Hamida EL-Dwebi, Mohamed Shafi, A lhashmie Mashina.
1- Dermatology department, Tripoli Central Hospital, Tripoli- Libya.
2-Dermatology department, Bir EL-Aussta Milad, Tajura
JMJ Vol.4 No. 3 (Winter) 2005: 229-230
Hailey-Hailey disease (familial benign chronic pemphigus) is a rare autosomal dominant inherited disease due a genetic defect in calcium ATP-ase. Hailey-Hailey disease (H.H disease) is recognized clinically by recurrent patches and plaques of flaccid vesicles or vesiculopustular lesions on an erythematous base affecting mainly flexural areas. A 37 years old man was referred to our clinic because of painful fissures within erosive erythematous plaques located on the sides of neck, groins and inner thighs of 8 years duration and negative family history. Histopathology examination confirmed the diagnosis of the Hailey-Hailey disease. The patient did well on local steroid and antifungal cream. To our knowledge this is the first reported case of H.H disease in Tripoli and we recommended that for any patient with recurrent and persistent disease of the flexures a biopsy should be preformed.
Keywords: Pemphigus, Hailey-Hailey disease, Intertriogous areas