Hypopigmented Facial Macules | Dermatology | JN Learning | AMA Ed Hub [Skip to Content]
[Skip to Content Landing]

Chronic, Scaly, Hypopigmented Macules on the Face

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

An otherwise healthy Black woman in her 30s presented to the dermatology clinic for evaluation of hypopigmented, intermittently pruritic patches. Patches were confined to her face and neck and occurred intermittently after her last pregnancy approximately 10 years ago. She had no history of eczema, atopic skin conditions, or autoimmune conditions and denied family history of such. In previous evaluations, the areas were treated with petroleum-based creams, low- to medium-potency topical steroids, pimecrolimus, and topical antifungals, without consistent improvement. Physical examination identified multiple hypopigmented macules and subtle patches symmetrically distributed across the lower aspect of the forehead, cheeks, nasofacial sulci, and nasolabial folds; and along the jawline and neck (Figure, A and B). Many had a slight overlying scale that was tested with potassium hydroxide and was negative for hyphae. No hair or nail abnormalities were noted. A 4-mm punch biopsy of a hypopigmented patch on the right cheek was performed (Figure, C and D).

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

A. Pemphigus foliaceous

Biopsy revealed focal epidermal parakeratosis with minimal acanthosis. The stratum corneum was uniformly lifted off the granular layer, with subtle portions still attached. The dermis showed mild perivascular lymphocytes and melanin incontinence (Figure, C and D). Periodic acid-Schiff stain result was negative for fungus. Subsequent enzyme-linked immunosorbent assay (ELISA) for antibodies against desmoglein (Dsg) 1 and 3 was positive against Dsg 1. Histology demonstrating a subcorneal blister and positive ELISA for antibodies to Dsg 1 supported the diagnosis of pemphigus foliaceus (PF). The patient began receiving a prednisone taper with transition to mycophenolate mofetil, resulting in improvement.

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

Article Information

Corresponding Author: Kristina Burke, MD, Wenatchee Dermatology, 707 N Emerson, Wenatchee, WA 98801 (kristina.burke@confluencehealth.org).

Published Online: July 14, 2021. doi:10.1001/jamadermatol.2021.2466

Conflict of Interest Disclosures: None reported.

Disclaimer: The views expressed in this article are those of the authors and do not reflect the official policy of the Uniformed Services University, Department of the Army/Navy/Air Force, Department of Defense, or US government.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Schmidt  E , Kasperkiewicz  M , Joly  P .  Pemphigus.   Lancet. 2019;394(10201):882-894. doi:10.1016/S0140-6736(19)31778-7 PubMedGoogle ScholarCrossref
2.
Hans-Filho  G , Aoki  V , Bittner  NRH , Bittner  GC .  Fogo selvagem: endemic pemphigus foliaceus.   An Bras Dermatol. 2018;93(5):638-650. doi:10.1590/abd1806-4841.20188235Google Scholar
3.
Murrell  DF , Peña  S , Joly  P ,  et al.  Diagnosis and management of pemphigus: recommendations of an international panel of experts.   J Am Acad Dermatol. 2020;82(3):575-585.e1. doi:10.1016/j.jaad.2018.02.021 PubMedGoogle ScholarCrossref
4.
Méndez-Flores  S , Avalos-Diaz  E , Dominguez-Cherit  J , Saeb-Lima  M , Esquivel-Pedraza  L .  Pemphigus foliaceus with circinated plaques and neutrophil pustules.   J Cutan Pathol. 2016;43(11):1062-1066. doi:10.1111/cup.12793 PubMedGoogle ScholarCrossref
5.
Lorente Lavirgen  AI , Bernabeu-Wittel  J , Dominguez-Cruz  J , Conejo-Mir  J .  Neonatal pemphigus foliaceus.   J Pediatr. 2012;161(4):768. doi:10.1016/j.jpeds.2012.04.036 PubMedGoogle ScholarCrossref
6.
Jacobson  RR , Krahenbuhl  JL .  Leprosy.   Lancet. 1999;353(9153):655-660. doi:10.1016/S0140-6736(98)06322-3 PubMedGoogle ScholarCrossref
7.
Elgash  M , Dlova  N , Ogunleye  T , Taylor  SC .  Seborrheic dermatitis in skin of color: clinical considerations.   J Drugs Dermatol. 2019;18(1):24-27.PubMedGoogle Scholar
8.
Rodney  IJ , Kindred  C , Angra  K , Qutub  ON , Villanueva  AR , Halder  RM .  Hypopigmented mycosis fungoides: a retrospective clinicohistopathologic study.   J Eur Acad Dermatol Venereol. 2017;31(5):808-814. doi:10.1111/jdv.13843 PubMedGoogle ScholarCrossref
9.
McCarty  M , Fivenson  D .  Two decades of using the combination of tetracycline derivatives and niacinamide as steroid-sparing agents in the management of pemphigus: defining a niche for these low toxicity agents.   J Am Acad Dermatol. 2014;71(3):475-479. doi:10.1016/j.jaad.2014.04.040 PubMedGoogle ScholarCrossref
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_Multimedia_LoginSubscribe_Purchase
Close
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_Multimedia_LoginSubscribe_Purchase
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close

Name Your Search

Save Search
Close
With a personal account, you can:
  • Track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
jn-learning_Modal_SaveSearch_NoAccess_Purchase
Close

Lookup An Activity

or

Close

My Saved Searches

You currently have no searches saved.

Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close