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1.
Tunis Med ; 88(7): 519-22, 2010 Jul.
Article in English | MEDLINE | ID: mdl-20582892

ABSTRACT

BACKGROUND: Cutaneous aspergillosis is rarely reported in diabetic patients. AIM: The objective of our study is to report a case of lethal disseminated aspergillosis revealed by multiples skin necroses, with pulmonary and sinusal involvement in a diabetic patient. CASE REPORT: A 60-year-old diabetic woman, presented with one month -rapidly -extensive, 1 to 10 cm skin necroses of the trunk, limbs and eyelids. Few days after her admission, she developed dyspnoea. Chest X-ray showed an interstitial and alveolar syndrome with multiple excavated anfractuous-edged-opacities. Facial CT scan showed a right orbital cellulitis with Pansinusitis. The methaminesilver stains on a cutaneous biopsy showed filamentous septate fungal hyphae with branches at right angles. The immunofluorescence with an anti-aspergillus serum was positive. The diagnosis of secondary disseminated aspergillosis to a primary pulmonary focus with cutaneous, sinusal, and upper airway's dissemination was made. The patient died despite an intravenous amphotericin B therapy. CONCLUSION: This report emphasizes the importance of evoking and seeking for a mycosis in every skin necrotic and ulcerative lesions occurring in an immunocompromised patient. The prognosis depends on the diagnosis and treatment institution delay.


Subject(s)
Aspergillosis/diagnosis , Dermatomycoses/diagnosis , Diabetes Complications/diagnosis , Diabetes Complications/microbiology , Pulmonary Aspergillosis/diagnosis , Sinusitis/diagnosis , Sinusitis/microbiology , Fatal Outcome , Female , Humans , Middle Aged
2.
Dermatol Online J ; 16(3): 1, 2010 Mar 15.
Article in English | MEDLINE | ID: mdl-20233558

ABSTRACT

Peeling skin syndrome (PSS) is a rare form of ichthyosis with a probable autosomal recessive inheritance that exhibits superficial, painless, continual, or seasonal cutaneous exfoliation. The syndrome generally appears at birth or in infancy. We report a case of generalized PSS and provide a literature review. A 34-year-old woman reported a lifelong history of generalized and painless peeling of the skin that worsened in summer. Her parents were third degree cousins. Her twin sister and her two cousins presented with the same condition. Physical examination showed widespread superficial sheets of variable size that could be easily removed without bleeding or pain. No underlying erythema was noted. Otherwise, the patient was in good health. Histological findings showed an epidermal cleavage within the stratum corneum. The generalized form of PSS is classified into 3 types, A, B, and C, according to the classification system of Traupe and Mevorah. We have tried to classify the cases of generalized PSS already reported in the literature into one of these three types. Thirteen reported cases probably presented PSS-type A. Sixteen patients are best described as PSS-type B. Two patients exhibit PSS-type C. Fifteen reported patients had an acral form of peeling skin syndrome. The classification of the eleven remaining patients was difficult to determine. Our patient presented clinical and histological features of generalized PSS-type A.


Subject(s)
Ichthyosis/classification , Ichthyosis/pathology , Adult , Consanguinity , Female , Humans , Ichthyosis/genetics , Syndrome , Transglutaminases/genetics
4.
Tunis Med ; 86(11): 983-6, 2008 Nov.
Article in French | MEDLINE | ID: mdl-19213489

ABSTRACT

BACKGROUND: Ichthyosis are a group of inherited keratinizing disorders. The cutaneous abnormalities may be isolated or associated with extra-cutaneous symptoms. AIM: To report the epidemiological and clinical profiles of patients with these genodermatoses from a hospital tunisian study. METHODS: A retrospective study of all cases of ichthyosis referred during a period of 5 years to the department of dermatology of Charles Nicole's hospital of Tunis. RESULTS: Sixty cases of hereditary ichthyosis were seen. The sex-ratio was of 0.5. Parental consanguinity was noted in 36 patients (60%). Seventeen patients (25.7%) had a positive familial history of ichthyosis. The clinical form of ichthyosis was determined in 52 cases. The nonbullous ichthyosiform erythroderma was observed in 25 patients (41.6%). Sixty patients presented an ichthyosis vulgaris (26.6%). The other forms of ichthyosis were rarely observed : 4 cases of X-linked recessive ichthyosis, 2 cases of lamellar ichthyosis and 2 cases of bullous ichthyosiform erythroderma. Two patients were born with collodion-like membranes. Two cases presented a complex syndrome. CONCLUSION: The NBIE, commonly considered as a rare form of ichthyosis, was the most frequently form seen in our study (41.6%), probably because of the high frequency of consanguineous marriages in Tunisia. The IV represents the most frequent form reported in the literature and was observed in 25% of our patients. The classification of some ichthyosis associated with other extracutaneous abnormalities (found in 2 of our patients) remains difficult.


Subject(s)
Consanguinity , Genetic Diseases, Inborn/epidemiology , Ichthyosis/epidemiology , Ichthyosis/genetics , Adolescent , Age of Onset , Child , Child, Preschool , Female , Genes, Recessive , Genetic Diseases, Inborn/genetics , Genetic Diseases, Inborn/pathology , Genetic Predisposition to Disease , Humans , Ichthyosis/pathology , Ichthyosis, Lamellar/epidemiology , Ichthyosis, Lamellar/genetics , Incidence , Male , Pedigree , Retrospective Studies , Tunisia/epidemiology
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