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Patient Case: Familial Atypical Mole Syndrome

Patient Case: Familial Atypical Mole Syndrome

Discussion

A 35-year-old man presents to a dermatology clinic for evaluation of multiple unusual moles on his back, chest, and arms. He reports that his father and older brother both had melanoma, and he is concerned about his own risk. He states that he has had these moles for years, but recently noticed that some appear to be getting darker and slightly larger.

On physical examination, the patient has over 50 melanocytic naevi, some of which are more than 5 mm in diameter with irregular borders and varying shades of brown. A dermoscopic exam reveals mild asymmetry and pigment network irregularities in several lesions. A biopsy of one of the most concerning lesions shows architectural atypia but no dermal invasion.

The dermatologist educates the patient on his increased risk of melanoma and recommends strict sun protection, routine skin self-examinations, and follow-ups every six months. A full-body photographic record is created for monitoring, and the patient is advised to seek immediate medical attention if any mole changes significantly in shape, size, or color.

Questions
  1. Which of the following best describes familial atypical mole syndrome?
    a) A benign condition with no risk of melanoma
    b) A hereditary disorder characterized by multiple atypical naevi and an increased risk of melanoma
    c) A viral skin condition causing wart-like lesions
    d) A bacterial infection leading to mole ulceration
  2. Which feature of the patient’s history is most concerning for melanoma risk?
    a) Having more than 50 moles
    b) A family history of melanoma
    c) Some moles appearing darker over time
    d) All of the above
  3. What is the most appropriate management for this patient?
    a) Immediate excision of all atypical moles
    b) No further follow-up since the biopsy was benign
    c) Routine dermatologic follow-ups, sun protection, and self-examination
    d) Use of over-the-counter skin-lightening creams
  4. Which of the following is NOT a typical dermoscopic feature of atypical naevi?
    a) Irregular pigment network
    b) Homogeneous blue-black coloration
    c) Asymmetry in shape and color
    d) Ill-defined borders
Reveal answers

Answers

  1. (b) A hereditary disorder characterized by multiple atypical naevi and an increased risk of melanoma
    • Familial atypical mole syndrome is an inherited condition where individuals develop numerous atypical naevi and have an elevated risk of developing melanoma.
  2. (d) All of the above
    • The combination of multiple moles, a family history of melanoma, and changing lesions significantly increases the patient’s risk of melanoma.
  3. (c) Routine dermatologic follow-ups, sun protection, and self-examination
    • Management focuses on early detection of melanoma through regular skin checks, strict sun protection, and close dermatologic monitoring.
  4. (b) Homogeneous blue-black coloration
    • A homogeneous blue-black color is more characteristic of blue naevi or melanoma, rather than atypical naevi, which tend to have irregular pigment networks and ill-defined borders.