Wednesday, December 14, 2022

Keratosis Pilaris Rubra

Presented by Makayla Powers PA-C
Pontoosac Shores, Massachusetts

HPI: Patient is a 15-year-old boy who presents, with his mother, for a facial eruption. His mother states this rash on his face has been present for about three years. His pediatrician prescribed a topical tretinoin, however, his mother has not used this for him yet as she is worried about it causing more skin irritation. Patient has asthma and currently uses a Flo-vent inhaler daily and albuterol inhaler as needed.

Physical exam: On exam, patient has erythematous patches with follicular papules on the bilateral cheeks, extending to the neck. He also has these erythematous follicular papules on the bilateral upper arms.



Diagnosis: Keratosis Pilaris Rubra


Discussion: We believe the patient has keratosis pilaris rubra. From the research we have been doing, topical therapies do not seem to not work well and laser therapy may be the most effective. We are asking if any of our readers has any experience treating this entity and what treatments they have found to be most successful? Thank you in advance for your time!



References:

1. Marqueling AL, et. al. Keratosis pilaris rubra: a common but underrecognized condition. Arch Dermatol. 2006 Dec;142(12):1611-6. doi: 10.1001/archderm.142.12.1611. PMID: 17178988.


2. Schoch JJ, et. al. Successful Treatment of Keratosis Pilaris Rubra with Pulsed Dye Laser. Pediatr Dermatol. 2016 Jul;33(4):443-6. doi: 10.1111/pde.12892. Epub 2016 Jun 10. PMID: 27282957.

 

3. Fekete GL, et. al., Keratosis pilaris atrophicans faciei: An observational, descriptive, retrospective clinical study. Exp Ther Med. 2021 Nov;22(5):1331. Free PMC article. [Dr. Warren Heymann brought this to our attention]

See: Comment # 1 from Omid Zargari



Saturday, December 03, 2022

Caruncular Tumor in a Child

 

The patient is an eight year-old girl with a  pigmented caruncular lesion of the left eye.  The child is of mixed ethnicity having light hair and green eyes.

 

The lesion is a circumscribed dark brown to black papule with slightly irregular borders.  No worrisome dermatoscopic features.


 

Diagnosis:  Melanocytic caruncular lesion in a child.

 

Plan:  She has been referred to a pediatric oncologic-ophthalmologist for evaluation.

 

Comment:  Based on a literature review it seems like melanoma is exceedingly rare in such a young child.  Nonetheless, it is important for this girl to be followed by a clinician with a special interest in childhood tumors.

 

Follow-up:  The patient saw a pediatric oncological-ophthalmologist at Boston Children's Hospital.  He felt her lesion is a benign caruncular nevus and will follow her at regular intervals.

 

References:

1. Carol L Shields, et. al. Clinical Features Differentiating Benign From Malignant Conjunctival Tumors in Children. JAMA Ophthalmol. 2017 Mar 1;135(3):215-224. Abstract.

2. Puneet Jain, et;; al. Conjunctival melanoma treatment outcomes in 288 patients: a multicentre international data-sharing study. Br J Ophthalmol. 2021 Oct; 105(10): 1358–1364. [There were no pediatric patients this study of almost 300 patients.]

3. Camille Yvon, et. al.  Caruncular lesions: A 16-year single centre review in the United Kingdom. Eur J Ophthalmol. 2022 Nov 10;11206721221137938.
Results: A total of 31 lesions from 31 patients were analysed. 18 of 31 patients were men (58%), and the age ranged from 12 to 91 years. 13 different histopathological types of lesions were identified in our case series, including 9 melanocytic nevi (29%), 7 benign squamous papillomas (23%), 5 skin adnexal lesions (16%), 3 chronic inflammation (10%), 3 epithelial cysts (10%), 1 basal cell carcinoma (3%), 2 malignant melanomas (6%) and l lymphoproliferative disorder (3%). Pre-operative suspected diagnoses were often vague but correct in 12 of 18 cases (67%), where data was available.
Conclusion:
The uncommon nature and variety of caruncular lesions make the diagnostic process difficult. Our case series is the first reported in the UK, showing a slightly higher proportion of malignant melanomas, in keeping with the population demographics. Excisional biopsies would, therefore, be prudent in the majority of cases to rule out any possible malignancy.

4. (Dr. Patrick Kenny (Victoria, BC, Canada) suggested Spitz/Reed nevus.  This reference may be helpful) Ana F Pedrosa, et. al.   Spitz/Reed nevi: a review of clinical-dermatoscopic and histological correlation. Dermatol Pract Concept. 2016 Apr 30;6(2):37-41. Full Text PubMed Central

 5. J Levy et. al. Lesions of the caruncle: a description of 42 cases and a review of the literature. Eye volume 23, 1004–1018 (2009) Full Text.  Helpful review. This study presents the clinical and histological data of 42 consecutive caruncular lesions processed at our laboratory and reviews previously reported cases of caruncular lesions.



 

 



 

 

 

Thursday, April 28, 2022

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

 

 

Friday, April 22, 2022

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)
40x

100x

400x

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.

 

enz 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.


Tuesday, March 15, 2022

Acral Lentiginous Melanoma During the COVID Pandemic

presented by Dr. Henry Foong, 
Ipoh, Malaysia

During the pandemic, clinical services in general hospitals were disrupted and patients tend to delay treatment due to lockdown and restriction in movement.  Here is one such example where the tumor had progressed to an advanced stage before the patient sought treatment from healthcare service.

This is the case of a 65-year-old housewife from Sungai Siput who presented with a 2-year history of an asymptomatic  pigmented growth on the left foot. It started as a pigmented macule which gradually increased in size.  She tried topical creams and traditional topical ointments without any improvement. She denied any excessive sun exposure. There was no family history of skin cancer.  Her medical history included hypertension, diabetes mellitus and hypercholesterolemia. She is not on any immunosuppressive drugs. She is married and has 4 children.

Examination showed a darkly pigmented nodular growth 4 x 5 cm on the interdigital webspace of the 2nd/3rd left toe. The lesion was well demarcated, and hard in consistency. The regional nodes were not enlarged.  There were no surrounding satellite lesions.

Clinically this patient has an acral lentiginous melanoma


A skin biopsy was performed. 

MICROSCOPIC APPEARANCE
Sections of the ulcerated epidermis show irregular nevus nests at the epidermis as well as the dermis. The irregular nevus cells are distributed along the basal layer of the epidermis and Pagetoid spread of these atypical nevus cells are noted. The dermis shows irregular distribution with lack of maturation. The nevus cells exhibit nuclear enlargement, contour angulation, nuclear hyperchromatic and prominent nucleoli. Moderate lymphocytic upper dermal infiltrate is present. The mitosis figure is 3 per mm squared. Maximum Tumor Thickness Breslow: 4.0 mm

INTERPRETATION
Skin punch biopsy Left foot: Malignant melanoma.
Maximum Tumor Thickness Breslow thickness Specify: 4.0 mm.
Ulcerated

No immunohistochemisty was done.




 




 

 

Follow-up: She is doing well at the moment.  She was seen at the Department of Orthopedic Surgery, Ipoh General Hospital and had surgical ray amputation of the toes. She is due for follow-up by the chemotherapy team from Kuala Lumpur Hospital, a Malaysian government-owned public hospital later this month.

Discussion:

While acral lentiginous melanoma is rare in the Western population, it is among the commonest type of malignant melanoma in the South East Asian countries (29-46% of melanomas in Asians).  Accurate pathological staging of the melanoma is useful to guide the treatment and management of the tumor.  

Questions:
Is sentinel node biopsy necessary in this patient?  

In Malaysia, it is commonly managed by orthopaedic surgeons together with the oncology team, most of the time partial amputation of the fore foot with the preservation of the ankle is done.
What approaches would a dermatological surgeon have?

References:

1.  J Pailoor et al Malays J Pathol 2012 Dec;34(2):97-101 Cutaneous malignant melanoma: clinical and histopathological review of cases in a Malaysian tertiary referral centre

2. A I Zainal et al. Med J Malay 2012:67;1:60-65 Acral melanoma of the extremities: A study of 33 cases in Sarawakian patients

3. F Durbec , L Martin, C Derancourt, F Grange. Melanoma of the hand and foot: epidemiological, prognostic and genetic features. A systematic review. Br J Dermatol. 2012 Apr;166(4):727-39.
Abstract

Background: While early recognition and prognosis of melanoma as a whole have improved, particular forms of rarer, under-recognized or more severe tumours require increasing attention. Among them, melanomas located on the hand and/or foot (hand and foot melanoma, HFM) have been the subject of few and heterogeneous studies, with variable and sometimes confusing results, and have not been targeted to date by comprehensive literature reviews.

Objectives: To perform a formal, systematic review of the literature, focusing on epidemiology, risk factors, prognosis and genetic characteristics of HFM. Methods All data sources were identified through searches on Medline, Scopus and Cochrane databases. Articles were selected and evaluated according to predefined quality criteria

Results: Among 1185 articles screened for relevance, 37 met the inclusion criteria. Data analysis brought to light important particularities of HFMs: they are rare in all ethnic groups of developed countries, but have been insufficiently studied in the developing world. About half are of the specific acral lentiginous melanoma (ALM) subtype. Previous trauma and naevi on the soles/toes were identified as two main risk factors in case-control studies. Genetic or environmental factors other than sun exposure are likely to play a role but require further investigation. Compared with melanoma at other sites, their prognosis is poor mainly as a consequence of later diagnosis, but possibly also because of an intrinsic negative effect of the HFM/ALM subtype. Standard prognostic factors of melanoma have been insufficiently validated to date in HFM. Finally, their molecular genetic particularities could lead to specific targeted therapies in the near future.

Conclusions: Overall, HFM could represent a particular subgroup of rare, potentially severe melanomas, requiring specific management from their prevention up to their treatment.

Saturday, March 12, 2022

Melanoma Neglecta

Presented by Jorge Delgado, M.D.
Brownsville, Texas

 

The patient is a single 65 yo man who lives alone.  He does not have a physician and has received no medical care in years.  His sister, who lives outside of the country, came to visit and noted that his shirts were stained by a malodorous exudate of blood and pus. Questioning, led to the discovery of a tumor on his back that apparently had been growing for months to years.

 

Usually fastidious about his personal hygiene, she also observed that he’d become forgetful, unsteady on his feet and had fallen recently.

 

The examination showed a 5 cm tumor on his back, portions of which had ulcerated.  No pigment was discernable.


 

 

She took him to a dermatology clinic where a biopsy was done.  It was reported as melanoma, greater than 4 mm thick with many mitoses.  We don’t have the path report.

 

This represents an example of Melanoma Neglecta.  People who lack basic medical care, who are isolated or who live in undeserved areas are recognized to be at risk for tumors that are detected at a late stage.

 

References:

1. Ana-Maria Forsea. Melanoma Epidemiology and Early Detection in Europe: Diversity and Disparities. Dermatol Pract Concept. 2020 Jun 29;10(3):e2020033.  PMC

 

2. Sauaia A , Dellavalle RP. Health care inequities: an introduction for dermatology providers. Dermatol Clin. 2009 Apr;27(2):103-7, PMC

 

3. Rachidi S, Deng Z, Sullivan DY , Lipson EJ. Shorter survival and later stage at diagnosis among unmarried patients with cutaneous melanoma: A US national and tertiary care center study. J Am Acad Dermatol. 2020 Oct;83(4):1012-1020.
Conclusions:
Unmarried patients, especially men and those younger than 68 years, are diagnosed at more advanced stages, even in readily visible sites such as the face. They also experience worse survival independent of stage.


Wednesday, March 02, 2022

Pumped and Tanned

Pumped and Tan, Melanotan Man

Presented by Dr. M. Chester Morris

Cosmetic and Aesthetic Dermatology Centre

Nanaimo, British Columbia, Canada

 

A 42 yo Man presented with a history of new naevi on torso a few months

 

He was in good general health.  His only medication was oral minoxidil, 5 mg a day, for male pattern alopecia.

 

O/E:  About a dozen naevi, 5 – 7 mm in diameter,

were scattered on his torso..  They were clinically benign appearance, but as it is unusual for a 42 year-old individual to have eruptive naevi. The most atypical one was biopsied.

 Pathology shows a junctional and compound nevus without atypia.

The patient is well-known to our clinic.

He has a history of

  • Ongoing mild alopecia areata of the bearded area and receives ILK every few months. 
  • Mild male-pattern alopecia.  He’s had 3 hair transplants, 2 locally and one at a specialty clinic in Southern California where they do PRP
  • Expensive cosmetic dental capping

 

He spends lots of time in the gym (uses supplements)

 

Further history reveals that he bought Melanotan. a synthetic analogue of the peptide hormone α-melanocyte-stimulating hormone (α-MSH) that stimulates melanogenesis and is purported to increases sexual arousal among other things, at his gym and has been injecting himself with it for a few months.  When I asked to see the product, he said he couldn’t find it.

 

Diagnosis: This 42 yo man developed Eruptive Melanoytic Naevi after the use of Melanotan obtained from a contact at his gym.  When I expressed interest in the drug; he disappeared. I have known him for a few years and he appears to have body dysmorphic syndrome (BDD) and that may have induced him to purchase Melanotan.  His form of BDD has been called “Muscle Dysmorphia” or The Adonis Complex.

 

 

Teaching points:

1. Use of Melanotan may be more widespread than we realize.

2. Patients such as this may have BDD, specifically the Adonis Syndrome

3.  There is an online literature on Melanotan and although it is risky, some patients nonetheless seek it out.

 

The medical literature contains references to various possible side-effects of Melanotan. These include: darkening of previous naevi, eruptive melanocytic naevi, isolated cases reports of melanoma and melanoma in situ, renal infarcts, and priapism.

 

Reference:

1. Ewa A Burian  1 , Gregor B E Jemec  Eruptive Melanocytic Nevi: A Review. Am J Clin Dermatol. 2019 Oct;20(5):669-682.

Abstract: Eruptive melanocytic nevi (EMN) is a phenomenon characterized by the sudden onset of nevi. Our objective was to compile all published reports of EMN to identify possible precipitating factors and to evaluate the clinical appearance and course. We conducted a systematic bibliographic search and selected 93 articles, representing 179 patients with EMN. The suspected causes were skin and other diseases (50%); immunosuppressive agents, chemotherapy or melanotan (41%); and miscellaneous, including idiopathic (9%). The clinical manifestations could largely be divided into two categories: EMN associated with skin diseases were frequently few in number (fewer than ten nevi), large, and localized to the site of previous skin disease, whereas those due to other causes presented most often with multiple small widespread nevi. In general, EMN seem to persist unchanged after their appearance, but development over several years or fading has also been reported. Overall, 16% of the cases had at least one histologically confirmed dysplastic nevus. Five cases of associated melanoma were reported. We conclude that the clinical appearance of EMN may differ according to the suggested triggering factor. Based on the clinical distinction, we propose a new subclassification of EMN: (1) widespread eruptive nevi (WEN), with numerous small nevi, triggered by, for example, drugs and internal diseases, and (2) Köbner-like eruptive nevi, often with big and few nevi, associated with skin diseases and most often localized at the site of previous skin disease/trauma. The nature of the data precluded assessment of risk of malignant transformation.

 

2. Melanotan https://en.wikipedia.org/wiki/Melanotan_IIWikipedia

 

3. Harrison G Pope Jr, Katharine A Phillips & Roberto Olivardia, The Adonis Complex: The Secret Crisis of Male Body Obsession (New York: Free Press, 2000)

 

4. Muscle Dymorphea: https://en.wikipedia.org/wiki/Muscle_dysmorphia

 

5. Louis Habbema  et. al.  Risks of unregulated use of alpha-melanocyte-stimulating hormone analogues: a review. Int J Dermatol. 2017 Oct;56(10):975-980.

Abstract

Recently, the unregulated use of untested synthetic alpha-melanocyte-stimulating hormone (α-MSH) analogues, commonly known as melanotan I and II, appears to have increased. These analogues are primarily used for their tan-stimulating effects. Dermatologists see many patients in their clinic who tan. This review provides an overview of the risks of the unregulated use of these substances.  Although afamelanotide has been thoroughly tested and deemed safe, illegal melanotans are likely risky for several reasons. There are questions regarding the preparation, administration, and dosage of these substances. In addition to these general risks, increasing numbers of case reports indicate that the unregulated use of both melanotan I and II is associated with cutaneous complications, particularly melanocytic changes in existing moles and newly emerging (dysplastic) nevi. Four case reports have described melanomas emerging from existing moles either during or shortly after the use of melanotan. Although conclusive evidence linking these phenomena is lacking, publications have stressed the importance of awareness that melanotan is a part of a 'tanning culture' in certain subpopulations. Multiple national health organizations have issued safety warnings regarding the use of melanotan I and II.