Linear immunoglobulin a bullous disease of childhood responsive to intravenous immunoglobulin monotherapy.

We report a case of linear immunoglobulin A bullous disease in a 9-year-old boy who presented with rapidly progressive severe disease and could not tolerate dapsone because of high liver enzymes within a week after a low dose of dapsone in association with an underlying fatty liver. He showed remarkable improvement with intravenous immunoglobulins used as monotherapy, with a rapid clearance and a sustained remission after stopping the treatment.

Clinicoepidemiological features of mycosis fungoides in Kuwait, 1991-2006.

BACKGROUND: Mycosis fungoides (MF) is an indolent, most common type of cutaneous T-cell lymphoma (CTCL) with an average estimated incidence of 0.5 cases per 100,000 persons per year in the western world. Although various clinical and epidemiological features are well delineated in the western population, the data is scarce from our region. OBJECTIVES: To study the clinicoepidemiological features of MF from Kuwait. SETTING: A referral photobiology unit for cutaneous lymphomas in a national dermatology department in collaboration with three other dermatology departments in Kuwait and Kuwait cancer center. PATIENTS AND METHODS: One hundred and ninety-three cases of MF registered between July 1991 and June 2006 were included for this study. RESULTS: Eighty-six percent of our MF cases were of Arab ethnicity. Males outnumbered the females by 2:1 ratio. Mean age at diagnosis was 35.20 ± 14.37 years, and 16% of the patients were diagnosed by the age 20 years. The annual incidence rate (IR) of MF in Kuwait was observed to be 0.43 cases per 100,000 persons with a significantly higher IR among Arabs as compared to non-Arab Asians (RR = 4.4; 95% CI = 2.9-6.6). A successive rise in the IR of MF was noticed with the advancing age. The annual IR among males was more or less comparable to that of females. Skin patches were the most prevalent skin lesions (67%) at diagnosis, and 22% of the patients had a pure hypopigmented variant. Patients with hypopigmented MF were observed to have younger mean age at diagnosis (27.60 ± 12.42 years) as compared to other MF cases (38.14 ± 14.37 years) (P = 0.000). Ninety-two percent of the patients had the early stage (IA, IB, and IIA) of disease. CONCLUSIONS: Our patients with MF were observed to have a relatively younger age at diagnosis, with a high proportion of patients diagnosed by the age 20 years. Arabs were observed to have a higher annual IR of MF as compared to non-Arab Asians. Hypopigmented MF is prevalent in our population. The study highlights the ethnic and/or regional variations in the clinicoepidemiological characteristics of MF.

Mycosis fungoides in Arab children and adolescents: a report of 36 patients from Kuwait.

Mycosis fungoides (MF) is rare in children and adolescents. This study was aimed to determine the clinicoepidemiologic features of juvenile onset (≤18 yrs) MF in Kuwait. Thirty-six children and adolescents (≤18 yrs) with MF registered in a referral photobiology unit for cutaneous lymphomas between July 1991 and June 2009 were included in this study. Children and adolescents were observed to constitute 16.6% of the total number of patients with MF, with 97% of patients of Arab ethnicity. The age-adjusted incidence rate of MF in children and adolescents among the total population was 0.29/100,000 persons/year. Among 36 Arab children and adolescents, boys outnumbered girls by 1.25:1. Mean and median age at onset of disease was 9 years, and age at diagnosis was 13 years. Patch stage disease was the most common clinical variant (75%) with 56% with pure hypopigmented MF-variant. The majority of patients (75%) had stage IB (TNM and B staging) disease. The study highlights a high prevalence and incidence of juvenile MF in Kuwait with a predominantly hypopigmented presentation.

Congenital yellow nail syndrome: a case report and its relationship to nonimmune fetal hydrops.

Yellow nail syndrome (YNS) is an uncommon disorder characterized by a triad of nail dystrophy, lymphedema, and pleural effusion. It is rare in children and congenital occurrence of YNS has been very rarely described. We report a 2-year-old Arab boy having congenital yellow nail syndrome with mild facial dysmorphism and bilateral conjunctival pigmentation born to consanguineous parents. One of his older siblings had died of nonimmune fetal hydrops (NIFH). The case supports the genetic basis of yellow nail syndrome with a possible relationship to nonimmune fetal hydrops.

GAPO syndrome: a report of two siblings and a review of literature.

Growth retardation, alopecia, pseudoanodontia, optic atrophy (GAPO) syndrome is a rare autosomal recessive disorder. The molecular nature of the disease is not fully understood and is considered to be one of the ectodermal dysplasia defects. In this report, we describe clinical, histologic, and ultrastructural features in two siblings born to consanguineous parents with a brief review of the literature.

Multiple milia in a newborn with congenital malformations: oral-facial-digital syndrome type 1.

Oral-facial-digital syndrome type 1 (OMIM #311200) is an X-linked dominant, developmental disorder. Among the 13 described clinical variants of oral-facial-digital syndrome, oral-facial-digital syndrome type 1 is of significance to dermatologists due to presence of congenital milia and hypotrichosis, not described in other variants. Since oral-facial-digital syndrome type 1 is genetically a distinct entity, awareness of these features help to clinically delineate this from other variants.

Gerodermia osteodysplastica/wrinkly skin syndrome: report of three patients and brief review of the literature.

Gerodermia osteodysplastica and wrinkly skin syndrome are rare autosomal recessive disorders. Due to the many phenotypic similarities in these two conditions, it has been proposed that they represent the same disorder. Both conditions are well delineated in the genetic literature, but despite skin involvement being a striking feature, they are rarely reported in dermatology journals. In this report, we describe three Arab children from two consanguineous families who exhibit overlapping features of gerodermia osteodysplastica and wrinkly skin syndrome. All the patients had dysmorphic facial features, wrinkled skin more marked on the hands and feet, hyperextensible joints, intrauterine growth retardation, developmental delay, congenital dislocation of hips, and osteoporosis. Our observations also support the contention that gerodermia osteodysplastica and wrinkly skin syndrome have the same clinical spectrum; however, this needs to be confirmed at the molecular level.

Linear IgA bullous disease of childhood: an experience from Kuwait.

Linear IgA bullous disease of childhood is a rare autoimmune blistering disease. We report eight patients with this disease seen in our autoimmune bullous diseases clinic over a span of 12 years. They constituted 89% of the total number of those with linear IgA bullous disease of childhood seen during this period, with an age-adjusted minimum estimated incidence of 2.3 cases/million population/year. Males outnumbered females by a 1.7:1 ratio. The age at onset ranged between 10.5 months and 13 years, with a mean of 6.8 +/- 4.17 years. The majority of patients (62.5%) had moderately severe disease. Fifty percent of patients were observed to have an association with either an autoimmune disease (Crohn disease and post-streptococcal glomerulonephritis in one each) or an infection (beta-hemolytic streptococcal and hepatitis A virus infection in one each). The treatment of choice was dapsone alone or in combination with systemic steroids. Seventy-one percent of patients achieved complete remission by the end of 2 years. The study highlights the significance of systematic clinicoepidemiologic surveys from different regions.

Spectrum of autoimmune bullous diseases in Kuwait.

BACKGROUND: Autoimmune bullous diseases (ABDs) are a rare but significant group of dermatoses that pose great challenges to the treating dermatologist. Most epidemiological studies have focused on a single ABD. Few surveys have been carried out to describe the whole spectrum of ABDs in a region, and no such studies are available from the Arabian Peninsula. OBJECTIVES: To determine the clinico-epidemiological features of various ABDs in Kuwait, and to compare the results with those reported elsewhere. METHODS: A total of 128 cases of ABDs were studied over a span of 11.5 years. The diagnosis in all cases was confirmed by histopathology, and direct and indirect immunofluorescence (IMF). The diagnosis of various subepidermal ABDs was further confirmed by indirect IMF on salt-split skin (SSS) and that of pemphigus by desmoglein 1 and 3 enzyme-linked immunosorbent assay (ELISA). RESULTS: Eighty seven per cent of patients were of Arab ethnicity. Pemphigus was observed to be the commonest ABD (47%), followed by pemphigoid (22%), pemphigoid gestationis (PG) (19%), linear IgA bullous disease (LABD) (7%), lichen planus pemphigoides (LPP) (3%), and epidermolysis bullosa acquisita (EBA) (2.3%). The minimum estimated incidence in the local population was 4.6, 2.14, 1.83, 0.69, 0.30, and 0.23 cases per million per year, respectively. Pemphigus patients were observed to have a younger age of onset (36.50 +/- 11.36 years) than reported elsewhere. BP, although the second commonest ABD, was less prevalent than in Europe and Singapore, and BP patients were observed to have a striking female predominance (85%). The prevalence of PG was much higher than that reported elsewhere. LABD was the fourth commonest ABD, and 89% of patients were children. CONCLUSIONS: The study suggests that similar surveys from different regions would expand our understanding of ABD.

Unilesional (segmental) mycosis fungoides presenting in childhood.

Mycosis fungoides is rare in children, and a unilesional presentation is also rare. A 13-year-old Kuwaiti boy with unilesional mycosis fungoides is described. Clinically he had a single indurated large plaque on the left shoulder with histopathologic features typical of cutaneous T-cell lymphoma. The diagnosis was further supported by the presence of a T-cell clone discovered through molecular biology studies of paraffin-embedded material. No other lesions were detected. The lesion showed a favorable response to local radiotherapy.

Thyroid function, autoantibodies, and HLA tissue typing in children with alopecia areata.

A total of 80 Kuwaiti children with alopecia areata (AA), without clinical evidence of thyroid disease, were screened for the presence of thyroid abnormalities, and 50 unrelated children with AA were tissue typed for human leukocyte antigen (HLA) class I and class II antigens. Thyroid abnormalities were detected in 14 children (17.5%). Among these, 11 children (14%) had thyroid autoantibodies. These observations highlight the significance of screening for thyroid abnormalities in children with chronic, recurrent, and/or extensive disease. The Kuwaiti children with AA were observed to have a significant association with HLA B21 (OR 18.850, 95% CI 4.404-80.677), B40 (OR 6.767, 95% CI 1.818-25.181), and HLA B12 (OR 4.833, 95% CI 1.198-19.505) antigens. These findings differed from those reported elsewhere.