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We report a new case of postvaccination morphea profunda (MP) in a child and discuss its different clinical presentations, prognosis, and therapy and its relationship with "solitary morphea profunda." A 2-year-old healthy girl presented with an induration of the anterior aspect of the left thigh of 9 months duration. The lesion had appeared 3 months after a third dose of diphtheria-tetanus-pertussis vaccine. Cutaneous examination showed an induration of 7 × 7 cm with an "orange peel" texture after pinching the skin. Histologic examination confirmed the diagnosis of MP. Systemic steroids (1 mg/kg/day) led to the stabilization of the lesion. After 4 months of treatment, we began the concomitant use of oral methotrexate (10 mg/wk) for 2 months. Methotrexate was then continued alone for 10 months, leading to a significant regression of the induration with no relapse., (© 2011 Wiley Periodicals, Inc.)

Background: Cutaneous adverse drug reactions (CADR) are frequent in children. They have different clinical presentations and may be caused by several drugs., Aim: To evaluate the epidemioclinical features of cutaneous adverse drug reactions (CADR) and the different causative drugs in a Tunisian paediatric series., Methods: We have retrospectively included 90 children (under 16 years old) with a well documented cutaneous drug reaction, seen in the Department of Dermatology of Charles Nicolle hospital of Tunis over 18 years (1991-2008). Age, gender, duration of skin disorders, type of cutaneous lesions, incriminated drugs, delay between drug consumption and eruption, validation by the national pharmacovigilance centre, treatment and outcome were recorded., Results: Our patients were 6.9 year-aged (sex-ratio M/F 1.19). They had maculopapular eruption (MPE) (57.7%), acute urticaria (16.6%), fixed drug eruption (14.4%), erythema multiform (2.2%), photosensitization (1.1%) or severe cutaneous drug reactions (10%).Incriminated drugs were: Antibiotics (55.5%), non-steroidal antiinflammatory drugs (18.8%), antiepileptics (11.1%), and analgesics (5.5%). Betalactamins were the most commonly incriminated antibiotics (32 out of 50 patients; 64%). Barbiturates were the most commonly incriminated anti-epileptics (7/90 cases, 7.7%). Favourable outcome was noted in all patients, even those with severe drug reactions., Conclusion: MPE to antibiotics were the most common kinds of CADR in children. Drug responsibility should be based on solid criteria given the frequency of MPE of infectious origin and the frequent prescription of antibiotics in paediatric population.

Background: The bullous variant of Darier's disease (DD) is a rare type characterized by histological and clinical overlap with Hailey-Hailey disease (HDD)., Case Reports: The following case report describes two cases of familial DD; a 51-year-old woman who presented with erythematous plaques, covered by small blisters in axillary and inguinal areas, and the first patient's daughter, who presented with keratotic papules localized on the axillary and inframammary folds., Conclusion: These two cases are original by the predominant flexural distribution, and by a bullous form in the first case, clinically and histologically mimicking HHD.

Purpose: To remind special attention to atypical symptoms of Hansen's disease, we report a case of an atypical case due to a delayed diagnosis., Background: Clinical features of leprosy are well known, cutaneous lesions and involvement of the peripheral nerves being the cardinal clinical signs. Among these presentations, systemic involvement, including mucous membranes of the upper respiratory tract and eyes, is rarely reported even if it is still commonly seen in endemic areas, in particular lepromatous leprosy., Case Report: We describe here a new case of Hansen's disease in a 51-year-old Tunisian woman with an atypical presentation and a delayed diagnosis. The early symptoms of the disease were different from the main clinical signs of Hansen's disease since they involved the upper respiratory tract and the eyes. A nasal smear was positive for acid-fast bacilli, thus confirming the diagnosis of bacilliferous leprosy. Histological findings suggested the diagnosis of leprosy and were somewhat more characteristic of the borderline lepromatous type., Conclusion: Diagnosis of Hansen's disease in patients with neither apparent skin lesions nor neurological signs is still problematic. Clinicians should not only pay attention to the more obvious signs in their own fields of expertise but should be aware of the possible systemic involvement of leprosy., (© 2011 The International Society of Dermatology.)

Objective: We present a case of acrodermatitis enteropathica in a full-term, breast-fed, 7-mo-old infant born from consanguineous parents with a family history of acrodermatitis enteropathica., Methods: The patient presented with periorificial and symmetric acral lesions, which prompted us to review the clinical features of acrodermatitis enteropathica and its pathogenesis. Laboratory investigations showed low zinc levels in the infant's and mother's sera and in the mothers' milk., Results: A diagnosis of acrodermatitis enteropathica was made. A mutation screening of the SLC39A4 gene in the patient and his mother showed heterozygosity for the deletion c.1223_1227delCCGGG. The diagnosis of transient symptomatic zinc deficiency was then established., Conclusion: Transient symptomatic zinc deficiency is generally reported in premature infants but should also be considered in full-term, breast-fed infants, as in the present case., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: Disseminated superficial actinic porokeratosis (DSAP) is the most common form of porokeratosis. A case of late-onset DSAP is reported with an uncommon presentation in an elderly patient., Methods: An 80-year-old woman, with diabetes mellitus treated with metformin, and hypertension treated with metoprolol and captopril, presented with multiple 2 mm-to-7 cm brown patches, for 5 years. The patches were often confluent with an atrophic center and a well-demarcated keratotic border located on the thighs, lower legs, and feet., Results: Histological examination of a cutaneous biopsy showed the presence of rare cornoid lamellae, confirming the clinical diagnosis of DSAP., Conclusion: In this report, the atypical presentation of DSAP is discussed in this elderly patient and also the possible triggering factors at this age.

Background: Chronic actinic dermatitis (CAD) is a debilitating photodermatosis with characteristic clinical, histological and photobiological features (reduced minimal erythema dose: MED). Its management involves various therapeutic approaches, among them there is phototherapy. Efficacy of psoralen ultraviolet therapy (PUVA therapy) was previously demonstrated but there are no current data on the use of narrowband ultra violet B (UVB) therapy (NB-UVB) in CAD. NB-UVB has already been proven to be effective and safe in several other photodermatoses., Case Reports: We report here two dark-skinned patients (skin type IV and V) with CAD, successfully treated with an incremental regimen of NB-UVB phototherapy coupled to a 3 month-course of systemic steroids (1mg/Kg/day)., Conclusion: Our protocol of NB-UVB with steroids seems to be effective for the management of CAD with a good short term safety profile., (© 2011 Société Française de Pharmacologie et de Thérapeutique.)

Introduction: Kindler's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia. We report three new sibling cases of this rare syndrome., Cases Reports: The condition was seen in three sisters aged 12, 16 and 20 years, born of a first-degree consanguineous marriage with no family history of Kindler's syndrome. The three patients presented spontaneously regressive bullous eruptions, poikilodermia of gradual onset, major cutaneous atrophy on the back of the hands and the feet, photosensitivity and gingival hypertrophy. Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage., Discussion: Diagnosis of Kindler's syndrome is based upon clinical evidence. Kidler's syndrome is a well defined clinical entity. Ultra-structural studies show intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and other bullous hereditary poikilodermas., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Erythema elevatum diutinum (EED) is a rare condition with an unclear pathogenesis. Initially classified within neutrophilic dermatoses, it is now considered as a leukocytoclastic vasculitis accordingly to its histopathologic pattern. Several clinical presentations as well as many associated diseases are reported in the literature. We report a new case of EED in a 58-year-old man who presented with a three-month history of plaques and nodules on the extensor surfaces of hands, elbows, knees, ankles, forearms, and buttocks. Histology showed a leucocytoclastic vasculitis, suggestive of the diagnosis of EED. Screening for an associated pathology, namely a paraproteinemia or a solid cancer, was negative. Treatment with dapsone leads to amelioration within few weeks.

Background: Trichoblastoma is a rare and benign adnexial tumor with characteristic histological features. It occurs on any hair folliclebearing location, and usually presents as a solitary lesion most often less than 2 cm in size. Giant trichoblastoma has been rarely reported in the literature., Aim: To report a new case of giant trichoblastoma, misleading for malignancy., Case Report: A 57-year-old woman presented with a 5 cm-solitary asymptomatic nodular lesion of the scalp, of 28 years. It had been previously excised with recurrence and progressive regrowth. On examination, it was a dome-shaped, erythematous, firm, papillomatous, non infiltrated nodule. Full body work up revealed no metastases. Cutaneous biopsy concluded to trichoblastoma but failed to eliminate malignancy. After excision with secondary skin graft, histological examination confirmed the benignity with clear margins. There was no evidence of recurrence after a 5 year-follow-up period., Conclusion: This case illustrates a rare clinical variant of trichoblastoma with an unusual important size. This can be misleading for malignancy, but the slowly progressive course of the tumour in our patient, together with histological benignity led to the correct diagnosis. This tumour is considered as a distinct entity by some authors.

Background: Malignant proliferating trichilemmal cyst is a rare tumour usually located on the scalp of elderly women. About 40 cases of malignant proliferating trichilemmal tumour have been documented., Case Report: We report a case of a malignant proliferating trichilemmal cyst of the scalp in a 57-year-old woman. On the vertex she had a voluminous vegetated and multinodular tumour measuring 7 x 5 cm with spontaneous and abundant bleeding, and another lesion of the scalp corresponding to a trichilemmal cyst. Based on histopathological findings, the case was diagnosed as malignant proliferating trichilemmal cyst., Conclusion: Diagnostic, clinicoprognostic and histological features of this tumour are discussed. Treatment is not yet standardized given its rarity.

Background: Hydroxyurea (HU) is an antineoplastic drug commonly used to treat chronic myeloproliferative disorders. Dermatological side effects are frequent and usually benign. Leg ulceration following HU therapy is less common., Aim: To describe epidemioclinical and therapeutic features of leg ulcers induced by HU., Case Report: A 70-year-old woman is treated with hydroxyurea for polycythemia vera. One year later; she presented with a malleolar painful ulcer, initially healed without discontinuation of the treatment, but has been recurred 2 months later, becoming multiple and bilateral. HU has been discontinued and ulcers were completely cured., Conclusion: Leg ulcers induced by hydroxyurea are rare. Pathogenesis of HU-induced ulcers remains unknown and is multi factorial. Discontinuation of treatment is still the option of choice for complete recovery.

Background: Endogenous ochronosis (EO) is a rare autosomal recessive disorder due to accumulation of oxidized and polymerized forms of homogentisic acid (HGA) in connective tissues, giving them a deep dark blue pigmentation., Aim: Through a new Tunisian case of EO and a review of the literature, we aimed to define the epidemioclinical features of EO, its diagnostic criteria, and evolution., Methods: Three hundred and forty patients were enrolled through 54 articles and four abstracts., Case Report: A 35-year-old woman, born in consanguineous parents, presented with blue-grey patches of fingernails, first interdigital spaces, and ears with brown conjunctival pigmentation. Urine specimen turned dark on standing overnight. The diagnosis of EO was confirmed by urinary high levels of HGA. Investigations revealed radiologic signs of ochronotic arthropathy., Review of the Literature: EO is ubiquitary. Its prevalence was estimated at almost 6.5 cases/year. The mean age at diagnosis was 55.9 years (M/F: 1.85). Onset symptoms mainly consisted in cutaneous signs. Ochronotic arthropathy was the most frequently reported manifestation. Treatment was mainly symptomatic., Discussion: EO is often revealed in adulthood mainly after the fourth decade. Urinary darkening is the first sign of the disease but is rarely reported as an onset sign. Skin signs are the alerting features. Ochronotic arthropathy is insidious but may be debilitating. No specific medical treatment of EO is available., Conclusion: Cutaneous manifestations are the hallmarks of OE. As vital organ involvement has been reported, close monitoring and continuous surveillance is warranted., (© 2011 The International Society of Dermatology.)

Vulvo-perineal Crohn's disease is a rare condition either when it is isolated or associated with digestive manifestations. In the former condition named metastatic Crohn's disease, it may constitute a diagnostic challenge and may be confused especially with other infectious or inflammatory disorders. We report a case of vulvo-perineal Crohn's disease in a 46-year-old woman. A 46-year-old woman was diagnosed with a vulvo-perineal Crohn's disease without digestive involvement. There was a chronic edema of the vulva with linear ulcerations on the inguino-crural regions and the buttocks fold, of 3 years. Treatment with metronidazole (1 g/day for 6 months) led to almost complete healing of the ulcerations with a sustained result. Physicians must be aware of the diverse manifestations and confusing presentations of vulvo-perineal Crohn's disease.

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.

Background: Chronic lupus erythematosus is a cutaneous form of lupus erythematosus, usually involving photoexposed areas., Aim: The aim of our study is to evaluate the epidemio-clinical trends, therapeutic features and outcome of patients with chronic lupus erythematosus, through a Tunisian hospital series. We have tried to compare our results with those of other African and western series., Methods: It is a retrospective study, concerning all patients with chronic lupus erythematosus, attending the Dermatology department of Charles Nicolle Hospital over an 11 years period. The patients' age, sex, clinical features, explorations results, treatment and evolution were recorded., Results: A total of 104 patients were included. Chronic lupus erythematosus represented 0,1 % of all the dermatitis seen over 11 years. Chronic lupus erythematosus affects young women with a ratio F/M of 1,97 and an average age of 42 years. The discoid form was the most frequent clinical shape, observed in 73 % of cases (76 patients). The face was the most frequent localization of the lesions (91%). Five patients (4.8%) presented a generalized chronic lupus erythematosus. The direct immunofluorescence in sick skin was positive in 54 % of cases. Treatment included sun avoidance and oral antimalarials drugs in the majority of cases (95%). The progression from chronic lupus erythematosus to systemic lupus erythemetosus was observed in two cases (2%)., Conclusion: Chronic lupus erythematosus is a relatively rare disorder in Tunisia, compared to other African countries, where prevalence is 7 to 10 times more frequent. As shown in our study, the most common clinical feature is the discoid form. The diagnosis can be confirmed by histopathological and direct immunofluorescence examination. Progression of chronic lupus erythematosus to systemic lupus erythematosus is possible, as observed in two of our patients. Thus, Patients with chronic lupus erythematosus should be continually followed up.

Introduction: Acrodermatitis enteropathica is a rare autosomal recessive disease due to an abnormality in a zinc transporting molecule., Methods: We conducted a retrospective monocentric study on 29 Tunisian cases of Acrodermatitis enteropathica (AE) treated in our Department of Dermatology in Tunisia, between January 1981 and June 2008., Results: The age of onset of disorders was between 15 d and 12 months (mean 6.86 ± 3.25 months). The delay of consultation ranged between 15 d and 8 months (mean of 2.8 ± 2.17 months) after onset. Onset of gastrointestinal and psychiatric signs depended significantly on consulting times. Plasma zinc levels ranged between 14 and 88 lg/100 ml (mean 44.86 ± 18.4 lg/100 ml). There was not a significant relation between zincemia and clinical features. Genetic analyses in 13 of our patients showed three different mutations in the SLC39A4 gene: c.1223_1227del (p.Trp411ArgfsX7) in exon 7,c.143T>G (p.Leu48X) in exon 1 and c.1784T>C (p.Gly595Val) in exon 11. No significant genotype-phenotype correlations could be established., Conclusion: Acrodermatitis enteropathica is a rare disease which diagnosis is easy. Its biological confirmation is made on a simple dosage of zincemia. However, the diagnosis is not always suggested, and is unfortunately made late. At present, there is a molecular test to detect SLC39A4 mutations., (© 2010 The International Society of Dermatology.)

Background: Rosacea is a common, chronic facial dermatosis of uncertain etiology, several well-defined types and variable progression. There is a paucity of epidemiologic studies from North African countries especially in Tunisia., Aim: to determine epidemiological, clinical, histological features, treatment and outcome of rosacea in a Tunisian study., Methods: A retrospective study of all rosacea cases diagnosed in the outpatient Dermatology Department of Charles Nicolle hospital of Tunis was conducted between 1990, January and 2003, May., Results: Our study included 244 patients. The diagnosis of rosacea was made on symptoms and clinical features in 185 cases and on histological findings in 59 patients. The hospital prevalence of rosacea was 0.2%. The sex ratio M/F was 0,4. Patient's Mean age was of 49 years. Triggered factors mentioned included most often: sun exposure (64%) and thermal stimuli (25%). Mean duration between the onset of symptoms and the first consultation was about 20 months. The rosacea subtypes were: erythematotelangiectatic type (12%), papulopustular rosacea (69%), and rhinophyma (3.7%). Granulomatous rosacea was diagnosed in 8 patients and steroid rosacea in 28 cases. Ocular rosacea was observed in 41 cases. Treatment modalities included: topical treatment (1.6%), systemic treatment (85%), systemic and topical treatment (5%) and surgical procedures (1.2%)., Conclusions: Rosacea seems to be a frequent dermatosis in Tunisia where most of the population is phenotype IV-V. It affects mostly middle-aged women. Most of our patients present with papulopustular rosacea (69%). More epidemiological and clinical studies in North African countries should be conducted to emphasize these results.

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.

Background: Erythroderma is a severe syndrome and prognostic studies are rare in the literature., Objectives: Through a retrospective study of erythroderma in adults, we have analysed epidemiological and clinical data and precised the relevant aetiologies and survival in our patients., Methods: This study was performed at the Department of Dermatology of Charles Nicolle Hospital of Tunis (1995-2007) including 82 cases of acquired erythroderma (>16 years). We have recorded epidemio-clinical, biological and histological data, treatment and outcome. Clinical-histological correlation was analysed [kappa coefficient (kappa)]. Follow-up time and disease-free survival time were calculated as were Kaplan-Meier estimates of overall survival and relapse-free survival for some aetiologies., Results: Erythroderma represented 0.44 per thousand of all dermatoses with an age of 55.13 +/- 18.16 and no sex predilection. Psoriasis was the predominant aetiology (32.9%) with a median duration of 6.75 years and previous one or more episodes of erythroderma. Psoriasis was significantly associated with pruritus (P = 0.0001), pachyonychia (P = 0.00001), palmoplantar keratoderma (P = 0.0001) and hypereosinophilia (P = 0.008). The latter is then not specific for drug induced erythroderma (P = 0.004). Carbamazepine (27.8%) and penicillin (22.2%) were the most implicated drugs. Positive Clinical-histological correlation was found in 77% of cases (kappa = 0.753). Relapse was seen in all aetiologies, but drug reactions and had occurred in the first 3 years in 90% of them. Mortality rate was 11.3 per 1000 patients-years., Conclusions: Our study illustrates the severity of erythroderma. It alters heavily the quality of life of patients which is initially altered by the pre-existent dermatosis. It may be life threatening as mortality rate is high.

A 46-year-old woman with a medical history of chronic juvenile arthritis with bilateral prosthetic hips presented with vulvoperineal ulcerations of 3 years' duration. There was no diarrhea or recent weight loss. Cutaneous examination showed asymmetrical vulvar edema of the labia minora and labia majora with deep and linear ulcerations having verrucous borders located on the inguinocrural regions and the buttocks fold (Figure 1). On physical examination there was bilateral limited mobilization of the hips. A biopsy specimen was taken from the border of the vulvar ulceration and histologic examination showed under a hyperplasic epidermis an epithelioid granuloma with multinucleated giant cells of the dermis without caseification. Laboratory analyses and results from chest x-ray were normal. Results for Koch bacilla in the spittle, microbiologic studies (staining for microorganisms and cultures), and tuberculin intradermoreaction were negative. There was no Crohn's disease aspect on colonoscopy, and there was normal small bowel enterography. Systematic intestinal biopsies were also with normal aspect. Based on the clinical data and granulomatous histologic characteristics, the diagnosis of metastatic Crohn's disease without digestive involvement was obtained. The patient was started on metronidazole 1 g/d. After 6 months of treatment, there was an almost-complete healing of ulcerations (Figure 2). Treatment was well-tolerated.

Summary Background: Most of the published reports on Hailey-Hailey disease (HHD) come from European and Asian countries. We report here the clinical and genetic investigation of 20 patients affected with HHD in Tunisia., Methods: Affected individuals from three large teaching hospitals in Tunis were recruited for the study over a 25-year period. Nine patients were identified through the active files and examined together with their family members that were visited in their respective regions. We have clinically examined in total 65 individuals and then identified 11 new cases. Patients were included on the basis of evocative skin lesions, biopsy proven HHD and negative immunofluorescence. Investigations to rule out fungal, bacterial and viral infections were done according to clinical symptoms., Results: Twenty patients (12 males and 8 females) from 8 families were included in the present study with more than 55% that were undiagnosed before this investigation. Four patients had mild disease, eight had moderate disease and another eight had severe disease, among whom seven were females. Parental consanguinity was found in 7 cases out of 20 cases (35%). The neck region was first affected in half (4/8) of the male patients. Groins were first affected in 42% (5/12) of the female patients. Depression complicated the course of the disease in two female patients with severe HHD. We report an original association of supernumerary nipples with HHD in two sisters from the north of Tunisia. In 10 patients, the disease has become less troublesome with aging., Conclusion: HHD is underestimated. Physicians must be aware of this disease in case of resistant intertriginous dermatosis especially with a positive family history as nine out of 20 patients were misdiagnosed.

A unique feature of the skin immune system is its proximity to cells continuously exposed to sun rays, as it is located in the interface between the body and the environment. In this study, we aimed to determine the impact of DNA damaged keratinocytes on the expression of apoptotic-related molecules, in T-cells of the inflammatory component of the tumor environment. Immunohistochemistry was performed on tissue sections derived from skin biopsies of basal cell carcinomas (BCCs) of xeroderma pigmentosum (XP) patients, non-XP patients and nevoid basal cell carcinoma syndrome (NBCCS) patients, using antibodies against B-cell lymphoma/leukemia-2 (Bcl-2), Bcl-2 associated X protein (Bax), CD95, CD3, CD8 and CD56. Our results showed significantly lower levels of expression of the antiapoptotic Bcl-2 molecule, in XP, in comparison with non-XP and NBCCS T-lymphocytes, leading to the highest Bax/Bcl-2 ratio for XP T-cells. For the CD95 receptor expression levels, there were significant differences among T-cells of the three patient subgroups as well. The higher propensity of XP T-cells to undergo apoptosis may have evolved in individual XP patients, apparently during the course of their disease, to maintain a special skin as an immune privilege site for tumors' development.

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.

Cicatricial pemphigoid is an extremely rare condition in children, since only 17 cases have been reported in the English literature, so far. We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation.

Tacrolimus 0.03% ointment is licensed for second-line treatment of children with atopic dermatitis (AD). Although data are available from clinical trials, no study has enrolled only second-line patients. This double-blind, non-inferiority study compared tacrolimus 0.03% and fluticasone 0.005% ointments in children with moderate-to-severe AD, who had responded insufficiently to conventional therapies. Children (aged 2-15 yr) were randomized to tacrolimus ointment (n = 240) or fluticasone ointment (n = 239), twice daily until clearance or for a maximum of 3 wk and, if lesions remained, once daily for up to 3 wk further. Primary end-point was week 3 response rate (improvement of >or=60% in modified Eczema Area and Severity Index and not withdrawn for lack of efficacy). Secondary end-points included pruritus and sleep quality, global assessment of clinical response, incidence of new flares and safety. Response rates were 86.3% with tacrolimus ointment and 91.5% with fluticasone. Lower limit of the 95% confidence interval was -11.8%, exceeding the non-inferiority limit of -15% and meeting the primary end-point. Moderate or better improvement on the physicians' global assessment occurred in 93.6% and 92.4% of patients in the tacrolimus ointment and fluticasone arms, respectively, while median pruritus scores improved by 84.0% and 91.5%. Sleep quality improved by approximately 92% in both treatment arms. After day 21, new flare-up occurred in 5.5% and 11.3% of patients receiving tacrolimus ointment and fluticasone, respectively; mean times to new flares were 6.5 +/- 5.0 and 8.6 +/- 5.2 days. Adverse events were similar between the two arms, with the exception of application-site skin burning sensation in the tacrolimus ointment group. In conclusion, efficacy of tacrolimus 0.03% ointment as second-line treatment was not inferior to that of fluticasone 0.005% ointment, with similar benefits on global disease improvement and quality of sleep.

Transversal case-control study was conducted among 42 patients aged between 20 and 35 years who had diffuse alopecia. Alimentary inquiry according to the 3-day method was achieved in each case. The following nutriments were evaluated: total proteins, calcium, copper, iron, iodine, magnesium, manganese, phosphorus, potassium, selenium, zinc, omega 3 and omega 6. A control group (composed of 230 individuals), matched for age, gender and metabolic profile was established. These persons did not suffer from hair and nail disorder. The nutriments were codified according to the data of the software Food processor 8.3 version. The data were analyzed using "SPSS" 11.5 version. Comparisons of the means were performed using the Student's t test. ROC graphics allowed to determine the statistically significative limits for the comparison of both groups. On multivariate analysis, only a protein intake was directly associated to alopecia, odds ratio of 1,5 (1,06 - 2,3) p=0,02.

Background: Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa. We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBD) in Tunisian children., Methods: We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for whom direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population., Results: Thirty-one children with LAD were collected representing 65.9 percent of all AIBD of children collected in the same period, with a mean age of 5.5 years and a sex ratio M/F of 2.4. Most of the children had a generalized eruption (28/31) but more profuse on the face, pelvic region, buttocks, and limbs. Mucosal lesions were present in only 4 children (12.9%). The mean duration of the disease was 14 months. Direct immunofluorescence demonstrated predominantly linear IgA deposits along the dermal-epidermal junction in all patients. Faint IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67 percent of cases. Eight patients responded to Dapsone, but prednisone had to be added in 7 children and erythromycin in 4 others to control the disease. A long term remission period (34 months) was achieved in 61.9 percent of patients., Conclusion: This study confirms that LAD is the most common AIBD in children in Tunisia and it frequently occurs in preschool-aged males. Independently of the medication chosen for treatment, a long term remission is frequently observed.

Background: Leishmaniasis is wide spread parasitic disease considered to be endemic in 88 countries in both old and new world. The standard treatment remains Meglumine antimoniate., Aim: We study the side effects of systemic meglumine antimoniate in cutaneous leishmaniasis., Methods: We conduct a retrospective study covering 3-year period (2002- 2005). All medical reports of cutaneous leishmaniasis treated by systemic Meglumine antimoniate are reviewed., Results: The study comprise 63 patients all treated by systemic meglumine antimoniate at the dose of 60 mg/kg/day for 10-15 days. Side effects were noted in 15 cases (12 females and 3 males). The subject's age range from 11 to 78 years. Stibio-intolerance (fever, rash, arthralgia, abdominal pain) was observed in 12 cases and stibiotoxicity in 3 cases: precordialgies 1 case, hyperamylasemia and increase liver enzyme: 1 case, pancytopenia, renal and hepatic failure leading to death: 1 case, skin eruption: 7 cases, pruritus and erythema in the site of injection: 5 cases, urticaria: 1 case. Meglumine antimoniate was stopped in 13 cases., Conclusion: Meglumine antimoniate is the generally recommended treatment of cutaneous leishmaniasis. In spite of the rarity of Glucantime's side effects, we recommend a careful survey especially in older patients.

Background: Ectodermal dysplasias are rare hereditary diseases characterised by congenital absence of ectodermally derived structures and classified according to four symptoms: trichodysplasia, hypodontia, onychodysplasia and hypohidrosis., Aim: The objective of our study is to precise the epidemioclinical characteristics, the diagnostic tools, the evolution and the treatments of this rare disease through a 10-case series of hypohidrotic ectodermal dysplasia (HED)., Methods: The present report is a retrospective study of all cases of an/hypohidrotic ectodermal dysplasia collected from 1977 to 2006. We have specified age, sex, parental consanguinity, similar familial cases, clinical and histological features, dental, oto-rhino-laryngologic, ophthalmologic and respiratory examinations., Results: Ten cases of HED were collected (average age: 14 years, sex ratio 9/1). The mean duration diagnostic period was of 14 years. Parental consanguinity was registered in 3 cases but only one patient had similar familial cases. All patients had facial dysmorphy, hypotrichosis and hypo/anodontia (respectively 8/10 and 2/10). All patients had clinically and histologically documented hypoplastic (6/10) or aplastic sweat glands (4/10). Extra-cutaneous manifestations were noted in 8 patients (recurrent rhinitis 6/10, recurrent pneumopathies 3/10, xerophtalmy 3/10)., Conclusion: Our series deals with 10 cases of HED, consisting in Chris-Siemens Touraine syndrome. It highlights the delayed diagnosis of this disease (mean: 14 years) with a diagnosis made at an adult age in four patients. Our study confirm the X-linked heredity (9/10) with a possible autosomal transmission (one female-case). HED is rarely life-threatening, but early diagnosis allows a better quality of life to patients and genetic counselling to parents. Our series illustrates the rarity of HED which is also probably due to its underestimation by clinicians.

Background: PUVA therapy and narrowband UVB therapy (NBUVB) constitute a widely used first line therapy in mycosis fungoides (MF)., Methods: It is a retrospective study dealing with 7 patients with MF treated by phototherapy [3 with PUVA (III, IV, V phototype) and 4 with NBUVB (III-IV phototype)]. One patient had both treatments (PUVA then NBUVB for a relapse)., Results: Complete response (CR) was achieved in 2 of the 3 patients treated with PUVA (they had stage IB-MF). Both had relapsed after 40 sessions and 7 months later, respectively. Relapses were treated by PUVA maintenance treatment (2 sessions/2 weeks) and NBUVB leading to CR. The third patient (stage IIA) did not respond to PUVA. CR was obtained in the 5 patients treated with NBUVB (stage IA-IB), after a mean cumulative dose of 58.3 J/cm(2). No relapse was noted with NBUVB after a mean follow-up period of 13.2 months (extremes: 2 -31 months)., Conclusion: NBUVB is only effective on non-infiltrated plaques independently of phototype. PUVA therapy is effective even on infiltrated plaques. Both treatments do not prevent relapses.

Background: Cryotherapy with liquid nitrogen is the most widely used method to treat hand and feet warts. Therapeutic response to this method depends on many factors related to warts and to the patient. The aim of this study is to determine factors influencing therapeutic response of warts to cryotherapy by liquid nitrogen., Methods: It was a prospective transversal study including 100 patients with warts of the hands and/or feet treated by cryotherapy with liquid nitrogen (cotton wool bud) and referred to Dermatology Department of Charles Nicolle Hospital of Tunis. Demographic data, so as characteristics of warts were recorded. Patients received one treatment/week with a maximum of 4 sessions. Patients whose warts were seen to be resolved were classified as cured. Cure-predictive factors were studied with a multi varied study with logistic regression., Results: Of the 100 patients (56 females/44 males, Mean age: 22 years), ten were withdrawn. In 89 patients, warts were present on hands, whereas 23 had warts on feet and 12 had warts on both hands and feet. The mean number of warts per patient was 7. The total cure rate was of 64.4% and was more elevated in hands compared to feet (70.8% versus 10.5%). There was no difference between mean ages of cured group and not cured one (22.2 years versus 21 years). The mean duration of warts in cured patients was lower than that of not cured patients. The mean number of warts before treatment was 4.3 in cured patients and 12.3 warts in not cured patients. The mean number of treatments was 2.3 in cured patients and 4 treatments in not cured patients. The difference between these factors into cured and not cured groups was statistically significant in uni-varied study but not significant in multi-varied one., Conclusion: The effectiveness of liquid nitrogen used by traditional method in the treatment of hand and feet warts seems to depend on multiple factors: wart's duration, number of warts and number of treatments. These factors depend on each other.

Background: Sclerodermas are rare affections which can be located or generalized. Localized form is the most frequent., Aim: The purpose of this study was to describe epidemiologic, clinics, biological, immunological, therapeutic, evolutionary characteristics of the localized scleroderma through a personal series and the data of the literature., Method: We have performed a retrospective study on all patients followed in the department of dermatology of the Hospital Charles Nicole during 14 years period., Results: Our study was about 92 cases of localized scleroderma (73 were females and 19 males). The mean age was 35 years (between 2 and 72 years). The majority of localised sclerodermas (66.2% of the cases) appeared before 40 years with a maximum of frequency between 10 and 30 years (41.6%). Only 11.9% of the cases were observed before 10 years. They were 51 cases (55%) of morphea, 35 cases (38%) of scleroderma in bands including 32 linear scleroderma and 3 scleroderma en coup de sabre, 5 cases (5.5%) of generalized morphea and 1 case (0.15%) of deep morphea. Average therapeutic was specified among 63 patients (87%), and the evolution could be appreciated among 45 patients., Conclusion: The epidemiologic data observed in our series are comparable with those reported in the literature. Therapeutic difficulties and risks of functional after-effects, particular in scleroderma in bands, remain the principal concern for all the authors.