Indian Journal of Dermatology
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Year : 2018  |  Volume : 63  |  Issue : 6  |  Page : 527-529
Genitocrural plane-topped papules in a young female


1 Department of Dermatology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Pathology, Dr. SN Medical College, Jodhpur, Rajasthan, India
3 Department of Pathology, AIIMS, Jodhpur, Rajasthan, India

Date of Web Publication2-Nov-2018

Correspondence Address:
Dr. Saurabh Singh
Department of Dermatology, All India Institute of Medical Sciences, Jodhpur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_198_18

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How to cite this article:
Bains A, Singh S, Dubey A, Rao M. Genitocrural plane-topped papules in a young female. Indian J Dermatol 2018;63:527-9

How to cite this URL:
Bains A, Singh S, Dubey A, Rao M. Genitocrural plane-topped papules in a young female. Indian J Dermatol [serial online] 2018 [cited 2018 Dec 11];63:527-9. Available from: http://www.e-ijd.org/text.asp?2018/63/6/527/244814




A 27-year-old female presented with complaints of gradually progressive asymptomatic brownish-to-gray colored raised lesions over the genital region and thighs for the last 2 years. Her sexual and family histories were noncontributory. On mucocutaneous examination, there were multiple grouped brownish-to-gray-colored soft papules over the labia majora, inguinal folds, and medial aspect of both thighs [Figure 1]. Few lesions were also present in both inframammary regions [Figure 2]. There were no lesions elsewhere on the body. Biopsy from lesions on the medial aspect of the thigh revealed hyperkeratosis, focal parakeratosis, and papillomatosis along with suprabasal cleft filled with acantholytic cells. Cells with rounded nuclei with perinuclear halo and pale cytoplasm were also seen in the granular layer. Biopsy from the inframammary region showed, in addition, upward proliferation of papillae lined by basal cells (villi) and communication of the clefts with the stratum corneum [Figure 3]a and [Figure 3]b.
Figure 1: Multiple grouped brownish-to-gray colored papules over the labia majora, inguinal folds, and medial aspect of both thighs

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Figure 2: Few brownish papules over the left inframammary region

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Figure 3: (a) Photomicrograph showing an intraepidermal (suprabasal) cleft with upward proliferation of papillae lined by basal cells (villi), filled with acantholytic cells (H and E, ×100). (b) Photomicrograph showing an intraepidermal (suprabasal) cleft filled with acantholytic cells, which are rounded and have deeply eosinophilic cytoplasm, which is relatively paler in perinuclear location and denser or condensed in the periphery (black arrow) (H and E, ×400)

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   Question Top


What is your diagnosis?


   Answer Top


Papular acantholytic dermatosis.


   Discussion Top


Papular acantholytic dermatosis (PAD) is a rare type of focal acantholytic dyskeratosis clinically characterized by the presence of skin-colored to grayish-white papules localized to anogenital and genitocrural areas.[1] In a review of cases by Al-Muriesh et al., the mean age at the time of presentation was 38.8 years with a male-to-female ratio of 0.8:1.[2] The common types of lesion were papules (55.6% of patients) followed by papuloplaque lesions (25.9% of patients) with a history of pruritis in 59.3% of patients.[2] In females, labia majora is the most common site of involvement.[3] In our patient in addition to the genitocrural area, lesions were also present in the submammary area. Similar site of involvement was reported by Van Joost et al.[4] in a 35-year-old female. Moisture and occlusion had been suggested as contributing factors based on the location of lesions.[1],[2] PAD is clinically confused with genital warts, molluscum contagiosum, eczema, lichen planus, and candidiasis.[2] Histopathologically, it needs to be differentiated from other types of focal acantholytic dyskeratoses, i.e., Hailey–Hailey disease, Darier disease, Grover's disease, warty dyskeratoma, and acantholytic acanthoma.[4] Both Hailey–Hailey disease and Darier disease are autosomal dominant conditions with a positive family history. In Hailey–Hailey disease, there are moist erosive plaques with linear fissures in intertriginous areas, while Darier disease is characterized by the presence of warty papules in seborrheic distribution along with characteristic nail changes [Table 1].[1],[2],[3],[4],[5],[6] These were excluded in our patient because of late age of onset, absence of family history, and presence of grayish-brown papules in genitocrural location. Grover's disease usually presents with transient lesions over the trunk and proximal extremities and is not localized to the genital area. Warty dyskeratoma presents with solitary papule with a keratotic plug.
Table 1: Differential diagnosis of papular acantholytic dermatosis

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The histopathology of PAD shows hyperkeratosis, papillomatosis, dyskeratoses, and acantholysis along with variable presence of corps ronds and grains.[2] There have been reports of PAD patients showing ATP2A2 and ATP2C1 mutations which are otherwise typically seen in cases of Darier disease and Hailey–Hailey disease, respectively. The above finding in addition to histopathological overlap suggests that PAD may be a mild variant or mosaic form of these diseases.[3],[5]

Treatment is usually difficult and disappointing. Topical steroids and topical calcineurin inhibitors provide symptomatic relief in pruritus with no change in lesions. Topical retinoids, oral retinoids, destructive therapies such as electrocautery, cryotherapy, and CO2 laser are other treatment options which provide a temporary reduction in lesions.[2]

To conclude, proper diagnoses and awareness among the clinicians and pathologists alike about this rare entity are important for pertinent counseling of an anxious patient.

Learning points

  • PAD is characterized by the presence of skin colored to grayish-white papules localized to the anogenital and genitocrural areas
  • It may mimic venereal diseases such as genital warts and molluscum contagiosum, so its correct identification is important to allay patient anxiety
  • The histopathology of PAD shows hyperkeratosis, papillomatosis, dyskeratoses, and acantholysis along with variable presence of corps ronds and grains
  • Some cases have shown mutation similar to that of Hailey–Hailey disease and Darier disease. Furthermore, its histopathology overlaps with these diseases; therefore, it may be considered as a mild variant or mosaic form of these diseases.


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Verma SB. Papular acantholytic dyskeratosis localized to the perineal and perianal area in a young male. Indian J Dermatol 2013;58:393-5.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Al-Muriesh M, Abdul-Fattah B, Wang X, Zhao M, Chen S, Huang C, et al. Papular acantholytic dyskeratosis of the anogenital and genitocrural area: Case series and review of the literature. J Cutan Pathol 2016;43:749-58.  Back to cited text no. 2
    
3.
Xiao X, Chen L, Wang B, Li C. A novel missense mutation of the ATP2C1 gene in a Chinese patient with papular acantholytic dermatosis of the anogenital area. Indian J Dermatol Venereol Leprol 2016;82:429-31.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Van Joost T, Vuzevski VD, Tank B, Menke HE. Benign persistent papular acantholytic and dyskeratotic eruption: A case report and review of the literature. Br J Dermatol 1991;124:92-5.  Back to cited text no. 4
    
5.
Knopp EA, Saraceni C, Moss J, McNiff JM, Choate KA. Somatic ATP2A2 mutation in a case of papular acantholytic dyskeratosis: Mosaic Darier disease. J Cutan Pathol 2015;42:853-7.  Back to cited text no. 5
    
6.
Zamiri M, Munro CS. Inherited acantholytic disorders. In: Griffiths CE, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's Textbook of Dermatology. United Kingdom: Wiley-Blackwell; 2016. p. 66.1-66.13.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
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