Skin Changes in Proteus Syndrome Thomas N. Darling, M.D. Ph.D. …

Skin Changes in
Proteus Syndrome

 Thomas N. Darling, M.D. Ph.D.
 Department of Dermatology
 Uniformed Services University
Objectives

 Identify common skin lesions
 Anticipate changes in skin lesions
 Apply principles of good skin care
 List risks and benefits of potential treatments
Types of Skin Lesions

 Epidermal nevus
 Vascular malformations
 Lipomas
 Connective tissue
 nevus
 Others
Locations of Skin Lesions

Epidermal
Nevus                Torso, Arms, Legs

Vascular             Torso and Legs
Malformation


Lipoma               Torso

Connective           Feet and Hands
Tissue
Nevus
Onset of Skin Lesions
                                    10


                                     8


Epidermal                            6




Nevus
                                     4


                                     2
                                                             First year
                                     0

                                    10


                                     8



Vascular                                                     First month
               Number of Patients



                                     6


                                     4

Malformation                         2


                                     0

                                    10




                                                             Later in first year
                                    8




Lipoma
                                    6


                                    4


                                    2


                                    0


                                    10


Connective                           8


                                     6
                                                             Later in first 2 years
Tissue                               4



Nevus                                2


                                     0




                                         A   B   C   D   E
Additional Skin Lesions
                                    12

                                    10



Epidermal                           8




Nevus
                                    6

                                    4

                                    2
                                                      Few new lesions
                                    0

                                    12

                                    10



Vascular
                                    8


                                                      Few new lesions
               Number of Patients



                                    6



Malformation                        4

                                    2

                                    0

                                    12




                                                      New lesions later
                                    10

                                    8

Lipoma                              6

                                    4

                                    2

                                    0

                                    12


Connective                          10

                                    8
                                                      New lesions later
Tissue                              6

                                    4


Nevus                               2

                                    0




                                         None   New
Growth of Skin Lesions
                                    12

                                    10



Epidermal                           8




Nevus
                                    6

                                    4

                                    2
                                                           Stable
                                    0


                                    12

                                    10



Vascular
                                     8


                                                           Stable
               Number of Patients



                                     6



Malformation                         4

                                     2

                                     0

                                    12




                                                           Spread
                                    10

                                     8

Lipoma                               6

                                     4

                                     2

                                     0

                                    12


Connective                          10

                                     8
                                                           Spread
Tissue                               6

                                     4


Nevus                                2

                                     0




                                         Stable   Spread
Trends

 Early onset, stable size and number
   Epidermal nevus
   Vascular malformations (capillary and
   venous, not lymphatic malformations)

 Later onset, new lesions, grows
   Lipomas
   Connective tissue nevus
Common Treatment Concerns

 Appearance
 Impaired function
 Pain
 Dry skin
 Sensitive skin
 Odor
Treatment of Dry Skin

 Washing
  Tepid water
  Non soap cleanser (e.g. Cetaphil)
  Bath oil
  5 - 10 minutes
  Shower
  Pat dry
Treatment of Dry Skin

 Moisturizers
  Apply after bathing and 2 ­ 4 times a day
  Lotions, ointments
  Active ingredients
    Alpha hydroxy acid, salicylic acid, ammonium lactate,
    urea
  Patient approval
Treatment of Sensitive Skin

  Treat dry skin
    Watch for preservatives, fragrances in
    moisturizers
  Avoid irritants
    Fabrics (wool, rough synthetic clothing)
    Soaps and detergents (industrial skin cleansers,
    borax, cleansers with abrasives)
    Solvents (gasoline, kerosene, turpentine)
    Acids, alkalis, metal salts, plants
Causes of Odor

 Moisture
 Microorganisms
 Skin folds
Treatment of Odor

 Keep skin dry
  Shoes that breathe
  Cotton socks
  Change socks
  Foot powder
  Hair dryer
  Gauze wedge
Treatment of Odor

 Reduce perspiration
  Antiperspirants
    Aluminum chloride (Certain Dri)
  Iontophoresis (Drionic)
  ? Botox injection
Treatment of Odor

 Reduce bacteria
  Antimicrobial cleanser
    Switch brand periodically
  Foot soaks
    10 min, 2-3 times daily
    Burow's solution (Domeboro), dilute vinegar
  Clean between skin folds (cotton-tipped
  applicator)
Treatment of Odor

 Prescription
  Aluminum chloride solution (e.g. Drysol)
  Topical antibiotics (e.g. Clindamycin solution)
  Treatment of infection (oral antibiotics, topical or
  oral antifungals)
Treatment of Skin Lesions
 Different treatments for different types of skin
 lesions
 Seek treatment for:
   Oozing, bleeding
   Signs of infection (warmth, fever, redness,
   swelling, pain)
   Rapid change or growth
Types of Treatment
 Medical
  Topical, Oral
  Gradual and modest improvement
 Surgical
  Scalpel, Laser, Dermabrasion, Liposuction
  Dramatic change
  Scarring, recurrences
Choosing the Right Treatment
 Choice of therapy
  Reasons for treatment
  Effective
  Side effects
  Ease of use
 Timing of therapy
  Functional impairment, growth of lesion
  Age
Connective Tissue Nevi

 Good skin care
 Surgery ­ Caution!
   Excision (at time of orthopedic surgery)
   Scars on the sole of foot can be very painful
Epidermal Nevi
 Medical
  Topical retinoids, keratolytics
  Systemic retinoids
 Surgical
  Excision, shave
  Laser ablation
  Dermabrasion
Vascular Malformations
 Capillary malformation
  Pulsed-dye lasers
  Intense pulsed light source
 Venous malformation
  Compression stockings
  Embolization
  Sclerotherapy
  Excision
Vascular Malformations
 Lymphatic malformations
  Leave undisturbed
  Excision
  Sclerotherapy
  Control infection
Lipomas
Surgical
  Excision
  Liposuction
Summary

 Children and adults with Proteus syndrome
 have special needs for skin care ­ dry skin,
 sensitive skin, odor
 Different types of skin lesions are treated in
 different ways, some medically and some
 surgically
Thank you!

 Proteus Syndrome Foundation
 NIH
 Patients and Families