[Bilateral pseudotumoral proptosis as presenting sign of orbital hyper-IgG4-related disease]. / Exophtalmie bilatérale pseudo-tumorale révélant un syndrome hyper-IgG4 orbitaire.

INTRODUCTION: Hyper-IgG4 syndrome is a rare cause of bilateral proptosis. It must always be considered after ruling out orbital lymphoma. CASE REPORT: We report a case of progressive bilateral proptosis for 4 years in a 34-year-old man. Orbital MRI showed an infiltrative process extending to the orbital fat, extraocular muscles and lacrimal glands. Lacrimal gland biopsy with immunohistochemical study showed a lymphoplasmocytic infiltrate rich in IgG4 and fibrosis. The diagnosis of orbital hyper-IgG4 syndrome was suggested. The patient responded well to systemic steroid treatment. DISCUSSION: Orbital hyper-IgG4 syndrome manifests most often as pseudo-tumoral bilateral proptosis. Elevated IgG4 levels are neither sensitive nor specific. Biopsy with immunohistochemical study is the key to diagnosis. Systemic steroid treatment is the gold standard, but recurrences may occur.

[Extraneural soft tissue perineurioma of the nose]. / Périneuriome extraneural des tissus mous localisé au nez.

BACKGROUND: Perineuriomas are tumours derived from the perineurial cells of the neural sheath. Soft tissue perineuriomas are rare. Herein, we report a case of perineurioma localized to the nose. CASE REPORT: A 24-year-old adult presented with a cutaneous nodule 1cm in diameter localized on the nose. Histologically, the tumour corresponded to a well-circumscribed fusocellular dermal proliferation organized in a storiform pattern. After surgical removal, the lesion did not recur. COMMENTS: Extraneural soft tissue perineurioma is usually a benign nerve sheath tumour composed mainly of perineurial cells. Our case occurred in a 24-year-old Caucasian adult. The tumour generally presents as a unique cutaneous nodule corresponding histologically to a well-delineated dermal fusocellular proliferation organized in a storiform pattern that stains positive for EMA and negative for S100. The tumour is usually localized to the trunk and extremities. Location on the nose as in our patient has been rarely reported. Surgical removal of the tumour is the treatment of choice.

[Extensive Dowling-Degos disease following long term PUVA therapy]. / Maladie de Dowling-Degos étendue après PUVA thérapie prolongée.

BACKGROUND: Dowling-Degos disease is a rare and benign inherited dermatosis. PATIENTS AND METHODS: A 53-year-old woman presented with generalized histologically confirmed Dowling-Degos disease revealed 8 years after psoralen photochemotherapy (PUVA) for psoriasis. This presentation was special in terms of its considerable spread as well as the absence of comedone-like and punctate scars. DISCUSSION: Dowling-Degos disease is a reticulate pigmentary disorder of the flexures associating prominent comedone-like lesions and pitted scars. Diagnosis is based on clinical and histopathological examination, which allows this entity to be differentiated from other reticulate pigmentary disorders. A literature review failed to provide any indication that PUVA therapy either aggravates or reveals Dowling-Degos disease, a finding which we feel merits mention.

Isolated genital annular lichen planus.

Annular lichen planus is a rarely reported variant of lichen planus (LP). Although genital lesions are frequent in patients with LP, isolated genital LP is rarely reported. We present a case of a 29-year- -old circumcised man with an asymptomatic annular lesion of the penis. Histopathological features were consistent with LP. Topical clobetasol was prescribed, with clinical improvement. It is important to consider annular LP among the possible diagnoses of individual annular genital lesions.

[The myofibroblastic inflammatory tumours: A fortuitous association or an unknown postpartum complication]. / Les tumeurs myofibroblastiques inflammatoires : association fortuite ou complication peu connue du post-partum ?

The inflammatory myofibroblastic tumour has clinical, biological or histological features sometimes misleading with a septic condition. Presenting symptoms are variable and arising circumstances remain obscure. We report three cases occurring in a postpartum context. The first patient, a 28-year-old female, had left psoitis with a sepsis the first day postpartum in relation with an inflammatory myofibroblastic tumour of the meso-ovary. The second patient, a 40-year-old woman, had a hepatic inflammatory myofibroblastic tumour revealed by a ruptured sub-capsular haematoma of the liver in the forth day postpartum. The third patient, a 32-year-old woman, had a pulmonary inflammatory myofibroblastic tumour, diagnosed 5 months after a delivery and which recurred 10 years after surgical treatment. These cases illustrate the difficulty to diagnose inflammatory myofibroblastic tumour, particularly in postpartum.

Adenolipoma of the skin.

Adenolipoma of the skin (ALS) is an uncommon histological variant of lipoma, characterized by the presence of normal eccrine sweat glands inside the fat proliferation. A 32-year-old woman presented to our department with a slow-growing, painless subcutaneous soft tumour located on the upper part of the right thigh. Microscopically, there was lobulated adipose tissue proliferation with well-differentiated eccrine glands and ducts in the periphery and centre of the nodule. These features were suggestive of ALS. ALS is a rare microscopic variant of cutaneous lipoma having similar clinical features to lipoma. The most frequent locations of this tumour are thighs (as in our patient), shoulders, chest and arms. Histologically, the tumour is composed of lobulated adipose tissue with larger and more prominent lobules than those in normal subcutaneous adipose tissue. A well-developed capsule may also be identified. Eccrine glands and ducts, without proliferative changes, are well-differentiated within the adipose tissue. Differential diagnosis of adenolipoma includes the common lipoma and its variants, skin tag and other hamartomatous lesions, such as nevus lipomatosus superficialis, and the lipomatous variant of eccrine angiomatous hamartoma.

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.

[Parotid mucormycosis: A new case and review of the literature]. / Mucormycose parotidienne : une nouvelle observation et revue de la littérature.

INTRODUCTION: Mucormycosis is a rare, devastating, fungal infection, which disproportionately affects non-controlled diabetic patients, notably during ketoacidosis. The authors report the case of cervical mucormycoses with a particularly favorable evolution in diabetic woman. REPORT: A 54-year-old woman, type 2 diabetic, had presented a left lateral cervical mass. The diagnosis was confirmed by histological examination. She was treated with Amphotericin B with favorable evolution. CONCLUSION: The mucormycose is a rare infection. The treatment is medical and surgical. The prognosis is severe with an overall mortality rate of 40%.

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.

Atypical clinical appearance and localization of trichilemmoma. a case report.

Trichilemmoma is a benign cutaneous tumor that shows characteristics of differentiation similar to the outer hair sheath. We report the case of a woman presenting with a nodular tender mass of the back that was diagnosed as an isolated trichilemmoma. Several lines of evidence suggest that trichilemmoma should be considered in the differential diagnosis of any indistinct facial papule. This report documents a non-facial example of trichilemmoma. Atypical clinical appearance and localization of this neoplasm in our patient suggest that only histological findings are specific of this tumor.