Table 4.

Treatment of Manifestations in Individuals with Hereditary Fibrosing Poikiloderma with Tendon Contractures, Myopathy, and Pulmonary Fibrosis

Manifestation/
Concern		TreatmentConsiderations/Other
Poikiloderma
  • Avoidance of excessive sun exposure
  • Use of sunscreens w/both UVA & UVB protection
Pulsed dye laserCan be discussed w/older patients for cosmetic management of telangiectatic component of the rash, but no data on its use are available.
Hypohidrosis
  • Avoidance of excessive heat exposure
  • Control of fever
Especially in 1st yrs of life to prevent overheating
Lymphedema
  • Manual lymphatic drainage
  • Compression
Eczema-like
lesions 		Topical treatment (e.g., emollients, topical steroids)
Muscle
contractures
& weakness
  • PT & exercise to promote mobility & prevent contractures
  • Calcium & vitamin D supplements may also be warranted in those w/muscle weakness to prevent osteopenia.
Lung disease
  • Use of self-inflating manual ventilation bag or mechanical insufflation-exsufflation device if needed
  • Noninvasive ventilation if needed
Pancreatic
exocrine
insufficiency 		Pancreatic enzyme supplementation
Liver
impairment 		Treatment of cholestasis w/ursodeoxycholic acid
Growth/
Nutrition 		Food supplement or enteral feeding as needed for poor weight gain
Cataract Surgical removal of visually significant cataracts

PT = physical therapy

From: Hereditary Fibrosing Poikiloderma with Tendon Contractures, Myopathy, and Pulmonary Fibrosis

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