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A possible etiological link between the onset of endemic pemphigus in Tunisia and bites of Phlebotomus papatasi, the vector of zoonotic cutaneous leishmaniasis, has been previously suggested. We hypothesized that the immunodominant P. papatasi salivary protein PpSP32 binds to desmogleins 1 and 3 (Dsg1 and Dsg3), triggering loss of tolerance to these pemphigus target autoantigens. Here, we show using far-Western blot that the recombinant PpSP32 protein (rPpSP32) binds to epidermal proteins with a MW of approximately 170 kDa. Coimmunoprecipitation revealed the interaction of rPpSP32 with either Dsg1 or Dsg3. A specific interaction between PpSP32 and Dsg1 and Dsg3 was further demonstrated by ELISA assays. Finally, mice immunized with rPpSP32 twice per week exhibited significantly increased levels of anti-Dsg1 and -Dsg3 antibodies from day 75 to 120. Such antibodies were specific for Dsg1 and Dsg3 and were not the result of cross-reactivity to PpSP32. In this study, we demonstrated for the first time to our knowledge a specific binding between PpSP32 and Dsg1 and Dsg3, which might underlie the triggering of anti-Dsg antibodies in patients exposed to sand fly bites. We also confirmed the development of specific anti-Dsg1 and -Dsg3 antibodies in vivo after PpSP32 immunization in mice. Collectively, our results provide evidence that environmental factors, such as the exposure to P. papatasi bites, can trigger the development of autoimmune antibodies., A specific binding between Phlebotomus papatasi salivary protein PpSP32 and desmogleins may trigger anti-Dsg antibodies in patients exposed to sand fly bites.

BACKGROUND Nail unit melanoma (NUM) is a rare melanoma variant, usually associated with a poor prognosis because of a delayed diagnosis. Few data are available concerning the management and long-term outcome of in situ NUM. OBJECTIVE To use a large cohort to provide comprehensive patient data and long-term follow-up information. This will serve to investigate distinctive epidemiological, clinical and histological features of in situ NUM. To report treatment modalities, assess conservative surgery and evaluate its long-term safety. METHODS Patients with confirmed diagnosis of in situ NUM were retrospectively reviewed. Demographics, clinical presentation, therapeutic data and follow-up were analysed. RESULTS Sixty-three cases of in situ NUM were identified as follows: 44 were women (70%), with a mean age of 51 years. The mean duration of symptoms prior to consultation was 4.3 years [range 3 months-28 years]. Clinically, with 58 cases, i.e. (92%) longitudinal melanonychia was the most common clinical presentation. The thumb was the most affected digit, being afflicted in 28 cases: 44%. Medical history found a widening and/or recent darkening of the melanonychia in 46 cases (82%). The treatment consisted of, respectively, 56 En bloc excisions of the nail apparatus 89% of the patients we studied, and seven amputations of the distal phalanx. During the follow-up period (mean: 10 years), two patients presented in situ recurrences. CONCLUSION The recognition of a NUM at an in situ stage allows early treatment and curing of this tumour. At this early stage, a 'functional surgery' is a rational approach with an excellent oncologic safety.

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A previously healthy 70-year-old woman presented with a 5-month history of an asymptomatic keratotic, papulonodular plaque on her right forearm. The lesion started as a follicular papule followed by progressive peripheral proliferation. No record of trauma, contact with any chemicals, use of immunosuppressive drugs, or history of neoplasm was noted. Clinical examination showed an arciform plaque of 10×5 cm, with infiltrated raised borders and central atrophy (Figure 1). Drops of yellowish material exuded from the coalescent nodules constituting an elevated and indurate border. Results from physical and laboratory examinations revealed no internal organ malignancy. The remainder of the physical examination (x-ray of the forearm and serologies for HIV, hepatitis, and syphilis) was normal.

Cutaneous peripheral T-cell lymphomas not otherwise specified (CPTL-NOS) are rare neoplasms accounting for just 2% of cutaneous peripheral T-cell lymphomas (CPTL). Only very few case series have been reported. They represent a phenotypically and prognostically heterogenous group of CPTL that do not fit into any of CPTL well-defined subtypes. The authors report a case of a 64-year-old man with simultaneous plaque-like lesions and disseminated nodules growing rapidly on the face, trunk, and extremities over a 6-month period. There was no a history of preceding patches, erythematous plaques, rash, or pruritic lesions. These lesions were extending over 80% of the skin surface. Histopathologic analysis revealed dense diffuse infiltrates composed of mostly medium-sized to large lymphoid cells throughout the entire dermis without epidermotropism. Neoplastic cells were atypical with markedly pleomorphic nuclei. Immunohistochemistry showed that the tumor cells were positive for CD3, CD4, and CD5 with a loss of CD7. They were negative for CD20, CD8, CD56, CXCL13, PD1, TIA-1, granzyme-B, perforin, CD25, and CD30. The proliferative fraction was low, with MIB-1 labeling less than 10% of cells. The authors diagnosed the patient with primary CPTL-NOS. Despite the rarity of these tumors, clinicians as well as dermatopathologists and pathologists should be familiar with these rare CPTL especially because most of these lymphomas have an aggressive behavior and exhibit an unfavorable prognosis.

Mal de Meleda (MdM, MIM: 248300) is a rare autosomal recessive skin disorder characterized by diffuse palmoplantar keratoderma and transgressive keratosis with onset in early infancy. The gene responsible for MdM, ARS, encodes for Secreted Lys6/Plaur domain-containing protein 1 which is essential for epidermal homeostasis. Tight junctions have been proposed to have two mutually exclusive functions: a fence function which prevents the mixing of membrane proteins between the apical and basolateral membranes; and a gate function which controls the paracellular passage of ions and solutes between cells. In this study we report immunohistochemical investigations of tight junction proteins claudin-1 and occludin in MdM Tunisian families. Nine skin biopsies from patients with MdM were analyzed. The control group was formed by skin biopsies belonging to healthy individuals. Immunohistochemical study was performed on fixed sections from biopsies of four microns with the following polyclonal antibodies: anti-claudin-1 and anti-occludin. In control skin, claudin-1 exhibited membrane expression throughout the epidermis with increasing and upward intensity, whereas occludin was detected in the cell membrane of keratinocytes of the stratum granulosum. In MdM skin, claudin-1 was expressed throughout the thickness of the spinous layers with membrane staining, and occludin had cytoplasmic staining in the granular layer. The immunohistochemical expression of TJ proteins in MdM patients harbors premature expression of occludin and decreased expression of claudin-1, highlighting further evidence for disorders in epidermal homeostasis.

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.

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. Renseignements de base: L'ochronose endogène (OE) est une affection héréditaire récessive autosomique où il se produit une oxydation incomplète de la tyrosine et de la phénylalanine en raison d'un manque d'oxydase de l'acide homogentisique. Objectif: Nous présentons une observation singulière d'OE dont l'issue a été fatale. Rapport de cas: Nous exposons le cas d'un homme de 46 ans né de parents consanguins ayant des antécédents médicaux de coliques néphrétiques récurrentes et une arthropathie chronique non spécifique. À l'examen clinique, nous avons observé une pigmentation bleu ardoise au niveau des joues, du front, et du nez, ainsi que des plaques bleu gris sur tous les ongles de doigts et une décoloration bleuâtre des gencives. Une étude des antécédents familiaux a révélé que sa sœur présentait une pigmentation semblable au niveau des oreilles, des mains, et des ongles de doigts. L'examen histologique de l'échantillon de la biopsie pratiquée sur une lésion pigmentée a montré un dépôt cutané de matériel éosinophile acellulaire sans réaction cellulaire. Selon les données cliniques et histopathologiques, combinées aux antécédents médicaux familiaux, notre patient présentait une OE avec une atteinte rénale, articulaire, et muco-cutanée. Malheureusement, le diagnostic a été tardif puisque notre patient est décédé quelques mois plus tard d'insuffisance rénale terminale. Conclusion: Les signes cutanés constituent les caractéristiques de l'OE et ceux-ci doivent alerter le clinicien de manière à ce qu'il cherche la contribution des organes vitaux.

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.

Author(s): Zaraa, Ines; Dorbani, Imen; Hawilo, Abdelmoati; Mokni, Mourad; Ben Osman, Abel | Abstract: Background: Ingrown toenail is a common problem causing significant morbidity. Various procedures used for treating this painful condition were discussed in the literature.Objetctive: Through a prospective study we aimed to evaluate in a long-term follow-up the efficacy of partial nail plate avulsion combined with phenolic chemical matricectomy for the treatment of Ingrown toenails.Methods: We recruited outpatient from January 2006 to December 2009; all treated by phenol matricectomy and assured a regular follow up.Results: Consecutive 146 patients with a total of 171 Ingrown toenails were enrolled. There was no significant morbidity and the procedure was tolerated well by all patients. The short-term results were excellent. No severe complications occurred during the 21-month follow-up period. The overall recurrence was 2.7 %.Conclusion: Phenol cauterization compared with other surgical ablation techniques may be the treatment of choice of Ingrown toenails. It’s an effective technique for the treatment of Ingrown toenails accompanied by less morbidity and lower recurrence rates.

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.To present the epidemiological, clinical, pathological, therapeutic features and out come of SCC of the lips.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.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.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 (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.

The pathogenesis of all forms of psoriasis remains obscure. Segregation analysis and twin studies together with ethnic differences in disease frequency all point to an underlying genetic susceptibility to psoriasis, which is both complex and likely to reflect the action of a number of genes.In the present study, we performed a family-based association study, and a transmission dysequilibrium test using the PLINK program, in a set of seven Tunisian multiplex families using a panel of 96 single-nucleotide polymorphisms localized in several regions across the genome. Ninety-five of them were reported to be associated with psoriasis in different populations.Besides the confirmation of association between previous associated regions: 6p, 1p, 2p, 13q, 14q, and 20p, and cutaneous psoriasis, we identified a new association with the rs1249564 in the IL17RD gene.Our results support the complex genetic basis of psoriasis.

Psoriasis is a chronic inflammatory skin disease often benign, affecting 2-3% of the total world population. Psoriasis is a multifactorial disease.To present recent advances in the immunologic mechanisms and susceptibility genes involved in the pathogenesis of psoriasis.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.Recent works focalized mainly in immunology and genetics. Current progresses in molecular biology have allowed to better characterize the immunogenetic abnormalities in psoriasis.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.

We describe the case of a 75-year-old man with a history of chronic lymphoid leukaemia stage B in Binet system treated by chemotherapy with chlorambucil and prednisolone. The patient was in complete remission with this treatment for 3 years. When he presented to our department, he complained of two painful and indurated swellings in the right fifth finger and in the right wrist that had appeared 7 months before (figures 1AandB). Blood tests showed increased erythrocyte sedimentation rate and C reactive protein level with a …

International audience; 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, 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.

Kaposi's sarcoma (KS) is a mutifocal angiogenic process characterized by cellular and vascular proliferation.To identify the epidemio-clinical, histological and therapeutic features of KS.Retrospective study of 75 cases of KS at the dermatology department of Rabta hospital in Tunis during a period of 25 years (1982-2007).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%.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.

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the erythrodermic psoriasis (EP) is a rare but severe form of psoriasis that may be potentially life-threatening.To study the characteristics of this severe form of psoriasis.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.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.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.

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Autoimmune bullous dermatoses (ABD) are a rare but significant group of cutaneous disorders posing great diagnostic and therapeutic challenges to the treating dermatologist. Few surveys have been carried out to describe the whole spectrum of ABD in a region.To determine the clinicoepidemiological features of various ABD.We retrospectively recruited all ABD cases seen during an 11-year period (1997-2007).One hundred and seventy-four patients were involved (16.3 cases/year). Pemphigus was observed to be the most common ABD (53%), with the majority being pemphigus vulgaris (61%) vs. 36% of pemphigus foliaceus (Tunisian pemphigus).Most epidemiological surveys have focused on a single or just one group of ABD. In our series, pemphigus foliaceus was relatively more prevalent than in occidental data. The frequencies of subepidermal immunobullous dermatoses in our study were in agreement with those in the literature.

Erythema nodosum (EN) in association with kerion celsi is a rare condition in children, with only 11 cases having been reported in the English literature. We describe a new case in a 7-year-old boy in whom the disorder had begun 2 months before. He had many inflamed, boggy, suppurative nodules over the left occipitoparietal area of the scalp and, 2 weeks later, developed multiple painful, erythematous subcutaneous nodules of the shins, thighs, and upper limbs. EN was confirmed by histologic examination. Our review of the literature of all cases of EN during kerion showed that it usually occurs at or slightly after the height of infection or after drug introduction. Trichophyton mentagrophytes was involved in the majority of cases, and improvement of EN usually occurs with griseofulvin.

Dermatomyositis (DM) is a rare inflammatory autoimmune disease for which an iatrogenic origin has been described in a few cases. The authors report a case of DM occurring after simvastatin intake. A 50-year-old male sought medical attention for a photodistributed rash and considerable muscular weakness present for 3 months. One year earlier, simvastatin had been introduced. Serum creatine kinase levels were elevated. Histological examination of a muscle biopsy was consistent with a diagnosis of DM. Investigation for neoplasia and associated autoimmune disease proved negative. All clinical and laboratory abnormalities diminished corticosteroid therapy (1 mg/kg/day). Case reports have suggested that lipid-lowering drugs, especially statins, could induce or reveal chronic muscle diseases. In statins myopathy, reduction of coenzyme Q has been discussed as a key mechanism. Our case of DM in a patient receiving simvastatin adds to the previous reported cases in the literature and highlights the potential role of statins as triggers of immune systemic diseases.

Drug-induced lupus erythematosus is defined as a lupus-like syndrome temporally related to a drug exposure. We report a 34-year-old woman with multiple sclerosis who developed, while being treated with interferon β-1a, myalgia and associated with wrist synovitis. Clinical and immunologic investigations were in favor of systemic lupus erythematosus. Interferon therapy was maintained. Under corticosteroids and antipaludeen treatment, the patient did not develop any other lupic manifestations.

Chronic urticaria (CU) is a common condition. Usually benign, it can be debilitating. The main step was to identify the potential causes of CU to institute the strategy for management of patients.To present the epidemiological, clinical and therapeutic features of CU.233 cases of CU were enrolled through a retrospective study conducted in the dermatology department of La Rabta hospital during 10 years (1997-2006).The mean age of our patients was about 36 years with a sex ratio of 2.28. The mean duration of the CU before the consultation was 21 months. Inducing Factors were reported in 109 cases, dominated by physical factors (73 cases). Severe symptoms as facial edema were observed in 44 cases. The CU was considered as idiopathic in 179 cases (77%). An etiology was found in 54 cases, dominated by physical origin, noted in 25 cases. All patients were treated by antihistaminic. Systemic corticosteroids were associated in 41 patients. Partial improvement was the mainly outcome (166 cases).CU is a frequent disease which the diagnosis is based on clinical exam. The causes of CU were numerous and sometimes intricate, that gives the etiologic courses difficult and usually disappointing.

Behçet's disease (BD) is a systemic inflammatory disease involving many systemic lesions characterized by vasculitis as a common basic pathologic process. Mucocutaneous (MC) features are considered as the diagnostic hallmarks.To retrospectively determine the epidemiological and clinical aspects of BD mucocutaneous symptoms in Tunisia compared to those found in the literature.Twenty-eight patients were involved in a single-center dermatological recruitment for a 27-year period (1980-2007) on the basis of international BD criteria.Our series included 19 males and 9 females. The mean age at onset was 28 years and the mean age at diagnosis was 37.9 years. The frequency of MC manifestations was as follows: oral ulcers (100%), genital ulcers (92.8%), other cutaneous signs (82.1%) such as papulopustular lesions (53.5%), and dermohypodermal nodes (11.53%), aphthous cutaneous ulcers (17.8%), and other lesions (leg ulcer: three cases, erythema multiform: one case, and infected pyodermatitis: one case). Systemic manifestations were present in 18 patients.MC manifestations are hallmarks of the disease and usually the onset symptoms. The frequencies of the various MC signs seen in our study broadly match those found in the literature.

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: Depilatory radiotherapy for ringworm was largely used before antifungals were available. Patients who underwent this treatment are at high risk of developing scalp tumors or other cancers. The aim of this study was to characterize scalp tumors occurring after X-ray therapy for ringworm. Methods: We included cases of postradiotherapy scalp tumors recorded at the Dermatology Department of the Charles Nicolle Hospital, Tunis between 1988 and 2001. We recorded clinical descriptions and all cases were resubmitted to microscopic analysis. Results: Sixty-one tumors occurred in 33 men and 12 women with a mean age of 49.8 years. Radiodermatitis was present in 21% of patients. Tumors were basal cell carcinomas in 47 cases, trichoblastomas in 10 cases and trichoblastic carcinomas in 4 cases. Twelve patients had 2–5 tumors, with combinations of tumor types in 3 of them. Mean delay of onset of tumors after radiotherapy was 39.4 years in basal cell carcinoma cases, 38.3 years in trichoblastoma cases and 35.6 years in trichoblastic carcinoma cases. Conclusions: This series shows that although basal cell carcinoma is the most frequent tumor in this situation, trichoblastomas are common. We describe, for the first time, radio-induced trichoblastic carcinomas. Trichoblastic tumors have not yet been described in this context because this concept is relatively recent.

Primary mucinous carcinoma of the skin is a rare sweat-gland neoplasm with a high recurrence rate. We report a new case of a primary recurrent mucinous carcinoma of the face in a 59-year-old man. Histopathologic examination of the neoplasm showed epithelial islands floating in mucoid material compartmentalized by fibrous septa. Cytokeratin 7, protein S100, estrogen and progesterone receptors were detected at immunohistochemical study, while cytokeratin 20 and actin were undetectable. Histologically, mucinous carcinoma of the skin can be mistaken for a metastasis from extracutaneous sites, particularly the breast or the gastrointestinal tract. Mucinous carcinoma of the skin has a relatively good prognosis with rare distant metastases, but high recurrence rate.

Michelin tire syndrome is a rare syndrome characterized by excessive folding of the skin. The diagnosis is mainly clinical. It has been found to be associated with noncutaneous anomalies, and probably reflects multiple underlying disorders. We report two siblings with Michelin tire syndrome, a 5-year-old boy and his sister both of whom had marked skin folds and facial anomalies. Histologic study found an increase in smooth muscle fibers of the dermis. Electron microscopy showed details of smooth muscle cells. We also review reported cases in the literature to contribute to a better understanding of this syndrome.

Background Dyskeratosis follicularis (Darier's disease) is rare autosomal dominant disease characterized by the loss of adhesion between epidermal cells and by abnormal keratinization. Methods We performed a retrospective study of all the patients diagnosed with Darier's disease at the Department of Dermatology of Charles Nicolle Hospital of Tunis, between 1971 and 2002. Results During the observation period, we identified 12 patients with Darier's disease; five males and seven females with a mean age of 17.36 years. No family history was found in eight patients. Skin lesions in the form of keratotic papules were noted in seborrhoeic areas, essentially the face (nine patients), chest and scalp. Seven patients had nail lesions. UV light exposure exacerbated the disease symptoms in seven cases. The patients were treated with topical and systemic retinoids (six cases). Conclusion Although Darier's disease has a chronic course, most patients manage to lead a relatively normal life. Treatment is usually unsatisfactory despite much progress in understanding of the underlying abnormalities in Darier's disease.

Sweet's syndrome (SS) is an acute neutrophilic dermatosis characterised by abrupt onset of fever, leukocytosis and cutaneous eruption, with dermal neutrophilia on skin biopsy. Most cases are idiopathic but SS can be associated with various affections, especially neoplastic, inflammatory and infectious diseases. The authors report the case of an SS occurring in a patient with a known rheumatoid arthritis associated with a secondary Sjögren's syndrome, with incidental finding of concurrent lymph node tuberculosis. In case of SS, an associated disease (malignant, inflammatory or infectious diseases) must imperatively be searched for, knowing that two or more of these affections can coexist.

INTRODUCTION Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasoproliferative disease of undetermined origin. It is characterized by the presence of nodular pseudo-tumors corresponding microscopically to a vascular proliferation within an inflammatory infiltrate made up of lymphocytes, macrophages, and eosinophils. The authors describe 7 cases of ALHE. METHODS The 7 cases were diagnosed over a period of 19 years (1990-2008). Clinical data and histological slides were brought from the departments of dermatology and pathology of the Rabta Hospital. RESULTS The 7 patients were 4 women and 3 men with an average age of 34.5 years. The cephalic localization was the most frequent. Lesions were solitary or multiple and formed papules or plaques of variable color. The diagnosis was based in all cases on histological findings. DISCUSSION The main disease in the differential diagnosis of ALHE is Kimura disease, but the 2 entities have several clinical and histological differences. The pathogenesis remains unclear and there is no consensus on the best treatment.