Anogenital Papular Acantholytic Dysteratosis

Presented by Makayla Powers, PA-C & David Elpern

HPI: An 18 year old woman was seen for lesions in her genital area. She noticed pruritus ani about 3 months earlier and was seen by a gynecologist who initially prescribed antibiotics, which patient states caused the lesions to “flatten,” but not completely resolve. A biopsy was performed that a pathologist signed out as molluscum.   Blood work for STIs was all normal. She was seen in the dermatology clinic for therapeutic suggestions. We requested a review of the biopsy by a dermatopathologist. 

On exam, multiple white papules in the perianal area and on the labia majorum.

Clinical Photos:  

                                                                                                                       

 

Pathology: 

This shows hyperkeratosis, parakeratosis and acantholytic dyskeratosis, characterized by suprabasilar clefting with acantholytic and dyskeratotic cells including corps ronds and grains within the epidermis.

 

Photomicrographs (courtesy of Dr. David Jones, Berkshire Medical Center)

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Diagnosis: Anogenital Papular Acantholytic Dyskeratosis

Discussion:
This is a rare entity that currently does not have many successful treatment options. On review of the literature, this condition may be seen with Hailey-Hailey disease and we wonder if it may be a localized variant of Hailey-Hailey. We reassured the young woman that she does not have an STI, however she is very self-conscious about the appearance of these lesions in her anogenital area. She is currently in a relationship with a partner who she is comfortable with, however she worries about what future partners may think of her condition.

Question: Do any of our readers have experience managing patients with this disorder?

 

References:

Bell HK, Farrar CW, Curley RK. Papular acantholytic dyskeratosis of the vulva. Clin Exp Dermatol. 2001 Jul;26(5):386-8. doi: 10.1046/j.1365-2230.2001.00840.x. PMID: 11488821.

Dittmer CJ, Hornemann A, Rose C, Diedrich K, Thill M. Successful laser therapy of a papular acantholytic dyskeratosis of the vulva: case report and review of literature. Arch GynecolObstet. 2010 Apr;281(4):723-5. doi: 10.1007/s00404-009-1313-8. Epub 2009 Dec 15.PMID: 20012979.

Hadjicharralambous E, Diamond S, Mehregan D. Papular acantholytic dyskeratosis of vulva in setting of Hailey-Hailey. Int J Dermatol. 2017 Jun;56(6):e126-e128. doi: 10.1111/ijd.12486. Epub 2017 Apr 12. PMID: 28401649.

 

Lee SH, Jang JG. Papular acantholytic dyskeratosis of the genitalia. J Dermatol. 1989 Aug;16(4):312-4. doi: 10.1111/j.1346-8138.1989.tb01270.x. PMID: 2689491.

 

Roh MR, Choi YJ, Lee KG. Papular acantholytic dyskeratosis of the vulva. J Dermatol. 2009 Jul;36(7):427-9. doi: 10.1111/j.1346-8138.2009.00660.x. PMID: 19583693.

 

Sáenz AM, Cirocco A, Avendaño M, González F, Sardi JR. Papular acantholytic dyskeratosis of the vulva. Pediatr Dermatol. 2005 May-Jun;22(3):237-9. doi: 10.1111/j.1525-1470.2005.22312.x. PMID: 15916573.