Hydroxychloroquine-induced Hyperpigmentation

 Presented by DJ Elpern

 

This 23-year-old woman presents for evaluation of patchy hyperpigmentation of her face, arms, and abdomen that has been progressing for the past two years.  She has had systemic lupus erythematosus with renal involvement for a decade.  Her current medications include prednisone 15 mg a day, mycophenolic acid, 2 tablets twice a day, hydroxychloroquine (HQ) 400 mg a day, torsemide, Zoloft, olanzapine, losartan, and recently voclosporin.  She has been on 400 mg of HQ since the early days of her Lupus diagnosis.

 

OE:  The examination shows large hyperpigmented patches on the face, ears, arms and in the abdominal striae.  There is no scarring.  She has Cushingoid facies.
 

 

IMPRESSION: Hydroxychloroquine-induced Hyperpigmentation

 

 

PLAN:  We will consult some colleagues with experience in this area and see the patient back in a few weeks.  Recent serologies may be helpful. 

 

Discussion:  HQ hyperpigmentation is well-reported; but most of the articles are small case reports.  It seems to be related to duration of treatment.  This young woman has been on 400 mg of HQ for at least eight years.  Other than stopping HQ, it’s unclear what may help.  The drug may have been of value for her renal disease; but the downside – the hyperpigmentation – is a significant problem for this young person.  So, too is the Cushingoid effects of her prednisone.  A review of the literature was not helpful for therapeutic guidelines regarding the hyperpigmentation other than discontinuing the HQ.

 

 

References:

1. Daniel Kwak, Pearl E Grimes. A case of hyperpigmentation induced by hydroxychloroquine and quinacrine in a patient with systemic lupus erythematosus and review of the literature. Int J Womens Dermatol. 2020 Jun 30;6(4):268-271. Free PMC

 

2. Moez Jallouli. Hydroxychloroquine-induced pigmentation in patients with systemic lupus erythematosus: a case-control study. JAMA Dermatol. 2013 Aug;149(8):935-40.

 

3. Michela Gasparotto et. al. Lupus nephritis: clinical presentations and outcomes in the 21st century. Rheumatology (Oxford). 2020 Dec 5;59(Suppl5):v39-v51.  Free PMC.

 

4. Sendhil Kumaran Muthu. Low-dose oral isotretinoin therapy in lichen planus pigmentosus: an open-label non-randomized prospective pilot study. Int J Dermatol. 2016 Sep;55(9):1048-54. PMID 27062273

 

 

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.