Mucosal leishmaniasis of the lip: an emerging clinical form in Tunisia.

BACKGROUND: Mucosal leishmaniasis (ML), which mostly occurs in the New World, is mainly associated with Leishmania braziliensis. Primary lip ML is very rare in the Mediterranean basin and particulary in Tunisia despite the endemicity of both cutaneous and visceral leishmaniasis in this area. OBJECTIVES: To highlight a recent emergence of primary lip ML in Tunisia, to describe its epidemiological and clinical features and to identify the causative Leishmania species. METHODS: Epidemiological, clinical and therapeutic data of 10 cases presenting a ML of the lip were collected. Diagnosis confirmation of leishmaniasis was obtained by microscopic examination of Leishmania parasites in Giemsa stained smears of the lesion sampling and in cutaneous biopsies. Polymerase chain reaction (PCR) detecting Leishmania DNA directly from dermal scraping was also performed for diagnosis and species identification. RESULTS: Seven men and three women with lip ML were diagnosed during the last 6 years (2008-2013). The mean age was 29.7 years. Clinical presentation was characterized by an infiltrated and ulcerated plaque leading to macrocheilitis involving the upper lip in eight cases and the lower lip in two cases. Mean diagnosis delay was 6.9 months. PCR identified L. infantum in seven cases and L. major in two cases. Seven patients received intramuscular injections of meglumine antimoniate (MA) and three patients received both MA intralesional injections of MA and cryotherapy. A clinical remission was rapidly observed in all cases (on average in 2.2 months). CONCLUSIONS: Primary lip ML is emerging in Tunisia. Macrocheilitis of the upper lip is the main clinical presentation. PCR revealed more sensitive than direct examination in the diagnosis of such form (P < 0.01). Leishmania infantum was the most identified species (7 cases) while L major was involved in only two lesions. A benign local evolution and a rapid recovery were observed in all cases after MA treatment.

Bowen's disease: epidemiological and clinical study of 9 Tunisian cases.

BACKGROUND: Bowen's disease (BD) is a form of in situ SCC, characterized by chronic and progressive course, with low potential for invasive malignancy. AIM: To assess epidemiology and clinical features of BD in a Tunisian cohort. METHODS: A retrospective study of 9 cases of BD managed in a Tunisian dermatology department. RESULTS: There were 7 males (77.8%) and 2 females (22.2%). The mean age of patients was about 68.8 years (46-89). Lesions were solitary in 7 cases and occurred in various sites: face (1 patient), trunk (2 patients), limbs (6 patients). The mean diameter of the tumour was about 3.4 cm. Lesions presented clinically as an enlarging well demarcated erythematous plaque with irregular borders and crusted or scaling surface. Histological examination showed in all cases abnormal keratinocytes with disordered maturation and loss of polarity replacing the epidermis in its whole thickness. The main treatment was surgery (N=5). Only one patient had radiotherapy (case 1). Outcome was mentioned in 2 patients who remained free from recurrence respectively after a follow-up of 1 and 12 years. CONCLUSION: Our series outlines epidemiological and clinical features of BD in Tunisia through a small but representative sample. As in the literature, this condition prevailed mainly over 60 years. In our study, BD occurred predominantly in men and affected nonexposed sites in 8 cases. This profile is uncommon in a sunny country in Tunisia, in the absence of other aetiological agents.

A solitary pilar leiomyoma of the trunk.

Smooth muscle tumours arising in the skin are divided into angioleiomyomas, genital leiomyomas and pilar leiomyomas. Limited data about solitary leiomyoma are available in the literature. We herein report a case of a 66-year-old man who presented to our department with a slowly progressing cutaneous tumour of the right scapular area that had developed over the past 12 years. Histopathological and immunohistochemical results were consistent with the diagnosis of pilar leiomyoma. Pilar leiomyoma is a benign smooth muscle tumour arising from arrector pili muscle. Tumours can be painful from compression of cutaneous nerves or because of fibre contraction within the tumour in case of cold weather or emotional stress. This case is noteworthy as the piloleiomyoma was solitary, located on the trunk and had an unusual nipple aspect.

Nodular hidradenoma in a 19-year-old woman.

Hidradenomas are rare adnexal tumors. Recently, two groups have been individualized: those with eccrine differentiation called poroid hidradenoma and those with apocrine differentiation called clear-cell hidradenoma or nodular hidradenoma. Herein we report a case of 19-year old woman with a nodular hidradenoma of the scapular region. Our report highlights the clinic-pathological characteristics of these tumors and emphasizes the benefit of complete local excision to prevent risk of recurrence and possible malignant potential.

Hailey-Hailey disease in Tunisia.

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.

Supernumerary nipples in association with Hailey-Hailey disease in a Tunisian family.

Supernumerary nipples (SNs) or polythelia are developmental abnormalities of breast tissue. They are located along the embryonic mammary lines. Polythelia usually occurs as a sporadic abnormality, although familial aggregation has been occasionally reported. Hailey-Hailey disease is a rare autosomal genodermatosis characterized by disturbed keratinocyte adhesion. These cutaneous disorders have been described in correlation with many other abnormalities. We report here the association of Hailey-Hailey disease and supernumerary nipples in a Northern Tunisian family. To our knowledge, this is the first report of such a clinical association.

[Primary Leishmania infantum MON-80 endonasal leishmaniasis in Tunisia]. / Leishmaniose endonasale primitive à Leishmania infantum MON-80 en Tunisie.

BACKGROUND: Mucocutaneous leishmaniasis is endemic in Central and South America. It causes massive mutilating and disfiguring lesions and can lead to destruction of facial structures. In Tunisia, leishmaniasis of the mucous membranes is rare, usually developing as a complication of cutaneous leishmaniasis via direct extension. We report the first case in Tunisia of isolated and primary nasal leishmaniasis. CASE REPORT: A 70-year-old man with a history of nephrectomy for renal lithiasis was seen with a painless nodule that had been present for one month. The latter was erythematous, polypoid and firm, with a diameter of 2 cm, and was situated in the right endonasal mucosa. The diagnosis of leishmaniasis was confirmed by histological and direct examinations revealing high numbers of amastigotes of Leishmania. Culture of the offending organism in NNN medium and isoenzymatic characterization resulted in identification of MON-80 Leishmania infantum leishmaniasis. The outcome was good with treatment, and the nodule was deflated after six months. DISCUSSION: There have been few reports of similar cases of primary and isolated mucosal leishmaniasis caused by Leishmania infantum. Our case is also unusual in that zymodeme MON-80 is only rarely a cause of Mediterranean leishmaniasis.