Epidermolysis bullosa in the eastern province of Saudi Arabia.

BACKGROUND: Epidermolysis bullosa is recognized to be rare, but its prevalence in the Eastern Province of Saudi Arabia had not been previously established. METHODS: We reviewed 49,902 dermatology cases seen in our clinic over a 7-year period (1984 through 1990) and carried out a therapeutic trial of oral phenytoin in three severe cases of epidermolysis bullosa dystrophica (Recessive dystrophic type). RESULTS: Sixteen cases of epidermolysis bullosa were found in this series, ten (62.5%) of which were of the dermolytic type (epidermolysis bullosa dystrophica), and four (25%) of the epidermolytic type (epidermolysis bullosa simplex). Parental consanguinity was established in fourteen (87.5%) of these cases. All ten cases of epidermolysis bullosa dystrophica developed complications. Three severe cases were treated with oral phenytoin and managed with meticulous nursing and nutrition with blended foods and protein and vitamin supplements and responded satisfactorily. CONCLUSIONS: This study confirmed the rarity of epidermolysis bullosa in this province (population 3,000,000), and demonstrated the usefulness of oral phenytoin therapy, meticulous nursing, and good nutrition in the management of epidermolysis bullosa dystrophica.

An open non-comparative pilot study of the safety and efficacy of oral fluconazole in the treatment of dermatophyte infections in Nigeria

In an non-comparative study to assess the efficacy and safety of oral fluconazole in superficial fungal diseases of the skin; 82 adult Nigerian patients (58 males and 24 females) with clinical and mycological diagnosis of dermatomycoses were enrolled for the trial. Sixty-five patients completed the trial and each of them received a daily dose of 50mg fluconazole for a period of 4 weeks. An overall 90 per cent cure rate was observed in patients with other forms of dermatomycoses and 80 per cent for patients with pityriasis versicolor. The drug was well tolerated by all the patients and very few side effects were noticed

Recessive dystrophic epidermolysis bullosa treated with phenytoin.

Three patients with severe recessive dystrophic epidermolysis bullosa were treated with oral phenytoin and palliative and supportive measures for variable periods. Their progress was compared with that of three milder cases managed only with palliative and supportive measures. The phenytoin-treated group showed marked decrease in blister count, increase in trauma tolerance, a rise in hemoglobin level, and considerable weight gain. The results support earlier reports that collagenase inhibitors are useful in controlling blister formation in recessive dystrophic epidermolysis bullosa.

Albinism in Nigeria with delineation of new recessive oculocutaneous type.

Seventy-nine Nigerian oculocutaneous albinos were investigated. Fifty-six had typical tyrosinase-positive albinism (TPA) and 23 had brown albinism (BA), a new oculocutaneous type. The TPA were characterized by localized but no generalized skin pigment, yellow hair, blue to brown irides, nystagmus, and reduced or absent retinal pigment. Localized skin pigment included freckles and lentigines. The iris and skin pigment were the result of the slow accumulation of pigment with age as both were found in older individuals. The most severe skin changes were premalignant keratoses and squamous cell carcinoma of the skin, and the skin malignancies were the major factor in limiting the lifespan for TPA. The BA were characterized by generalized light brown skin pigment, light brown hair, blue to brown irides, nystagmus, and reduced retinal pigment. There was little accumulation or change of pigment in the eyes or skin with age. The generalized light skin pigment was effective in reducing sensitivity to solar radiation and very few BA had premalignant keratoses. Pedigree analysis for BA suggested on autosomal recessive inheritance pattern.

Chromosome breaks and sister chromatid exchanges in albinos in Nigeria.

The prevalence of squamous cell carcinoma of the skin in albinos reaches approximately 90% in patients over 20 years of age in the vicinity of Enugu, Nigeria. Chromosome breaks and sister chromatid exchanges (SCE) were evaluated in tyrosinase-positive oculocutaneous albinos and pigmented controls of Ibo extraction who were life-long residents of Nigeria. No difference in the frequency of chromosomal breaks in 14 albinos compared to 6 pigmented controls, and no differences in the frequency of SCE in 9 albinos compared to 3 controls could be detected. Increased chromosomal abnormalities in lymphocytes do not appear to be assoicated with albinism or fulminating skin cancer present in albinos in the tropics.

African histoplasmosis in Eastern Nigeria: report of two culturally proven cases treated with septrin and amphotericin B.

Two cases of African histoplasmosis culturally proven to be caused by Histoplasma duboisii are reported from Eastern Nigeria. One case had cutaneous papules and subcutaneous nodules all over his body while the other patient had cutaneous papules, an abscess over the right scapula, enlarged cervical, axillary and inguinal lymph nodes, and possible lung involvement. None of the 35 soil samples collected from the environment of the two patients were found positive for H. duboisii. In the first case, most of the lesions cleared with Septrin given orally. The 2nd case responded very well to treatment with intravenous Amphotericin B except for persistance of swellings in the inguinal lymph nodes. The effect of intralesional injection of Amphotericin B on this residual lymph node infection is now being assessed.

Chromomycosis caused by Philaphora pedrosoi in eastern Nigeria.

Three cases of chromomycosis from Eastern Nigeria are described. The aetiological agent was identified as Phialophora pedrosoi. Amphotericin B cleared most of the lesions in one case while 5-fluorocytosine gave a complete cure in another case.

Albinism in Nigeria. A clinical and social study.

A study of 1000 Nigerian albinos, all of Negro stock, showed various types of albinism with their different modes of transmission--oculocutaneous, ocular and cutaneous. The much higher incidence among the more settled communities in the south, compared with the more nomadic communities in the north, may be related to greater inbreeding tendencies in the south. The sun and society are hostile to the albinos. Under the tropical sunshine, their melanin-deficient skin develops wrinkles, lentigines, actinic keratoses and epitheliomata from which they may die in early adult life or in middle age. Myopia and other ocular defects retard the progress of many albinos in school and they eventually drop out to seek disastrous menial outdoor occupations. Registering albinos early in life, assuring their families that albino defects are confined to the skin and eyes, advising on protective clothing and sun-screening agents, correcting myopia, assisting with indooor occupations, and early treatment of actinic keratoses and skin cancer should help many albinos to attain social acceptance and a ripe old age.