[Thrombotic skin gangrene: A rare extra-intestinal manifestation of ulcerative colitis]. / La gangrène cutanée thrombotique : une manifestation extradigestive rare de la rectocolite hémorragique.

BACKGROUND: Thrombotic cutaneous gangrene is a rare extra-intestinal manifestation of ulcerative colitis with a severe prognosis. CASE REPORT: A 35-year-old woman with a 7-year history of ulcerative colitis presented with extensive ecchymotic lesions that began a few hours earlier. On examination, she was febrile with multiple necrotic lesions. Skin biopsy showed multiple microthrombi in the dermal vessels. A diagnosis of thrombotic cutaneous gangrene was established. The patient was treated with heparin and systemic corticosteroids. The majority of cutaneous lesions showed improvement after 1 month. Thrombophlebitis of the left lower limb occurred subsequently. CONCLUSION: Thrombotic cutaneous gangrene is attributed to microvascular thrombosis, which arises from the hypercoagulability observed in ulcerative colitis. Complete blood and coagulation tests must be performed and early anticoagulation with heparin must be considered in order to prevent the progression of cutaneous infarction.

[Bowel-associated dermatosis-arthritis syndrome during ulcerative colitis: A rare extra-intestinal sign of inflammatory bowel disease]. / Syndrome arthro-cutané lors d'une rectocolite ulcéro-hémorragique : une manifestation extra-intestinale rare des maladies inflammatoires de l'intestin.

INTRODUCTION: Bowel-associated dermatosis-arthritis syndrome (BADAS) is characterized by combined pustular skin eruption and arthralgia. It may be associated with inflammatory bowel disease or bowel bypass surgery. We report a case of BADAS in a patient with ulcerative colitis. CASE REPORT: A 39-year-old woman was being treated for a severe flare-up of ulcerative colitis present over the preceding 2 months and treated with prednisone, azathioprine and cyclosporine. She was also presenting a cutaneous eruption and arthralgia that had begun three days earlier. Dermatological examination revealed profuse vesicular and pustular lesions. Biopsy specimens showed mature neutrophilic infiltrate within the dermis. A diagnosis of BADAS was made and the same treatment was maintained. Systemic symptoms were resolved but the vesicular lesions were superseded by hypertrophic scars. DISCUSSION: Bowel-associated dermatosis-arthritis syndrome consists of a vesiculopustular eruption associated with arthralgia and/or arthritis and fever, as was the case in our patient. The histological picture is characterized by abundant neutrophilic infiltrate in the superficial dermis. The clinical and histological features and the course of BADAS allow this entity to be classified within the spectrum of neutrophilic dermatoses. Treatment chiefly involves systemic corticosteroids.

Severe childhood lichen planus pemphigoides after hepatitis A vaccination.

Background: Lichen planus (LP) pemphigoides (LPP) is a very rare autoimmune bullous disorder, that is, exceptional in children. Case Report: We report a case of LP pemphigoides with severe cutaneous and mucosal involvement in an 8-year-old girl who consulted for multiple vesicular and bullous lesions associated with shiny erythematous-purple plaques. The eruption occurred 2 months after vaccination against hepatitis A virus. The diagnosis of LP pemphigoides was confirmed by histopathology and immunofluorescence examination. The patient received oral corticosteroid therapy with rapid improvement. Conclusion: To our knowledge, this is the first report of LPP following hepatitis A vaccination, among adults and children.

[Annular lipoatrophy of the ankles]. / Lipoatrophie annulaire des chevilles.

BACKGROUND: Annular lipoatrophy of the ankles (ALA) is an extremely rare form of localized lipoatrophy characterized by atrophy of subcutaneous fat. We report a case of annular atrophy of the ankles associated with a history of diabetes and Graves' disease. PATIENTS AND METHODS: A 22-year-old woman with a history of Graves' disease and diabetes consulted for annular lesions of ankles which had appeared three years earlier. She had suddenly developed erythematous annular plaques on the right ankle. One week later, the plaques had evolved towards progressive atrophic bands involving the lower third of the legs. The overlying skin was normal and muscle involvement was absent. Laboratory studies revealed an antinuclear antibody (ANA) titre of 1/640 while a skin biopsy specimen showed diffuse lobular lymphohistiocytic panniculitis rich in lymphocytes and lipophages foam cells without any evidence of vasculitis. A direct immunofluorescence microscopy study was negative. Lipoatrophy was still present after six months of chloroquine treatment (300mg per day). DISCUSSION: ALA is a rare and poorly understood condition. Based on the ten or so case reports in the literature, we attempted to define the clinical and histological profile of the disease as well as its course and its relationship with other forms of atrophic panniculitis. In particular, the onset of LAC in our patient presenting several autoimmune diseases underlines its close relationship to the condition described as "connective tissue panniculitis".

[Acute generalized exanthematous pustulosis following a spider bite: three cases from Tunisia]. / Pustulose exanthématique aiguë généralisée succédant à une morsure d'araignée : trois observations tunisiennes.

BACKGROUND: acute generalized exanthematous pustulosis (AGEP) is a severe eruption induced in most cases by medication; more rarely, it is associated with viral infection, food allergens or toxins. The literature contains only very rare reports of AGEP caused by a spider bite. We describe three cases of AGEP in Tunisian women following a spider bite. CASE REPORTS: three Tunisian women were hospitalized in the dermatology department during the summer months presenting acute febrile pustulosis and rash occurring after a spider bite. The diagnosis of AGEP was confirmed with certainty based on the EuroSCAR score. Two patients presented marked eosinophilia. The outcome was favourable in all cases after symptomatic treatment. DISCUSSION: the factors militating in favour of spider-bite origin in these three patients were: (1) the presence of skin lesions characteristic of a spider bite; (2) the chronology of events, with eruption occurring 24 to 48 hours after the bite; and (3) the exclusion of the common causes of AGEP and the presence of marked eosinophilia in two of the cases. CONCLUSION: Our cases and the few cases published in the literature indicate that spider bites may be added to the list of the possible causes of AGEP.

[Clinical and genetic characteristics of Buschke-Fischer-Brauer's disease in a Tunisian family]. / Etude clinique et génétique de la kératodermie palmoplantaire de Buschke-Fischer-Brauer dans une famille tunisienne.

BACKGROUND: Punctate palmoplantar keratoderma (PPPK), or Buschke-Fischer-Brauer's disease, is a rare form of genodermatosis with autosomal dominant transmission and with variable penetrance. Its molecular basis remains unknown. Two loci were found to be linked to this disease: one on 15q22 and the other on 8q24. We report the clinical and genetic characteristics of PPPK in a Tunisian family. PATIENTS AND METHODS: A Tunisian family with PPPK was identified through a proband. As far as possible, history taking, physical examination, histopathological tests and blood sampling for DNA extraction were carried out for each patient. RESULTS: Seventeen patients were included in this study. Age ranged from 15 to 81 years with a sex-ratio of 3.2 m/f. Lesions appeared between the ages of 10 and 65 years and at a mean of 28 years. Clinically, lesions ranged from few keratotic papules on the palms to coalescence of lesions in plaques over palmar and/or plantar surfaces. Hyperhydrosis, hypopigmented macules and nail dystrophy were frequently associated. In all patients, histopathological examination revealed thickening of the epidermis with compact orthohyperkeratosis overlying a small and sharply demarcated area of depressed epidermis. Mechanical measures and keratolytic ointments proved non-beneficial. Genotyping for chromosomes 8 and 15 as well as LOD scores confirmed genetic linkage with the suspected locus on chromosome 15q, with the interval of the locus in question reduced to 3.26 Mb. This region is flanked by markers D15S987 and D15S153. CONCLUSION: Our study of this family confirmed the classical characteristics of KPP-BFB as well as demonstrating several associated clinical signs of which the significance will be determined in subsequent studies. Further screening studies to identify mutated genes in the region of interest will help us to understand the molecular basis of this disease and hopefully to propose suitable treatment.

[Ghost cell odontogenic carcinoma of the mandible]. / Carcinome odontogénique à cellules fantômes de la mandibule.

INTRODUCTION: Ghost cell odontogenic carcinoma is a rare malignant tumor that occurs both in the mandible and maxilla. It has a variable non-specific clinical and radiological appearance. The authors report a mandibular case. CASE REPORT: A 89-year-old man consulted for swelling of the left lower mandible and gums having developed over the previous 8 months. The lesion was 6cm long, ulcerative and budding, bleeding on contact. Radiological assessment revealed blurred contour osteolysis with extension to the mouth floor and sub-mandibular compartment. Histological examination of the surgical piece supported the diagnosis of ghost cell odontogenic carcinoma. The tumor recurred 2 months later and the patient died 6 months after surgery. DISCUSSION: Ghost cell odontogenic carcinoma is a rare lesion. The patient was very old. Clinical and radiographic signs were unspecific. Histopathology proved the diagnosis. Evolution was unpredictable due to the wide spectrum of growth patterns. Extensive radical surgery is mandatory due to the high risk of recurrence.

[Thyroid involvement in Wegener's granulomatosis: a case report]. / Atteinte thyroïdienne au cours de la granulomatose de Wegener : à propos d'un cas.

Wegener's granulomatosis is a systemic necrotizing vasculitis affecting small to medium-sized vessels, characterized by involvement of the upper airways, lungs and kidneys. Endocrine involvement, particularly the thyroid gland is extremely rare. We report a 56-year-old woman presenting a limited form of Wegener's granulomatosis with the following clinical manifestations: sinusitis, pulmonary nodules, diabetes insipidus and a cold thyroid nodule. A right hemithyroidectomy was performed and the histopathological examination revealed a necrotizing vasculitis surrounded by granulomatous lesions. She was treated by corticosteroids without immunosuppressive therapy with a favourable outcome.

Nasal NK/T-cell lymphoma in the paediatric population. Two case reports.

Nasal natural killer (NK)/T-cell lymphoma is an uncommon disease, and only a few paediatric cases are found in the literature. We report 2 cases of NK/T-cell lymphoma occurring in children. The primary site was the the nasal cavity in the first case (5-year-old girl) and the tonsil in the second case (4-year-old boy). Histological study showed a polymorphic neoplastic infiltrate with an angioinvasive pattern and T-cell phenotype. Ebstein-barr virus infection was established by in situ hybridization. Both underwent an aggressive chemotherapy regimen. The outcome was favorable for the 4-year old boy; however, the 5-year-old girl died two months after diagnosis.

[Eighteen cases of craniofacial fibrous dysplasia]. / Dix-huit cas de dysplasie fibreuse craniofaciale.

INTRODUCTION: Fibrous dysplasia or Jaffe Lischtenstein's disease is sporadic and rare bone affection, mainly observed in young adults. It is characterized by a bone maturation disorder that can affect one or several bones. Fibrous dysplasia is relatively rare in the craniofacial region, (only 20% of all locations). The authors analyzed the epidemiological, anatomoclinical and evolutive features of fibrous dysplasia in a Tunisian population. MATERIAL AND METHOD: The authors retrospectively studied 18 cases of craniofacial fibrous dysplasia, diagnosed at the pathology department of the Sousse F. Hached hospital, beween1990 and 2005. RESULTS: Most craniofacial dysplasia cases were monostotic (94% of cases). One case was polyostotic. The maxilla and the mandible were the most frequent locations (83.3%). The patients' mean age was 28.6 years, ranging from 6 to 30 years. The sex ratio was 3.5 in favor of women. Follow-up ranged from one month to 10 years with an average of 26.4 months. One case of sarcomatous transformation was observed. DISCUSSION: The authors noted epidemiological discrepancies compared to what was usually reported, especially concerning the strong female predominance, the frequency of maxillary and mandibular locations and the predominance of monostotic presentations. Larger series are needed to validate these observations.

[Laryngeal cryptococcosis revealing AIDS: a case report]. / Cryptococcose laryngée révélatrice d'un SIDA: à propos d'une observation.

The cryptococcal infection of the larynx is uncommon. We present a case of a 65-year-old woman who was seen because of chronic hoarseness. Direct laryngoscopy revealed an infected lesion of the laryngeal vestibule. Histologic examination showed yeasts surrounding by capsules allowing a diagnosis of laryngeal infection by cryptococcus neoformans. The patient had also meningitis and pulmonary localisation. We have undergone a risk factors research and this results in finding out that HIV serology was positive. She received a treatment with amphotericin B followed by fluconazole during 6 months with complete recovery of her laryngeal infection. The patient died 11 months after the AIDS diagnostic.

Lymphatic spread in an immunocompetent adult.

Cutaneous tuberculosis with lymphatic spread is rarely described. We report the case of a woman aged 38, immunocompetent, working in a fruit sorting center, who consulted us for ulceration of the left third finger that had persisted for four months, following a prick from a prickly pear. Physical examination revealed a nodular, erythematous, and ulcerative lesion of the left third finger, associated with inflammatory subcutaneous nodules arranged in a line along the ipsilateral upper extremity and an ipsilateral axillary lymph node. Laboratory tests and chest X-ray were normal. Pathological examination revealed granulomatous chronic inflammation without necrosis. PCR detected DNA from Mycobacterium tuberculosis. No extracutaneous tuberculosis locations were detected. A tuberculous chancre by direct inoculation was therefore diagnosed. The patient received quadruple therapy (rifampicin + isoniazid + pyrazinamide + ethambutol) for 2 months, followed by a combination therapy based on isoniazid and rifampicin for 6 months. Marked regression of the cutaneous lesions occurred after 1 month of first-line therapy. A tuberculous chancre is a rare form of cutaneous tuberculosis, with possible lymphatic spread, in immunocompromised patients but also in immunocompetent children and young adults.

Dyschromatosis universalis hereditaria: two cases.

Dyschromatosis universalis hereditaria is a rare genodermatosis reported initially and mainly in Japan. However, subsequent cases have been reported from other countries. We describe two Tunisian cases of dyschromatosis universalis hereditaria in a 3-year-old and a 3-month-old girl. They presented to our department with asymptomatic progressive mottled pigmentation over the trunk and limbs, which had been noted since birth and had become more noticeable with age. Palms and soles were also affected in the first case. The two patients did not have any systemic or other cutaneous illness. They were born to healthy, second-degree consanguineous parents (case 1) and non consanguineous parents (case 2), following an uneventful pregnancy. No family members had a similar appearance. Physical examination revealed numerous, generalized, hyperpigmented macules interspersed with spotty de-pigmented macules. Hair, nails, teeth, and mucosae were normal. Systemic examination did not reveal abnormalities. Histological exam revealed basal layer hypermelanosis with pigmentary incontinence in some areas. So based on those findings a clinical diagnosis of DUH was made and the patients were followed up in our department for periodic general evaluation of their skin. After a follow up of 12 months, the first child didn't develop other lesions, but palms and soles were also involved in the second case.