Matrix metalloproteinase-7 could be a predictor for acute inflammation in psoriatic patients.

PURPOSE: The pathogenesis of psoriasis is characterized by a disruption of extracellular matrix (ECM) in which matrix metalloproteinases (MMPs) participate actively. We aimed to determine MMP-7 level and its association with the inflammatory response in order to determine its usefulness as a biomarker for psoriasis prediction. We also aimed to determine its distribution in uninvolved and involved psoriatic skin to evaluate the probable role of MMP-7 in psoriasis pathogenesis. MATERIALS AND METHODS: We recruited 108 psoriatic patients and 133 healthy controls. MMP-7, tissue inhibitors of metalloproteinases (TIMPs) and interleukin-6 (IL-6) levels were measured by Enzyme-Linked Immunosorbent Assay (ELISA) assay. MMP-7 expression was detected by Immunohistochemistry (IHC) study. RESULTS: ECM turnover and inflammatory biomarker levels were significantly higher in psoriatic patients. MMP-7 revealed to be independently associated to psoriasis even after adjustment for different models. The area under the curve (AUC) of MMP-7 and inflammation Z-score were similar. MMP-7 was positively correlated with IL-6 and inflammation Z-score. Psoriasis severity (PASI) was correlated significantly with IL-6 (p = 0.007). The MMP-7 expression was detected in the epidermis of involved and uninvolved psoriatic skin. In involved skin, MMP-7 was expressed by basal and mostly suprabasal keratinocytes. In uninvolved skin, expression of MMP-7 was restricted to basal keratinocytes. CONCLUSION: MMP-7 is independently associated to psoriasis disease and to inflammatory response which make it a potential biomarker for this dermatosis.

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.

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.

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.

Dermatomyositis and breast cancer: a multicenter Tunisian retrospective study of 13 cases.

AIM: To evaluate the epidemiological, clinical, biological features and prognostic factors in patients presenting an association of dermatomyositis (DM) and breast cancer (BC). METHODS: Medical records of 13 patients with DM and BC among 210 DM collected from January 1982 to march 2009 were retrospectively reviewed. RESULTS: Mean age was 47 ± 18 years. DM preceded BC in 3 patients, was concurrent with BC in 2 cases and followed it in 8 cases. Clinically, ulcerative and bullous lesions predominating on limbs were observed in 2 patients. A severe proximal muscular weakness was observed in 4 cases and one patient has presented an amyopathic dermatomyositis. BC was staged IV in 3 pts, IIIA in 2 cases while 3 had stage IIB, 3 stage IIA and 2 stage I according to TNM classification. Nine out of 13 patients had parallel improvement of DM symptoms after treatment of BC. Five patients died of recurrence or distant metastasis (mortality 41.66%). Median survival was 35 months (3-177) after DM diagnostic. CONCLUSION: In Tunisia, DM is associated with an increased incidence of BC. A paraneoplastic course of DM is noted in 70% of patients. In view of the increased risk of BC in our country, in addition to routine examination and laboratory screening, mammography, chest ultrasound, and gynaecological examination, are indicated in women with DM older than 40 years, particularly in case of previous personal or familial history of breast neoplasm.

[Erythrodermic psoriasis: epidemiological clinical and therapeutic features about 60 cases]. / Erythrodermie psoriasique: profil épidémio-clinique et thérapeutique à propos de 60 cas.

BACKGROUND: the erythrodermic psoriasis (EP) is a rare but severe form of psoriasis that may be potentially life-threatening. AIM: To study the characteristics of this severe form of psoriasis. METHODS: We present a retrospective study, including all cases of EP followed in the dermatology department of the La Rabta hospital of Tunis over a 31-year-period from January, 1980 to June, 2010. RESULTS: sixty patients were included, concerning 46 men and 14 women, with an average age of 53.7. A history of psoriasis was reported in 78 % of the cases. A triggering factor was found in 53 % of the cases. Systemic treatments were required in 55 % of cases. An improvement was noted in 69.4 % of the cases. A recurrence of the EP was observed in 15% of the cases. Three cases of sepicemia and one of stroke were noted. CONCLUSION: Erythrodermic psoriasis is the most common etiology of erythroderma. It represents more than half of severe psoriasis. As shown in our study it affects mainly adults' males. It complicates usually a common psoriasis. Septic and thromboembolic complications ones justify a close follow up.

[Acute generalized exanthematous pustulosis. Study of 22 cases]. / La pustulose exanthématique aiguë généralisée: etude de 22 cas.

BACKGROUND: Acute generalized exanthematous pustulosis (AGEP) is an uncommon but severe dermatosis, characterized by acute occurrence of fever, and erythemato-oedematous rash, covered by sterile nonfollicular pustules. Most cases of AGEP have been described in association with the intake of drugs. AIM: To determine clinical and prognostic features of AGEP in our patients. METHODS: All cases of AGEP diagnosed between 1992 and 2007 according to EuroSCAR criteria have been collected. RESULTS: Twenty two patients (16 female, 6 male) with a mean age of 40.9 years (19-81) were included in the study. Clinical features showed in all cases an acute eruption with oedematous erythema, rapidly covered by nonfollicular pustules. The rash was mainly localized on big folds, trunk and/or limbs in 14 cases and generalized in 8 cases. A biological cytolysis was noted in 5 cases and a functional acute renal failure was objected in 2 cases. Etiological work up has found an association with the intake of drugs in 14 cases, a toxic cause (mercury) in 1 case and a B19 parvovirus infection in one case. The mean delay between drug intake and beginning of the eruption was 5 days (24 hours- 15 days). Pharmacovigilance enquiry has concluded to a probable or plausible causality in all our cases. Clinical features improved with drug or toxic withdrawal with a mean delay of 7 days (4 -12 days). A relapse of AGEP was observed in 2 cases after accidental introduction of the drug. CONCLUSION: AGEP is a cutaneous side effect not to ignore, because of its severe prognosis in case of systemic involvement and the possibility of relapses in case of retake of the causal drug.

Childhood linear IgA bullous dermatosis in Tunisia.

The objective was to determine the demographic characteristics, the clinical features, the immuno-histological findings and response to treatment of childhood linear IgA bullous dermatosis (LABD) in Tunisia. We collected all the cases of auto-immune bullous diseases of childhood, diagnosed from January 1987 to December 2006. Based on clinical, histological, and immunofluorescent features, we identified 25 cases of LABD. Sixteen male and nine female children with a mean age of 7.5 years were identified. Clinical manifestations were characterized by a vesiculo-bullous eruption in all cases associated with mucous membrane involvement in two cases. Dapsone was the main therapy in 19 cases, associated with systemic corticosteroids in eight cases. Exclusive antibiotic therapy was successful in five cases. Sixteen of those patients had resolution of disease after a mean period of 15 months and eight patients had severe clinical presentation and required a prolonged follow-up. Childhood LABD is the most frequent bullous dermatosis in Tunisia. The majority of our patients responded rapidly to dapsone treatment and were stabilized for long time. Our cases were characterized by a minimal mucosal involvement and favorable outcome. Treatment with antibiotherapy was interesting. Erythromycin and oxacillin may be considered as an alternative therapy.

Hidradenitis suppurativa: a disease with male predominance in Tunisia.

BACKGROUND: hidradenitis suppurativa (HS) is a chronic inflammatory, suppurating, fistulizing, and scar-producing disease of apocrine gland-bearing skin. The diagnosis is primarily clinical, based on the presence of both sinus tracts and abscesses with a characteristic distribution. OBJECTIVE: Review of epidemiological, clinical, and prognostic characteristics of HS and discussion of the etiopathogenic aspects of this chronic problem. PATIENTS AND METHODS: We retrospectively report all cases of HS followed at the Department of Dermatology between January 1985 and December 2008. RESULTS: Eleven patients (10 male and 1 female), with a mean age of 35.2 years (range 21 dash, vertical53 years) at HS diagnosis were followed for HS. The average age of disease onset was 23.9 years. The median delay between onset of symptoms and diagnosis was 144 months (range 1 dash, vertical408 months). Clinical features showed inflamed discharging papules or nodules, painful tender erythematous nodules, and double-ended comedones. The disease mainly affected the axillary, anal, perineal, and genital areas. Histologically, dermal features showed active folliculitis or abscess, sinus tract formation, fibrosis, and granuloma formation. Pathological associations (Darier's disease and Down syndrome) were noted in two patients. Treatment consisted of antibiotics in eight patients, retinoids (1 mg/kg/ day) in three patients, and surgery in three patients. The mean follow-up was 13 months (range 2 dash, vertical30 months). Recurrence of lesions was observed in all patients approximately 1 month after treatment withdrawal. The Down syndrome patient developed vaginal hydrocele of the testis as a complication of his staphylococcic ulcers. In all cases healing occurred with substantial scarring. DISCUSSION: An obvious male predominance was noted in our patients as well as a delay in the diagnosis of HS, which could be explained by non-recognition of the disease by non-specialists that see the patients at the primary-care level.

Methotrexate-induced papular eruption following treatment of psoriasis.

OBJECTIVE: To report a case of a diffuse papular eruption following treatment of psoriasis with methotrexate injections. CASE REPORT: A 52-year-old woman presented with an extensive flare of psoriasis associated with joint pain, especially in her knees and elbows. She was treated with intramuscular injections of methotrexate 20 mg/wk. Ten hours after the second methotrexate injection, the patient experienced a diffuse pruritic papular eruption located mainly on the limbs. Histology showed foci of dyskeratosis in the mucosal layer and a polymorphic perivascular inflammatory infiltrate of the papillary dermis, suggesting a drug-induced skin reaction. According to the Naranjo probability scale, the papular eruption was probably caused by methotrexate. The drug was discontinued and papular lesions gradually disappeared. DISCUSSION: Methotrexate-induced papular eruption is rarely reported shortly after beginning methotrexate therapy in patients with acute exacerbation of collagen vascular diseases. Methotrexate-induced papular eruption following treatment of psoriasis has not been previously reported. CONCLUSIONS: The pathogenesis of methotrexate-induced papular eruption in collagen vascular diseases may suggest cutaneous small-vessel vasculitis. In our patient, histology showed aspects of drug-induced skin reaction without vasculitis. Pathogenesis of methotrexate-induced papular eruption in psoriasis may involve immune mechanisms other than those of methotrexate-induced cutaneous vasculitis in collagen vascular disease.

Post traumatic amelanotic subungual melanoma.

Subungual melanomas are rare; a delay in the diagnosis is common and is associated with advanced stage. Part of the reason for a delay in presentation to the physician is that patients often attribute the lesion to trauma. Trauma may play a role in the pathogenesis or just draw attention to a skin tumor that may be more susceptible to injury. We report a case of subungual melanoma that was observed in an 86 year old man. The preceding trauma history and misleading clinical appearance delayed the diagnosis slightly. Biopsy of every nodular acral tumor is very important. A direct role of the trauma in the pathogenesis of melanoma remains unclear.

A retrospective analysis of thalidomide therapy in non-HIV-related Kaposi's sarcoma.

BACKGROUND: Thalidomide has antiangiogenic and immunomodulatory properties and has recently been used in the management of human malignancies. Several studies have suggested its interest for treating AIDS-related Kaposi's sarcoma. OBJECTIVES: This study aimed to assess the efficacy and toxicity of thalidomide, an antiangiogenic agent, for the treatment of non-HIV-related Kaposi's sarcoma. METHODS: Eleven patients were included in this retrospective study conducted in the Department of Dermatology of Saint Louis, Paris, between 2000 and 2003. Among them, 2 were immunosuppressed (1 renal transplant recipient and 1 patient with microscopic polyangiitis treated by oral steroids) with stable immunosuppressive regimens during the past 6 months. The median daily thalidomide dosage was 100 mg and the median duration of drug treatment was 16 weeks. RESULTS: Three patients achieved a partial response and 4 had a stable disease. Although no grade 3 or 4 was observed, 3 (27%) out of 11 patients prematurely discontinued thalidomide because of grade 1 sensory neuropathy (paresthesia) and vertigo. CONCLUSIONS: Our results show a true although modest interest of thalidomide in non-HIV-related Kaposi's sarcoma and prompt us to evaluate less toxic thalidomide analogues for this indication.

5% topical imiquimod tolerance in transplant recipients.

BACKGROUND: Topical imiquimod is a new immunomodulator agent approved for genital warts, actinic keratosis (AK) or carcinoma, occurring in immunocompetent patients. OBJECTIVES: This study aimed to assess the safety and efficacy of imiquimod for the treatment of warts, AK and bowenoid papulosis (BP) in transplant patients. METHODS: 24 transplant patients (18 kidney, 4 kidney-pancreas and 2 heart) were included in this retrospective study conducted between June 2000 and February 2003 at the department of dermatology of 3 hospitals. Imiquimod cream was applied 3 times a week over a median period of 9 weeks. RESULTS: Graft function was not altered under therapy. Local tolerance was excellent. Complete responses were observed in 1 patient (1/12) with cutaneous warts and 1 (1/6) with AK. Two patients of 3 with BP had total clearance of their lesions. Partial responses were observed in 3 of the 6 AK-treated patients, 5 of 12 patients with cutaneous warts and 1 of 2 patients with anogenital warts. CONCLUSIONS: Imiquimod 5% cream is a promising, well-tolerated therapy for warts, AK and BP in transplant recipients.

Undifferentiated sebaceous carcinoma: an unusual childhood cancer.

Sebaceous carcinoma is an aggressive, adnexal, rare malignant tumor that may arise in ocular or extra-ocular sites. Extraorbital sebaceous carcinoma is exceptional in childhood. We report a 12-year-old boy with an ocular sebaceous carcinoma who was first seen with an asymptomatic firm, cutaneous nodule on the right eyebrow. The tumor developed slowly within 1 year. Histologically, it was an undifferentiated sebaceous carcinoma. The patient had surgery with wide surgical margins. He was alive and free from disease at a follow-up of 34 months. Close follow-up of this tumor is recommended because of the risk of aggressive behavior.

[Lyell's syndrome: a report of 16 cases]. / Le syndrome de Lyell: a propos de 16 observations.

BACKGROUND: Lyell's syndrome (SL) or toxic epidermal necrolysis is a rare mucocutaneous eruption, which is characterised by an acute necrosis of the totality of the epidermis +/- the mucosal epithelium. This is a serious affection considering the severity of systemic manifestations, the unpredictable evolution, and the absence of specific therapy. AIM: To assess epidemiological and clinical features of this condition in our departments. METHODS: This was a retrospective study concerning the cases of Lyell's syndrome carried in the dermatology and the intensive care department of Farhat Hached hospital over a 26 year period. RESULTS: We listed 12 women and 4 with a mean age of 48.9 years. Epidermal detachment varied between 26 and 80% of the body surface and mucus were involved in 87.5% of cases. Systemic manifestations were noted in 11 patients. A drug etiology was found in 87,5% of cases, half of which was due to antibiotics. CONCLUSION: Our results are similar to those in the literature. Our study illustrates the severity of this toxiderma with a high mortality rate (43.75% of cases), conformable with what was predicted by the severity-of-illness score "SCORTEN". Infectious complications were the principal cause of death.

[Norwegian scabies, a rare diagnosis in Tunisia]. / La gale norvegienne, parasitose rare en Tunisie.

UNLABELLED: Norwegian scabies is a particular form of scabies, rarely reported in Tunisia. It habitually occurs in immunocompromised hosts, with prolonged immunosuppressive therapy, congenital immune disease, HIV-1 infection or neoplasm. We report an observation of norwegian scabies, occuring in a woman with prolounged systemic corticosteroid therapy. OBSERVATION: A 65-year-old woman, with a long history of obstructive bronchoneumopathy and bronchiectasis, treated with systemic corticosteroids for 5 years, was referred with a mild pruriginous and scaly erythroderma. This dermatosis developed within 5 months, with fever and impairing of health. Skin parasitological exams showed a massive infestation by sarcoptes. Histologic findings revealed multiple sarcoptes scabiei and eggs in the stratum corneum. The patient was treated with benzyl benzoate during 48 hours. She died rapidly with acute respiratory distress. DISCUSSION: basing on this observation of norwegian scabies occuring in an old immunocompromised woman, we put the accent on this particular form of scabies, often misappreciated, and incite to practice skin and nail parasitological exams in case of mild pruriginous, chronic scaly erythroderma. developing in immunocompromised patients.

ESHAP for primary cutaneous T-cell lymphomas: efficacy and tolerance in 11 patients.

Systemic multiagent hemotherapy has been used to treat aggressive forms of primary cutaneous T-cell lymphomas (CTCL) with controversial results. Our objective was to retrospectively assess efficacy and toxicity of ESHAP (etoposide, cisplatin, high-dose aracytine, methylprednisolone) in patients with advanced CTCL. A total of 11 patients with aggressive primary CTCL, treated with the ESHAP protocol between 1995 and 2002, were studied. Two patients achieved complete remissions lasting 30+ and 6+ months, seven had partial remissions of short duration, one had stable disease and one experienced disease progression. ESHAP was poorly tolerated because of prolonged myelosuppression (91%) and infectious complications (82%). Our results suggest that ESHAP has a poor risk/benefit ratio in advanced CTCL because of the low number of complete remissions, the short duration of partial remissions and its high-grade toxicity.