Clinicopathological study of 55 cases of immunobullous diseases in Jordan

Abstract

Background: Immunobullous skin diseases are a serious group of skin disorders with variable presentations that need to be diagnosed early for better prognosis. A proper clinical and histopathological evaluation is needed for correct diagnosis, so it is an important issue to share experience and to highlight this hot spot in dermatology. Objective: To have a clinical and histopathological insight into the immunobullous skin diseases as a group to show their distribution in our region and to share experience for proper early diagnosis. Material and Methods: All cases of immunobullous skin diseases that had been diagnosed clinically and histopathologically at referral centers in Jordan, Amman (King Hussein Medical Center, Jordan University hospital) and in private practice from Jan. 2000 to Jan. 2012 were included in this retrospective study. All cases were analyzed clinically and histopathologically. Hematoxylin and Eosin stained sections were used for histopathological analysis. Immunoflourscence study was done on 16 cases only and Tzanck smear examination after staining with Giemsa stain was done on 5 cases. Results: From Jan. 2000 to Jan. 2012 a total number 55 cases of different immunobullous skin diseases had been diagnosed clinically and histopathologically from a total number of 2156 skin biopsies that had been performed for different reasons and examined during that period of time. There were 21 male patient and 34 female patients with M:F ratio 1.2: 2. Age range of the patients is 4-85 yr. The distribution of different immunobullous diseases in decreasing frequency was; bullous pemphigoid 24 patients (44%), Pemphigus vulgaris 14 patients (25%), Pemphigus folaceous 5 cases (9%), Pemphigoid gesatationis 4 patients (7%), dermatitis herpetiformes 3 patients (5%) chronic bullous dermatosis of childhood 2 patients (8%), pemphigus erythematosus, one patient (4%), linear IgA dermatosis, one patient (4%), and lichen planus Pemphigoides one patient (4%). Histopathological features were conclusive in most of the cases. Tazanck smear test was a helpful test in cases of pemphigus group. The study reveals a rare form of pemphigus vulgaris as pemphigus with scalp lesions only. Rare histopathological features of bullous pemphigoid including cell poor blister and festooning were also seen. Conclusion: Immunobullous skin diseases are serious skin diseases that affect all age groups. Proper clinical and histopathological examination is essentially conclusive for diagnosis. In some cases immunoflourscence study is needed for diagnosis. Simple bedside test like Tzanck smear test is helpful in some cases