Clinicoepidemiological features and course of 43 cases of bullous pemphigoid in Kuwait.

BACKGROUND: The clinicoepidemiological characteristics and course of bullous pemphigoid (BP) have not been described in populations from the Arabian Gulf. Hypothesis. Ethnic and regional variations can influence the clinical behaviour and course of autoimmune diseases. METHODS: In this study, 43 patients with BP, registered in our autoimmune bullous diseases (ABD) clinic over a span of 14 years, were studied to determine the clinicoepidemiological features and course of the disease in our region. RESULTS: BP was observed to be the second commonest ABD in our clinic (27%), with a minimum estimated incidence of 2.6 cases/million/year among the referral population. The largest proportion (93%) of the patients was of Arab ethnicity and the female to male ratio was 5.1 :1. Mean +/- SD age at diagnosis was 65.20 +/- 18.80 years. Most of the patients (96%) had moderate to severe disease, and mucosal involvement was seen in 37% of the patients. Systemic steroids (prednisolone 20-60 mg daily) alone or in combination with azathioprine, intravenous immunoglobulins, tetracyclines, mycophenolate mofetil or dapsone were used to treat theses cases. At the last follow-up, 32% of patients were in complete remission and off treatment. The first-year mortality was 30%. Old age and poor general medical condition were the significant risk factors (P < 0.05) contributing to the mortality. CONCLUSION: The study highlights the regional variations of BP and thus a need to uncover the ethnic, genetic and geographical influences, if any, responsible for these variations.

Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with adenocarcinoma of prostate and transitional cell carcinoma of urinary bladder.

Association of Sweet's syndrome (SS) with solid tumours is found in about 15% of all malignancy-associated cases, but an association with two malignancies occurring in the same patient has been rarely reported. In the present report, we describe an 82-year-old male with SS in association with adenocarcinoma of the prostate and transitional cell carcinoma of the urinary bladder.

Clinicopathological features and HLA tissue typing in pemphigoid gestationis patients in Kuwait.

Pemphigoid gestationis (PG) is a rare autoimmune disease of pregnancy. We report a series of 22 cases of PG in Kuwait. They constituted 18% of all the autoimmune bullous diseases registered in our centre over a span of 11 years. PG was observed to be the third most common bullous disease in our region. Ninety-five per cent of the patients were of Arab ethnicity. The clinical features observed in our patients were comparable to those reported elsewhere. Systemic steroids (prednisolone 20-60 mg daily) remained the mainstay of treatment to control the active disease and an optimal dose of 20 mg of prednisolone was maintained throughout the pregnancy and immediate postpartum period. We observed a favourable outcome of pregnancies complicated by PG without any associated maternal or foetal morbidity. Kuwaiti patients with PG were observed to have a predominance of HLA-DR3 and DQ2 antigens. No predominance of HLA-DR4 antigen was observed.

Childhood lichen planus: a report of 23 cases.

Lichen planus (LP) in children is a rare entity. We report 23 cases of childhood LP seen over a period of 7 years. Ninety-six percent of the children were of Arab ancestry. There were 52% boys and 48% girls. Classic LP was the most common clinical variant (70%), followed by eruptive generalized LP (13%). A majority of the patients had mild, localized disease. Oral involvement was seen in 39% of patients. Topical steroids were the mainstay of treatment in most of the cases. Children with chronic and recurrent disease responded to dapsone therapy, whereas in those with eruptive and widespread disease, UVB phototherapy was found to be safe and effective. The present report highlights the salient clinical features, treatment, and course of LP in children in Kuwait compared to those reported in children of other countries as well as those of adults.

Lipoid proteinosis: report of four siblings and brief review of the literature.

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal recessive disorder associated with deposition of periodic acid-Schiff (PAS)-positive hyaline material in various tissues including skin, mucous membranes, and internal organs. A family is reported in which four siblings (two boys and two girls) born to nonconsanguineous parents had lipoid proteinosis. All had the characteristic hoarseness of voice and three had skin lesions. The diagnosis was confirmed by the presence of typical features on light and electron microscopy.

Milia en plaque: a new site.

A 35-year-old Kuwaiti field worker presented with a history of an asymptomatic, erythematous plaque on the right side of the nasal bridge. It soon extended to the malar area, being studded with multiple yellowish papules (Fig. 1). He denied any history of photosensitivity, drug intake, local trauma, topical applications, or ionizing radiations to that area. Examination revealed an erythematous, 1.5 x 3 cm plaque on the right nasal fold, extending to the malar area, overlain by a group of tiny yellowish papules (15-20 in number). He also had a few discrete milia on the right cheek. The histology (Fig. 2) revealed multiple keratin-filled cysts, surrounded by a dense lymphocytic infiltrate, findings consistent with milia; 0.05% tretinoin was prescribed twice daily for 1 month without improvement; minocycline, 100 mg daily, was then employed, and at 1 month of follow-up there was a significant decrease in erythema and milia count.

Basal cell epithelioma arising from epidermoid cyst.

BACKGROUND: Basal cell epithelioma is a tumor arising from a pluripotential cell found within the basal cell layer of the epidermis or various appendageal structures. OBJECTIVE: We report two cases of basal cell epithelioma arising from the wall of an epidermoid cyst. METHODS AND RESULTS: Review of our files produced two cases of basal cell epithelioma arising from a cyst wall. CONCLUSION: Basal cell epithelioma arising from the wall of an epidermoid cyst is rare. Examination of all cysts removed surgically is important as not to overlook this possibility and to allow close follow-up for potential recurrence.