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Punctate palmoplantar keratoderma associated with morbus Bechterew and HLA B 27. A family study
1Department of Dermatology, Central Hospital, Halmstad, Sweden.
Acta Dermato-Venereologica
|January 1, 1988
Abstract:
Four patients in a family with punctate palmoplantar keratoderma (Buschke-Fischer) associated with Morbus Bechterew and HLA B 27 in 3 of the family members are reported. Without severe side effect, the proband was successfully treated with 50 mg etretinate per day for 6 weeks.
Insights
This study reports a family with punctate palmoplantar keratoderma linked to Morbus Bechterew and HLA-B27. The proband showed successful treatment outcomes with etretinate, a retinoid medication.
Area of Science:
- Dermatology
- Genetics
- Rheumatology
Background:
- Punctate palmoplantar keratoderma (PPKP) is a rare skin condition.
- Morbus Bechterew (ankylosing spondylitis) is an inflammatory disease.
- HLA-B27 is a genetic marker associated with autoimmune diseases.
Observation:
- A family presented with PPKP, Morbus Bechterew, and HLA-B27 positivity in three members.
- The proband, diagnosed with PPKP, also exhibited Morbus Bechterew and HLA-B27.
- This suggests a potential genetic link between these conditions.
Findings:
- The proband received a 6-week course of etretinate at 50 mg/day.
- Treatment with etretinate resulted in successful management of PPKP symptoms.
- No severe side effects were reported during the treatment period.
Implications:
- Etretinate may be an effective therapeutic option for PPKP associated with Morbus Bechterew.
- Further research into the genetic basis of this familial condition is warranted.
- Understanding the interplay between PPKP, Morbus Bechterew, and HLA-B27 could improve patient management.