Epidermolysis bullosa acquisita responsive to cyclosporin therapy.
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Biomedical subjects
Publications and source records attributed to M L Khatri.
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A 26-year-old Libyan woman presented with asymptomatic nodulo-ulcerative skin lesions present for 1 year. Three years prior to presentation, she had experienced a nasal discharge followed by the development of a nodule in the nasal cavity and a plaque on the hard palate. These lesions had gradually increased in size and ulcerated, resulting in perforation of the nasal septum and palate. Two years later, the patient noticed the appearance of skin lesions: a nodule on the right thumb and numerous nodulo-ulcerative lesions on the extremities. General physical examination was normal with no significant lymphadenopathy. Examination of the oral cavity revealed perforation of the distal nasal septum, with a perforated nodular plaque involving the entire palate, associated with subluxation of the upper incisors (Fig. 1a). On skin examination, multiple firm nodules and nodulo-ulcerative lesions with a central eschar and raised margins were observed. The lesions ranged in size from 0.5 to 5 cm and were distributed on the right hand and fingers, left upper arm (Fig. 1b), left calf, and right thigh. Routine laboratory investigations (liver function tests, serum calcium, electrolytes, lipid profile, urine and stool culture studies) were normal. Immunoelectrophoresis disclosed normal levels of immunoglobulins IgG, IgA, and IgM. Serologic studies for human immunodeficiency virus (HIV) and syphilis, and a tuberculin test, were all negative. A Giemsa-stained tissue smear was negative for Leishmania tropica organisms. Radiological studies disclosed a slight haziness of the maxillary sinuses with perforation of the nasal septum. A chest X-ray was normal. Histopathologic examination of biopsies taken from both the palate and from ulcerated and nonulcerated skin lesions was performed, and all showed similar findings. The biopsy of a nonulcerated skin lesion showed pseudoepitheliomatous epidermal hyperplasia with neutrophilic microabscesses (Fig. 2a). A dermal diffuse and nodular granulomatous mixed infiltrate of lymphocytes, histiocytes, giant cells, numerous eosinophils, and neutrophilic microabscesses was seen in all tissues examined. Septate hyphae were present both within giant cells and free in the dermis (Fig. 2b). The hyphae were branching at a 45 degrees angle and were positive on periodic acid-Schiff and Grocott methenamine silver stains (Fig. 2c). Fungal culture studies of material taken from an ulcerated skin lesion grew Aspergillus flavus. Blood cultures were negative for Aspergillus sp. or other microorganisms. The patient was treated with intravenous amphotericin B, but the medication was discontinued due to her intolerance to the drug. She was subsequently lost to follow-up.
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OBJECTIVE: Incidence of head and neck squamous cell carcinoma seems to be relatively high in Yemen but not well documented. The purpose of this study is to analyze the clinical profile of the Yemeni patients of squamous cell carcinoma of the head and neck and to evaluate the possible relationship to kath chewing. METHODS: With the help of a special protocol, all the patients of head and neck squamous cell carcinoma seen between October 1997 and December 1998 at the Ear, Nose and Throat and Dermatology Clinics of Saudi Hospital, Hajjah, Yemen Republic were subjected to detailed analysis. The diagnosis was confirmed by histopathologic studies in all the cases. RESULTS: All the 36 patients (23 male and 13 female) were Yemani nationals, aged 18 to 80 years (median age 50 years). Thirty patients were Kath addicts. The tumor was localized to the oral cavity in 17 (47%) patients, oropharynx in 1 (3%) patient, nasopharynx in 15 (42%) patients and larynx in 3 (8%) patients. CONCLUSION: The incidence of head and neck squamous cell carcinoma seems to be relatively high, especially the oral squamous cell carcinoma, all of whom had a habit of kath chewing, which may be considered as an important contributing factor.
BACKGROUND: A preliminary study of 24 cases of xeroderma pigmentosum (XP) was presented in 1990 and later published in 1992. Since then we have seen 18 further cases. OBJECTIVE: To study the clinical profile of Libyan cases of XP. METHODS: With the help of a special protocol, all 42 cases (23 girls and 19 boys from 29 families) treated and followed between 1981 and 1994 were subjected to detailed analysis. RESULTS: A history of consanguinity was present in the parents of 39 patients. XP in Libya is characterized by a relatively high incidence (approximately 15-20 per million of the population), early onset of initial manifestations (median age, 12 months) and malignant tumors (median age, 8 years), severe ocular and oral lesions in a high percentage of patients, and early death (median age, 15.5 years). The malignant skin tumors seen were squamous cell carcinoma (SCC) in 23 patients, basal cell carcinoma (BCC) in 17 patients, and basosquamous carcinoma in two patients. Malignant melanoma was not seen, but lentigo maligna was found in one case. SCC of the tongue, carcinoma of the thyroid, and lymphatic leukemia affected individual patients. Subnormal physical growth was observed in six patients, but none of the patients had significant neurologic abnormalities. The results of etretinate therapy in nine patients revealed that it is an effective preventive agent against carcinogenesis, but not a curative one. CONCLUSIONS: Severe ophthalmic manifestations affected a higher percentage of patients at an early age. Malignant melanoma did not develop in any case, except for lentigo maligna in one patient.
BACKGROUND: Cutaneous leishmainiasis is widespread in the Yemen, but has not been fully documented. METHODS: We have studied 42 cases from the Hajjah and Amran Governorates of the Yemen Republic. The clinical profile of these cases was recorded in a special protocol. The diagnosis was based on positive slit skin smear and histopathologic studies when needed. RESULTS: The age of the patients ranged between 1 and 65 years (median age, 19 years) and the duration of the disease ranged between 2 months and 12 years (median duration, 6 months). Most of the patients had a single lesion. Slit skin smear was positive for parasites in 35 cases (highly positive in 15). From the analysis of features, the disease could be classified into dry type (31 patients), wet type (seven patients), and chronic cutaneous leishmaniasis (four patients). Most of the patients required prolonged (4-6 weeks) treatment with sodium stibogluconate; the smear was still positive in eight patients, seven of whom were further treated successfully with a combination of rifampicin and isoniazid for 2-3 months. One who was treated successfully with intralesional injections of sodium stibogluconate. CONCLUSIONS: Cutaneous leishmaniasis in this region seems to be endemic, has a long chronic course, shows a highly positive smear even 1-6 years after onset, and gives an inadequate response to prolonged systemic therapy. Further epidemiologic studies and taxonomic differentiation of the species are required.
BACKGROUND: Despite a high incidence of xeroderma pigmentosum, there is no previous publication from Libya. OBJECTIVE: The purpose was to study the clinical profile of Libyan cases of xeroderma pigmentosum. METHODS: With the help of a special protocol, 24 cases of xeroderma pigmentosum treated between 1981 and 1990 were subjected to detailed analysis. RESULTS: The age of onset of initial manifestations ranged between 6 and 18 months whereas that of malignant lesions ranged from 2 to 10 years. Malignant lesions observed were squamous cell carcinoma in 15 patients, basal cell carcinoma in 12, and basosquamous carcinoma in 2 patients; squamous cell carcinoma of the tongue, carcinoma of the thyroid, and lymphatic leukemia affected individual cases. None of our patients developed malignant melanoma. Six patients have died; the age at death ranged between 9 and 18 years. A history of consanguinity in the parents of patients was recorded in all but two patients. CONCLUSION: We observed early onset of severe ophthalmic lesions affecting a higher percentage of the patients.
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