Premium
Hailey‐Hailey disease in Tunisia
Author(s) -
BenmouslyMlika R.,
Bchetnia M.,
Deghais S.,
Ben Brick S.A.,
Charfeddine C.,
Debbiche A.,
Haouet S.,
Mokni M.,
Abdelhak S.,
Kamoun M.R.,
Ben Osman A.,
Fenniche S.,
Mokhtar I.
Publication year - 2010
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4632.2010.04403.x
Subject(s) - medicine , disease , hailey–hailey disease , consanguinity , pediatrics , depression (economics) , dermatology , economics , macroeconomics
Summary Background Most of the published reports on Hailey‐Hailey disease (HHD) come from European and Asian countries. We report here the clinical and genetic investigation of 20 patients affected with HHD in Tunisia. Methods Affected individuals from three large teaching hospitals in Tunis were recruited for the study over a 25‐year period. Nine patients were identified through the active files and examined together with their family members that were visited in their respective regions. We have clinically examined in total 65 individuals and then identified 11 new cases. Patients were included on the basis of evocative skin lesions, biopsy proven HHD and negative immunofluorescence. Investigations to rule out fungal, bacterial and viral infections were done according to clinical symptoms. Results Twenty patients (12 males and 8 females) from 8 families were included in the present study with more than 55% that were undiagnosed before this investigation. Four patients had mild disease, eight had moderate disease and another eight had severe disease, among whom seven were females. Parental consanguinity was found in 7 cases out of 20 cases (35%). The neck region was first affected in half (4/8) of the male patients. Groins were first affected in 42% (5/12) of the female patients. Depression complicated the course of the disease in two female patients with severe HHD. We report an original association of supernumerary nipples with HHD in two sisters from the north of Tunisia. In 10 patients, the disease has become less troublesome with aging. Conclusion HHD is underestimated. Physicians must be aware of this disease in case of resistant intertriginous dermatosis especially with a positive family history as nine out of 20 patients were misdiagnosed.