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Antigen-Capture Enzyme-Linked Immunosorbent Assay for Specific Detection of Mycoplasma pneumoniae
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Hailey-Hailey disease in Tunisia.

R Benmously-Mlika1, M Bchetnia, S Deghais

  • 1Dermatology Department, Habib Thameur Hospital-Tunis.

International Journal of Dermatology
|May 15, 2010
PubMed
Summary

Hailey-Hailey disease (HHD) is often underestimated, particularly in Tunisia, with many patients misdiagnosed. This study highlights the importance of clinical and genetic investigation for accurate diagnosis and management of HHD.

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Published on: February 24, 2023

Area of Science:

  • Dermatology
  • Genetics
  • Rare Diseases

Background:

  • Hailey-Hailey disease (HHD) reports predominantly originate from European and Asian countries.
  • This study focuses on the clinical and genetic investigation of 20 HHD patients in Tunisia, a region underrepresented in existing literature.
  • The research addresses the underestimation and misdiagnosis of HHD in the Tunisian population.

Purpose of the Study:

  • To conduct a comprehensive clinical and genetic investigation of Hailey-Hailey disease in Tunisian patients.
  • To identify the prevalence and clinical characteristics of HHD in Tunisia.
  • To raise awareness among physicians regarding HHD, especially in cases of resistant dermatosis with a positive family history.

Main Methods:

  • Recruitment of affected individuals from three major teaching hospitals in Tunis over a 25-year period.
  • Clinical examination of 65 individuals, including 20 confirmed HHD patients and 11 newly identified cases.
  • Inclusion criteria involved characteristic skin lesions, biopsy-proven HHD, and negative immunofluorescence, with exclusion of other infections.

Main Results:

  • Twenty HHD patients (12 males, 8 females) from 8 families were studied; over 55% were previously undiagnosed.
  • Disease severity varied: 4 mild, 8 moderate, and 8 severe cases (7 females).
  • Parental consanguinity was noted in 35% of cases. Original association of supernumerary nipples with HHD observed in two sisters. Disease course improved with age in 10 patients.

Conclusions:

  • Hailey-Hailey disease is frequently underestimated and misdiagnosed, with 9 out of 20 patients misdiagnosed in this cohort.
  • Physicians should consider HHD in cases of persistent intertriginous dermatosis, especially with a family history.
  • Increased awareness and accurate diagnostic approaches are crucial for effective management of HHD.