revealed almost complete resolution of the plaque on the penile shaft. Penoscrotal porokeratosis appears to be a distinct entity in the family of porokeratotic diseases, described only in young males in their twenties with involvement of the penile shaft and anterior scrotum with severe burning and itching and histologically associated with multiple cornoid lamellae. It may represent an unusual epidermal porokeratotic reaction pattern and may be a self-resolving condition.

Keywords: Epidermal reaction pattern, genital porokeratosis, multiple cornoid lamellae, penoscrotal porokeratosis


How to cite this article:
Joshi R, Jadhav Y. Penoscrotal porokeratosis: A distinct entity. Indian Dermatol Online J 2015;6:339-41

How to cite this URL:
Joshi R, Jadhav Y. Penoscrotal porokeratosis: A distinct entity. Indian Dermatol Online J [serial online] 2015 [cited 2021 Dec 6];6:339-41. Available from: https://www.idoj.in/text.asp?2015/6/5/339/164487


   Introduction Top


Porokeratosis represents a group of disorders of keratinization, which clinically have varied presentations and may present with plaques with atrophic centers and a raised keratotic margin with a groove at the summit of the margin (Porokeratosis of Mibelli), or small keratotic lesions with a barely raised margin (disseminated actinic and nonactinic porokeratosis) or an eczematized and verrucous plaque restricted to the gluteal cleft (Porokeratosis ptychotropica).

The histologic hallmark of all clinical variants is the presence of cornoid lamellae, which are columns of parakeratosis that overlie an epidermal invagination with loss of the granular layer and dyskeratosis of upper spinous keratinocytes.

Porokeratosis restricted to the genitals is uncommon with few cases described in world literature and all of those were considered to be similar to porokeratosis of Mibelli. Recently, a series of cases of penoscrotal porokeratotic plaques has been described in young men in their twenties, who had identical clinical presentation of pruritic plaques on the penis and anterior scrotum with multiple cornoid lamellae on histopathology.[1]

We present an additional case and propose that penoscrotal porokeratosis is a distinct entity in the family of porokeratoses. It has so far been described only in young males in their third decade. The age restriction is very striking in this condition and is at variance with other forms of porokeratosis, which have been described at all ages. These patients present with pruritic burning erythematous plaques and ill-defined patches on the penile shaft and anterior scrotal wall that histologically show multiple cornoid lamellae. It appears to be distinct from porokeratosis ptychotropica, which occurs in the gluteal region but shares with it a common histopathological finding of multiple cornoid lamellae.


   Case Report Top


A 26-year-old man presented with five months history of persistent, itchy, and burning skin lesions restricted to the ventral part of the penile shaft and anterior scrotal skin. He had been treated with topical steroid and antifungal creams without any relief of symptoms or resolution of the skin lesions. There had been no episode of injury to that area either due to trauma or due to use of cleansing chemicals, and the person was in a sedentary job and had no exposure to extremes of temperature or humidity.

On examination, there was a sharply circumscribed erythematous atrophic plaque that involved almost two-thirds of the ventral surface of the penile shaft and had a very narrow raised keratotic margin with a tiny groove at the summit [Figure 1]. There was also an ill-defined area of erythema with a granular surface involving the anterior surface of the scrotum, which was more pronounced on the left side of the scrotum [Figure 2].
Figure 1: Erythematous plaque on ventral surface of penile shaft with a sharply defined thready keratotic margin

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Figure 2: Ill-defined erythema on scrotum with a granular surface

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A 4 mm punch biopsy of the plaque on the penis revealed multiple cornoid lamellae located adjacent to one another [Figure 3]. Individual cornoid lamellae showed a column of parakeratosis arising from an epidermal invagination with loss of granular layer at its base and few dyskeratotic upper spinous keratinocytes [Figure 4]. The patient was prescribed oral isotretinoin but did not take it and instead applied only white soft paraffin with gradual diminution of symptoms. Follow up after four months revealed that his pruritus had subsided and the plaque on the penile shaft had almost cleared [Figure 5].
Figure 3: Multiple contiguous cornoid lamellae in a mildly hyperplastic epidermis. Hematoxylin and eosin, ×100

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Figure 4: High power details of a cornoid lamella with column of parakeratosis with dyskeratotic cells at its base and absence of the granular layer. Hematoxylin and eosin, ×400

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Figure 5: Follow up at 4 months with clearing of the penile plaque

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   Discussion Top


Penoscrotal porokeratosis appears to be a distinct entity in the family of porokeratoses. It involves only the penis and scrotum and has been described only in young men in their third decade who present with pruritic and burning patches and plaques on the penile shaft and the scrotum. The patches on the penis are usually well-circumscribed plaques with a thin raised margin with a groove similar to porokeratosis of Mibelli but on the scrotum the eruption is more diffuse and has a pebbly or granular appearance. Histologically, multiple cornoid lamellae are seen arising from the epidermis as well as from acrosyringia and follicular infundibula.[1] These multiple cornoid lamellae are seen in biopsies both from the penis and from the scrotum and may be responsible for the granular or pebbly surface seen clinically.

Other types of porokeratosis restricted to the genital region are uncommon and few cases have been described in the literature ([Table 1]). A series of 10 patients was described by Chen et al.[2] and a recent report[3] from China studied 55 cases of porokeratosis of which 11 cases (one female) had lesions on the genitals. All reported cases of genital porokeratosis have been clinically and histologically similar to classical porokeratosis of Mibelli.
Table 1: Comparison of different types of porokeratosis occurring on the genitogluteal region

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Porokeratosis ptychotropica first described in 1995[4] is another uncommon type of porokeratosis that is restricted to the gluteal fold and presents with pruritic verrucous plaques that clinically are often misdiagnosed as psoriasis, lichen simplex chronicus, dermatophytic infection, or condylomata acuminata. Histologically, unlike classic porokeratosis of Mibelli this variant has been associated with the presence of multiple cornoid lamellae, which is similar to penoscrotal porokeratosis.

Wanat et al.[5] reported a case of a 28-year-old African American man who presented with pruritic erythematous plaques on the scrotum that showed multiple cornoid lamellae and considered it to be a variant of porokeratosis ptychotropica occurring on the scrotum. This case is very similar to our case and to the series of cases of penoscrotal porokeratosis described earlier[1] and should be considered as penoscrotal porokeratosis and not a variant of porokeratosis ptychotropica.

The age restriction of this condition is striking and while the pathogenesis is not known it may be a porokeratotic epidermal reaction pattern to friction in a hot and humid climate. The natural course of this condition is not known as all previous patients were lost to follow up after few months, however, considering that all patients were distressed by the itching and burning the lack of follow up after few months suggests that it may undergo a natural remission. Our patient had an almost complete remission of the skin lesions at 4 months follow up with only topical application of white soft paraffin and it suggests that this condition is an epidermal porokeratotic reaction pattern with a natural remission, which however may last several months.

In sum, we describe a case of penoscrotal porokeratosis and suggest that this is a distinct entity within the family of porokeratotic conditions and suggest the following criteria for its diagnosis:

  • Young men in their third decade.
  • Plaques and diffuse granular patches with severe pruritus and burning sensation restricted to the penile shaft and scrotal skin.
  • Presence of multiple contiguous cornoid lamellae some of which may arise from follicular and eccrine structures.
  • An initial poor response to treatment and a possible delayed natural remission of the condition.


More cases need to be studied to assess treatment modalities and the natural course of this condition.

 
   References Top

1.
Joshi R, Mehta SG. Pruritic porokeratotic peno-scrotal plaques: Porokeratosis or porokeratotic epidermal reaction pattern? A report of 10 cases. Indian J Dermatol Venereol Leprol 2014;80:24-8.  Back to cited text no. 1
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2.
Chen TJ, Chou YC, Chen CH, Kuo TT, Hong HS. Genital porokeratosis: A series of 10 patients and review of literature. Br J Dermatol 2006;155:325-9.  Back to cited text no. 2
    
3.
Gu CY, Zhang CF, Chen LJ, Xiang LH, Zheng ZZ. Clinical analysis and etiology of porokeratosis. Exp Ther Med 2014;8:737-41.  Back to cited text no. 3
    
4.
Lucker GP, Happle R, Steijlen PM. An unusual case of porokeratosis involving the natal cleft: Porokeratosis ptychotropica? Br J Dermatol 1995;132:150-1.  Back to cited text no. 4
[PUBMED]    
5.
Wanat KA, Gormley RN, Bennet DD, Kovarik CL. Genitogluteal porokeratosis involving the scrotum: An unusual presentation of an uncommon disease. J Cutan Pathol 2012;39:72-4.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1]


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