[Severe childhood atopic dermatitis]. / La dermatite atopique severe de I'enfant.

BACKGROUND: Atopic dermatitis (AD) is a chronic relapsing eczematous skin disease. It represents one of the symptoms of atopic diathesis. DA affects usually infants and children. aim : The aim of our study is to draw up the epidemiological, clinical features, treatment and outcome of severe childhood AD through a hospital series. methods: A retrospective study of 24 cases of severe childhood AD hospitalized in the Dermatology Department of La Rabta hospital of Tunis was conducted during a 28 year-period (1981 - 2009). results: The hospital incidence of severe childhood AD was 0,085‰. Patient's mean age at the beginning was 14 months. The sex ratio H/F was 1.66. Cutaneous manifestations occurred preferentially in face (75%). Generalized eczema was observed in 37.5% of cases. Pruritus and xerosis were constant. The mean duration of hospitalization was 11 days. Topical corticosteroids was the most effective method of treating severe DA, associated with antiseptic solutions emollient and antihistaminic drugs. Infectious complications were noted in 50% of cases. Ocular complications were observed in 16.7% of cases. Recurrences were reported in 9 cases. Conclusion :AD is an inflammatory, chronically relapsing, and pruritic skin disorder developing in a xerotic skin. Severe AD in childhood is rare in Tunisia. It requires a good understanding of therapeutic modalities by the patient and his family. It is a cause of important morbidity and it may have a bad impact on quality of life.

Serum lipid level in Tunisian patients with psoriasis.

BACKGROUND: Psoriasis is a common chronic inflammatory skin disorder that has been associated with oxidative stress, abnormal plasma lipid metabolism, and high frequency of cardiovascular events. The aim of this study was to determine lipid profile variations in Tunisian psoriatic patients. METHODS: This study was designed and conducted as a case-control assay with 91 psoriatic patients and 91 controls. The lipid profiles, including serum level of triglycerides, cholesterol, low-density lipoprotein (LDL), and high-density lipoprotein (HDL), were assessed in both groups. RESULTS: The two groups consisted of 91 patients and 91 controls, each with 45 males and 46 females. In the psoriatic group, serum triglycerides, low density lipoprotein, and very low density lipoprotein cholesterol were significantly higher than in the control group (p < 0.05), while the high density lipoprotein cholesterol (HDL-cholesterol) was significantly decreased in patients with psoriasis compared to controls (p < 0.001). There were no significant differences concerning insulin or insulin resistance and total cholesterol between the two groups. The insulin secretion was significantly higher in patients with psoriasis than in the control group (p = 0.003). However, there was no significant correlation between severity of psoriasis and serum lipid and insulin secretion. A negative correlation (r = -0.253, p = 0.019) was found between PASI index and HDL-C. CONCLUSIONS: A high serum lipid level is significantly more common in psoriatic patients. This could be responsible for higher prevalence of cardiovascular incidents in psoriatic patients. It may be useful to do early screening and treatment of hyperlipidaemia in psoriatic patients to prevent atherosclerosis and its complications.

Proliferative fasciitis in the abdominal region.

A 30-year old man with no trauma history presented to our department of dermatology with a 2-year history of abdominal painful masses. The spontaneous pain and tenderness in the abdominal region gradually worsened. Physical examination revealed 3 firm, irregular subcutaneous nodules measuring 1 x 0.5 cm, which were movable and unattached to the overlying skin. One of the nodules was ulcerated (Figure 1). Histopathologic examination showed spindle-shaped fibroblast cells intermingled with gangliocyte-like giant cells in the hypodermis with an infiltrate made of lymphocytes and histiocytes (Figure 2 and Figure 3). The immunohistochemical staining showed the negativity of the fusiform cells and the gangliocyte-like cells to anti-S100 protein and to anti-smooth muscle actin.

Severity of acne and its impact on quality of life.

Acne is a chronic disease that is especially common among adolescents. It can have a considerable psychological and social impact that is not always correlated with clinical severity. The aim of this paper was to evaluate clinical severity and alteration of quality of life in acne patients, and to investigate a possible correlation between the two. A total of 82 patients with juvenile acne were included in this study. The clinical severity of acne was evaluated using the Echelle de Cotation des Lésions d'Acné (ECLA) scale. The Cardiff Acne Disability Index (CADI) was used to assess acne-related quality of life. Acne was mild to moderate in 61% of patients (ECLA < or = 12). A considerable alteration of quality of life was present in 51% of cases. There was a positive correlation between overall scores on the ECLA and CADI scales (P = .012) before and after treatment. Additionally, CADI score improved after effective treatment of acne. Acne may have an important impact on teenagers' psychological and social life. An objective assessment of this impact seems to be necessary. ECLA and CADI scores appear to be objective and simple instruments that may be used in acne management.

[Cutaneous carcinoma induced by radiotherapy: a report of 31 cases]. / Carcinomes cutanés induits par la radiothérapie: a propos de 31 cas.

BACKGROUND: Depilatory radiotherapy was used in the sixties as a treatment for ringworm in Tunisia. Subsequently some of these patients developed radio-induced carcinomas of the scalp. AIM: To present the epidemiological, clinical, pathological,therapeutic features and out come of radio-induced cutaneous carcinomas. METHODS: We conducted a retrospective study performed in the dermatology department of the La Rabta hospital of Tunis over a 6- year-period recording all histologically confirmed carcinomas in patients irradiated in childhood for tinea capitis. RESULTS: Thirty one patients were included with 49 tumors: 47 basal cell carcinomas and 2 squamous cell carcinomas. The average latent period between the irradiation and the appearance of the carcinomas was of 35.7 years. The average age was 53 years. A male predominance was noted, with a sex ratioM/F of 6.75. Clinically, basal cell carcinomas were nodular in all cases. Surgery was indicated in 90% of cases. Cryosurgery and radiotherapy were used respectively in 1 and 2 patients. CONCLUSION: Our study shows that radio-induced cutaneous carcinomas are widely dominated by basal cell carcinoma. They arise, approximately, ten years earlier than carcinoma in patients with no history of scalp irradiation. However X-ray exposure does not seem to influence clinical or histological presentation, therapeutic modalities nor prognosis of these tumors. The prognosis of radioinduced cutaneous carcinomas was globally similar to that of other cutaneous carcinomas with same histological type and equivalent degree of invasion.

[Squamous cell carcinoma of the lip: a report of 30 cases]. / Carcinome épidermoïde des lèvres: A propos de 30 cas.

BACKGROUND: Squamous cell carcinoma (SCC) is one of the most common malignant tumors of the lips (90%). The prognosis of these SCC seems to be poor thus here periorificial localization. AIM: To present the epidemiological, clinical, pathological, therapeutic features and out come of SCC of the lips. METHODS: we conducted a retrospective study performed in the dermatology department of the La Rabta hospital of Tunis over a 11-year-period [2000-2010] recording patients with histologically confirmed SCC of lips. RESULTS: Thirty patients were included (26 men and 4 women) with an average age of 63 years. The most frequent risk factors were smoking and chronic sunlight exposure. The occurrence of the labial SCC on a precursor lesion was noted in 11 cases. It occurred more frequently on the lower lip (80%). Tumor was ulcero-vegetant in 21 patients. Twenty patients had a commune SCC, 19 of them were well-differentiated. Surgery was indicated in 18 cases and 10 patients were treated by exclusive radiotherapy. Lymph nodes metastases were noted in 2 cases. No visceral metastasis was observed. During the period of follow-up (20.12 months), two patients died. CONCLUSION: The diagnosis of SCC of the lips is late and the treatment often mutilating. The improvement of the prognosis depends not only on the early diagnosis and the treatment of the precursors, but also on the photo protection and alcohol and smoking eviction.

Lichen planus pemphigoides: four new cases and a review of the literature.

Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disease. It appears to be combination of lichen planus and bullous pemphigoid. We describe four new cases of LPP and discuss the epidemiological, clinical, pathological, and therapeutic features of this singular association through a review of the 74 published cases within the English literature. We report four cases of LPP (three women aged respectively 47, 51, and 53 years old, and a 53-year-old man). All patients presented with bullae on lichenoid and normal skin, predominately on the extremities. The diagnosis was confirmed by immunohistological findings. Our patients were treated with oral corticosteroids with a good response. Our review of the literature of 78 cases of LPP (65 adults and 13 children) showed that it involved adults (mean age: 54 years), with a slight female preponderance. A mean lag time between LP and the development of LPP was 8.3 months. LPP is characterized by developing blisters on lichenoid lesions and on uninvolved skin with more acral distribution of bullous lesions. Involvement of palms and soles was more frequent in children. The diagnosis is based on pathological and immunological confrontation. LPP is usually idiopathic, but some cases were reported in association with various drugs. There have also been reports of association with internal malignancy. Most cases of LPP are successfully treated with systemic corticosteroids. In most cases, the prognosis was good.

Inflammatory Tinea capitis: a 12-year study and a review of the literature.

Inflammatory Tinea capitis (TC) is a rare form of TC. The aim of this study was to review epidemiological, clinical and mycological profile of inflammatory TC. We present a retrospective study (1999-2010), enrolled all the cases of inflammatory TC observed at a referral hospital in the northern Tunisia. One hundred and twenty-one patients with inflammatory TC, 83 male patients (68.6%) and 38 female patients (31.4%) were enrolled. The mean age was about 8 years. A majority of TC (71.9%) were in patients lesser than 10 years of age. Positive family history and contact with animals were noted in seven and 35 cases respectively. Direct examination was positive in 110 cases (59 ectothrix, 51 endothrix) and positive cultures were obtained in 105 patients (49 Trichophyton violaceum, 31 Microsporum canis, 13 Trichophyton interdigitale complex, 12 Trichophyton verrucosum). Systemic treatment was carried out in 115 patients with griseofulvin, in one with terbinafine. A complete recovery was noted in 88 cases; and persistent alopecia in 28 cases. The inflammatory TC is rare, but more common in rural families. The disease mostly affected male genders (68.6%) and T. violaceum remains the common pathogen of inflammatory TC in northern Tunisia.

A case of pellagra associated with megaduodenum in a young woman.

Pellagra is a rare condition that has been known for many years to be related to niacin deficiency. Clinically known as the 4 "D" symptoms for dermatitis, diarrhea, dementia, and even death, skin changes remain one of the most important features of this pathology, leading frequently to the diagnosis. Pellagra is mostly seen in poor populations with a deficient diet; still, it is sporadically observed in developed countries, usually in association with digestive disorders. We report a new case of pellagra in a 29-year-old woman in whom the diagnosis of megaduodenum was made. Megaduodenum is a rare condition that can be idiopathic or secondary to visceral myopathy or neuropathy. Parenteral supplementation with niacin has resulted in a quick response of dermatological and psychiatric symptoms. To our knowledge, no case of pellagra due to megaduodenum has been reported in literature.

Psoriasis is associated with increased Framingham ten-year risk score for coronary heart disease in Tunisians.

BACKGROUND: To assess the ten-year cardiovascular risk for coronary heart disease (CHD) in psoriatic patients and to test the impact of psoriasis severity and duration on cardiovascular risk. METHODS: A case-control study included 202 adult psoriatic patients and 202 controls. RESULTS: Risk CHD was estimated using the Framingham risk score algorithm. Patients had a higher ten-year Framingham risk score (13.62 +/- 11.86 vs. 9.23 +/- 8.04; p = 0.002) than controls. In addition, a high risk score and a very high risk score (> 40%) were more frequent in psoriatic patients compared with controls (p = 0.043 and p < 0.001, respectively). According to the severity of psoriasis, the ten-year cardiovascular risk increases progressively and significantly (11.84 +/- 10.08; 15.59 +/- 11.79 and 16.92 +/- 14.13 for mild, moderate and severe psoriasis, respectively). CONCLUSIONS: Psoriatic patients have significantly greater risks of developing coronary heart disease than controls in relationship with psoriasis comorbidities such as hypertension, diabetes, dyslipidemia, inflammation and probably with psoriasis itself.

Endogenous ochronosis with a fatal outcome.

BACKGROUND: Endogenous ochronosis (EO) is an autosomal recessive inherited disorder where there is incomplete oxidation of tyrosine and phenylalanine due to a lack of the enzyme homogentisic acid oxidase. OBJECTIVE: We report a singular observation of EO with a fatal outcome. CASE REPORT: We report the case of a 46-year-old man born to consanguineous parents with a medical history of recurrent renal colic and chronic nonspecific arthropathy. On clinical examination, slate blue pigmentation was seen on the cheeks, forehead, and nose, as well as blue-gray patches on all fingernails and bluish discoloration of the gums. Familial investigation revealed that his sister had similar pigmentation on the ears, hands, and fingernails. Histologic examination of a biopsy specimen from a pigmented lesion showed a dermal deposit of an acellular, eosinophilic material without cell reaction. Based on the clinical and histopathologic data, combined with the family medical history, our patient was considered to have EO with mucocutaneous, articular, and renal involvement. Unfortunately, the diagnosis was late because our patient died a few months later of terminal renal failure. CONCLUSION: Skin signs are the hallmarks of EO and must alert the clinician to look for involvement of vital organs.

Letter: Tinea capitis in infants in their first 2 years of life: A 12-year study and a review of the literature.

BACKGROUND: Tinea capitis (TC) is a dermatophyte infection that occurs mainly in childhood; but it is uncommon in infants. The aim of this study was to review the clinical and mycological profile of TC in infants. PATIENTS AND METHODS: We present a retrospective study; we enrolled all the cases of infant TC over a period of 12 years (1999-2010). RESULTS: Thirty-five infants (21 boys, 14 girls) with a mean age of 20.16 months were diagnosed with TC among a total number of 881 cases of TC (3.9%). Scalp scaling and alopecia were the most frequent clinical features. Microsporic tinea (21 cases) was the most frequent followed by Trichophytic tinea (9 cases) and inflammatory tinea (5 cases). Direct microscopy of hair was positive in 33 cases (94.2%). Culture positivity was found in 82.8 percent of infants (29 cases). Four species of dermatophytes were isolated; Microsporum canis in 18 cases (62%) followed by Trichophyton violaceum, Trichophyton mentagrophytes, and Trichophyton verrucosum. Twenty-nine infants were treated successfully with griseofulvin. DISCUSSION: TC is rare in infants. The diagnosis of TC should be considered if scaling and/or alopecia are present and should be confirmed by mycology testing prior to initiation of treatment.

Association analysis of LCE3C-LCE3B deletion in Tunisian psoriatic population.

An association between a common deletion comprising the late cornified envelope LCE3B and LCE3C genes (LCE3C_LCE3B-del) and psoriasis has been reported in Caucasian and Asian populations. To investigate whether this deletion plays a role in the genetic of psoriasis in Tunisian population, we determined the LCE3C_LCE3B-del genotype in 180 Ps patients and 208 healthy controls from different regions of Tunisia. The LCE3B and LCE3C gene variant was determined in the patients through PCR amplification and the SPSS software package. The frequency of the LCE3C_LCE3B-del was similar between patients and healthy controls. Subanalyses by family history revealed that the frequency of LCE3C_LCE3B-del was significantly higher in patients with a positive family history than in control individuals, as well as in individuals with a positive family history versus those without in the case cohort. However, no significant difference was observed between psoriatic patients with no family history and controls. We also evaluated the relationship between LCE3C_LCE3B-del and PSORS1. No significant epistatic effect was observed suggesting that there was no significant epistasis of the two loci in the Tunisian population. Our findings indicate that the LCE3C_LCE3B-del might play a role in familial psoriasis in the Tunisian population.

Retronychia: a rare cause of chronic paronychia.

Retronychia, described in 1999, is a rare entity of ingrown toenails. Embedding of the nail into the proximal nail fold (PNF) leads to chronic inflammatory changes. Herein, we report a new case that exhibited persistent paronychia in a 23-year-old woman. Retronychia usually does not recur once treated with avulsion. It should be suspected in the event of chronic proximal paronychia.

[Immunogenetics of psoriasis: update]. / Immunogénétique du psoriasis : actualités.

BACKGROUND: Psoriasis is a chronic inflammatory skin disease often benign, affecting 2-3% of the total world population. Psoriasis is a multifactorial disease. AIM: To present recent advances in the immunologic mechanisms and susceptibility genes involved in the pathogenesis of psoriasis. METHODS: We presented a literature review of recent genetic and immunological basis of psoriasis to better understand the pathomecanisms of this disease and discuss the contribution of the Tunisian work in this area. RESULTS: Recent works focalized mainly in immunology and genetics. Current progresses in molecular biology have allowed to better characterize the immunogenetic abnormalities in psoriasis. CONCLUSION: Psoriasis is a multifactorial disease model in which environmental factors (psychological, climate, traumatic, infectious, and viral) seem to be triggering factors when associated with a particular immunogenetics predisposition.

Dermatologic manifestations in inflammatory bowel disease in Tunisia.

BACKGROUND: Cutaneous manifestations are the most common extra intestinal manifestations associated with inflammatory bowel disease (IBD). AIM: To assess the epidemio-clinical profile of skin manifestations in IBD. METHODS: A prospective and descriptive study was conducted. We have examined skin, mucosa, hair and nails, of all patients with an IBD during one year. RESULTS: One hundred-ninety-five patients were included. Crohn's disease (CD) was noted in 154 cases (79.8%), ulcerous rectocolitis (UC) in 39 cases (21.2%) and inclassable IBD in 2 cases. Cutaneous manifestations were found in 91% of Crohn's patients and in 92% of UC patients. Granulomatous perianal skin lesions were the main cutaneous manifestations of CD (53%). The most common affected sites were ano-perineal fistulae, perianal and perineal fissures and oedematous and infiltrated perianal and genital plaques. Reactive lesions (Erythema nodosum, Pyoderma gangrenosum, Aphthous stomatitis) were noted in 14 cases. Skin manifestations due to malabsorption were also frequently observed (101 cases: 51.7%).Other dermatoses implicating various mechanisms such as psoriasis, alopecia areata, vitiligo, rosacea, lichen planus, were also noted. Adverse skin manifestations due to treatment (folliculitis, acne, macula-papular rash and DRESS syndrome) were present in 16 cases. CONCLUSION: Our series is characterized by a high frequency of cutaneous manifestations associated to IBD. A better recognition of these skin manifestations by the physician may improve their management.

[Kaposi's sarcoma: epidemiological, clinical, anatomopathological and therapeutic features in 75 patients]. / La maladie de Kaposi: Caractéristiques épidémio-cliniques, anatomopathologiques et thérapeutiques à propos de 75 cas.

BACKGROUND: Kaposi's sarcoma (KS) is a mutifocal angiogenic process characterized by cellular and vascular proliferation. AIM: To identify the epidemio-clinical, histological and therapeutic features of KS. METHODS: Retrospective study of 75 cases of KS at the dermatology department of Rabta hospital in Tunis during a period of 25 years (1982-2007). RESULTS: Three epidemio-clinical forms of KS were observed in our study: the classic KS (70 cases), the AIDS-related KS (4 cases) and the iatrogenic KS (1 case). The mean age of our patients at diagnosis was 69.16 years with a sex ratio of 2.33. Elective site of cutaneous lesions in the classic KS was the limbs (87.1%). The cephalic part was concerned in 17.1% of cases. Mucosal involvement was found in 28.5% of patients. Extra dermatological localizations of KS were observed in 32.9%. CONCLUSION: Our study identified some epidemio-clinical features of the classic KS especially the high frequency of mucosal and cephalic involvement as well as extra dermatological localizations. Thus further exploration is required even without alarm signs.

Chronic lymphocytic leukemia revealed by a branulomatous zosteriform eruption.

A 70-year-old woman presented with an atypical erythematopapular zosteriform eruption of 3 weeks' duration. The patient had no history of previous vesicular eruption. She developed a painful burning sensation on the neck. Clinical examination revealed a cluster of small erythematous firm papules and plaques in a zosteriform distribution on the left ear, face, neck, and shoulder (Figure 1A). The lesions were unilateral and did not cross the midline. Multiple cervical and axillary lymph nodes were palpable. Laboratory tests revealed an increase in white blood cells of 25,000/mm3, with 17,910/mm3 lymphocytes and a normal range of hemoglobin, platelets, creatinine, and liver enzymes. Erythrocyte sedimentation rate was 87 mm. Blood smear results showed small, morphologically mature lymphocyte cells. In immune phenotyping, lymphocyte cells co-express CD5 and B-cell-surface antigens CD19 and CD23, as well as a restriction of kappa immunoglobulin light chains. The cells were CD22-, CD79b-, CD38-, CD10-, CD25- and FMC7-. Computed thoracoabominal tomography revealed cervical, mediastinal, abdominal, and pelvic adenopathy confirming the diagnosis of B-cell chronic lymphocytic leukemia (B-CLL) stage B. Histology of a skin biopsy from a papule showed a dense nodular granulomatous infiltrate in the dermis (Figure 2A). The infiltrate contained epithelioid and giant cells surrounded by lymphocytes and plasma cells. Small monomorphic lymphocytes without mitotic figures predominated (Figure 2B). The epidermis was irregularly thickened. Immunohistology revealed a polymorphous infiltrate with a phenotype of reactive T lymphocytes (CD3, CD5 positive) (Figure 2C), B lymphocytes (CD20 positive) (Figure 2D). Epithelioid and giant cells were positive for CD68 (Figure 2E). A latent herpes zoster infection with granulomatous reaction at the site ofzoster lesions was highly suspected as the patient reported a unilateral burning sensation without a history of vesicular zosteriform eruption. She received treatment with intravenous acyclovir 10 mg/kg every 8 hours. The papular lesions resolved markedly (60%) on macular plaques at the end of the treatment. Following topical treatment with corticosteroids, the lesions healed completely within 4 weeks (Figure 1B). Concerning leukemia, our patient was monitored without therapy by the hematologist.