[Wells syndrome misdiagnosed as bacterial cellulitis]. / Syndrome de Wells mimant une cellulite bactérienne : un piège diagnostique et thérapeutique.

Wells syndrome (WS), also called eosinophilic cellulitis is a rare inflammatory skin disease with about 200 cases reports in the literature. It is part of eosinophilic dermatoses and presents a diagnostic challenge. We report a case of WS presenting as bacterial cellulitis. A 59 year-old woman was referred to our department with a 10-day history of a rapidly extending erysipelatoid rash of the left upper limb, non-responding to oral antibiotics. Upon physical examination, erythema with swelling and blisters were found on the left upper limb with urticarial-like lesions on the breast, the belly and on the inner thighs. The patient was admitted with presumed bacterial cellulitis and was treated with intravenous ampicillin. Blood count showed raised eosinophils (2050/ul) along with a biological inflammatory syndrome. Biopsies were performed on both bullous and urticarial-like lesions showing perivascular and interstitial inflammatory infiltrate, made essentially of eosinophils with flame figures. The diagnosis of WS was made and the patient received local and oral corticosteroids (0,5mg/kg/d) with good evolution. The rarity and clinical manifestations of WS makes it sometimes difficult to differentiate between a WS and a bacterial cellulitis. However, biological and histological findings can help the clinician make the differential diagnosis between these two diseases for a better management.

DNA variation and polymorphism in Tunisian plum species (Prunus spp): contribution of flow cytometry and molecular markers.

Plums (Prunus spp) are among the most important stone fruit crops in the world. European (Prunus domestica) and Japanese (Prunus salicina) plums are characterized by different levels of ploidy. Because genetic variability is the prerequisite for any plant-breeding program, we aimed to establish the taxonomic status of Tunisian plums and study their genetic variability. The nuclear DNA content of 45 wild and cultivated Tunisian plums was determined by flow cytometry. Two arbitrary primers (AD10, AD17) were used to elaborate SCAR markers useful to identify plum species. Three wild trees, Zenou 1, Zenou 6, and Zenou 3, which had 2C nuclear DNA contents of 1.99, 2.05, and 2.13 pg, were shown to be hexaploid (2n = 6x = 48), whereas the others were diploid (2n = 2x = 16). These results suggest that the three hexaploid wild plums belong to Prunus insititia, and the others belong to Prunus salicina. No SCAR markers were revealed using the AD10 and AD17 RAPD primers in relation to the ploidy of plums. We note also that AD17 primer appears to be the most informative concerning the genetic diversity. Morphological and pomological traits revealed similarity between introduced and Tunisian plum cultivars. Despite the significant morphological differences found, all the cultivars studied belong to P. salicina. The information obtained in this analysis provided on local plum genetic resources will be helpful to establish a core collection, to evaluate genetic diversity, and to initiate an improvement and selection program.

Syringocystadenoma papilliferum: Unusual location.

Syringocystadenocarcinoma papilliferum (SCAP) is rare. We describe a 55-year-old man with a partially eroded lesion on the trunk that developed over one year. Histopathological examination revealed syringocystadenoma papilliferum. A wide excision was performed to remove the tumor. The patient has been well without relapse or metastasis for 8 months.

[Familial histoid leprosy]. / Lèpre histoïde familiale.

BACKGROUND: Histoid leprosy is a very particular and rare anatomoclinical form of lepromatous leprosy. Familial cases are extremely rare. We report two new cases of histoid leprosy in a sister and brother born of a non-consanguineous marriage. PATIENTS AND METHODS: We report the case of a sister and brother, aged 21 and 25 years respectively, who were admitted to the National Leprology Centre for multibacillary leprosy. Questioning revealed the existence of family contact with an uncle and two maternal cousins, all of whom had histoid leprosy. On clinical examination, multiple erythematous papules and nodules were noted, some of which were umbilicated while others were ulcerated. Palpation also revealed subcutaneous nodules on the thighs and forearms. No neurological disorders were found in the two patients. Histopathological analysis of cutaneous biopsy samples of nodular lesions for the two patients showed dermal-hypodermic nodular infiltrates comprising spindle cells with a few macrophages. Ziehl-Neelsen staining showed numerous alcohol-acid resistant bacilli (BAAR) without globi. Both patients were placed on the multi-drug regimen recommended by the WHO for multibacillary leprosy. An extremely favourable therapeutic response was obtained after 3 months of treatment. The patients had presented no relapse of erythema nodosum leprosum after two years of follow-up. CONCLUSION: Histoid leprosy is a clinical entity that must be clearly differentiated through its clinical and histopathological features, which distinguish it from polar lepromatous leprosy. There may be a genetic predisposition to this clinical leprosy form, but this requires confirmation by genetic studies.

[Idiopathic granulomatous mastitis with favorable outcome with medical treatment]. / Mastite granulomateuse idiopathique d'évolution favorable sous traitement médical.

Granulomatous mastitis is a rare inflammatory disorder. Its etiology remains unknown. We report a 42-year-old female who presented with an idiopathic granulomatous mastitis. Outcome was favourable with corticosteroids. The disease course of this entity is unpredictable and a consensual treatment is difficult.

[Suppurations of the perineal and gluteal region: An aetiological study of 60 cases]. / Suppurations périnéofessières : étude étiologique de 60 cas.

BACKGROUND: The gluteal region is a complex area of the body that may be the seat of several diseases with dermatological manifestations. The purpose of this series was to study the aetiological profile of suppurative lesions of the gluteal region in patients in Morocco. PATIENTS AND METHODS: A retrospective study was carried out on patients presenting suppuration of the gluteal region hospitalised in our department between 1987 and 2008. The epidemiological, clinical and aetiological data for 60 cases were reviewed. Skin biopsies with bacterial, fungal and histological studies, as well as bacterial and fungal cultures of pus and anorectal examination, were performed for all patients. RESULTS: We examined the files for 44 males and 16 females; mean age was 44 years (range: 13 to 70 years) and the average duration of illness was 6.5 years. The various aetiologies were as follows: hidradenitis suppurativa (27 cases), cutaneous tuberculosis (10 cases), Crohn's disease (nine cases), phlegmon-carbuncle (eight cases), tumours (four cases) and deep mycosis (two cases). Medical and surgical treatments were aetiological. DISCUSSION: There are various aetiologies of suppurations of the gluteal region. In Morocco, hidradenitis suppurativa, cutaneous tuberculosis and Crohn's disease are the most frequent causes. Multidisciplinary management is essential.

Cystadenome papillaire de l'epididyme: un nouveau cas

Le cystadenome papillaire de l'epididyme est une tumeur paratesticulaire benigne rare. Se presentant comme une masse epididymaire uni ou bilaterale. Son association avec le syndrome de Von Hippel-Lindau est frequente; en particulier dans les lesions bilaterales. Nous rapportons l'observation d'un patient age de 36 ans; qui presentait depuis un an des douleurs scrotales gauches; une grosse bourse chronique; sans fievre ni signes fonctionnels urinaires. L'examen avait mis en evidence une masse testiculaire dure; irreguliere et indolore sans adenopathies inguinales ni masse abdominale. L'echographie scrotale avait montre une masse testiculaire gauche solide hypoechogene bien limitee de 3 x 2;5 x 2;2 cm. Les marqueurs tumoraux etaient normaux (BetaHCG : 2 UI/j; AlphaFoetoProteine : 2;94 UI/l). La masse testiculaire a ete exploree a travers une incision inguinale gauche. A la palpation; c'etait une tumeur testiculaire dure. Une orchidectomie gauche a ete realisee. L'examen anatomopathologique de la piece d'exerese avait conclu a un aspect morphologique et immunohistochimique d'un cystadenome papillaire sereux borderline paratesticulaire sans signe d'invasion. A travers notre observation et les donnees de la litterature; nous proposons de mieux definir le diagnostic clinique et anatomopathologique ainsi que le traitement de ces tumeurs testiculaires rares

[Neuroendocrine carcinoma with large cells of the breast: case report and review of the literature]. / Carcinome neuroendocrine à grandes cellules du sein : à propos d'un cas et revue de la littérature.

Neuroendocrine carcinoma with large cells is a slightly different tumor from the high rank of malignity. We report a case of breast localization in a 28-year-old patient. It is a locally advanced classified T4dN1M0 tumor that required neoadjuvant chemotherapy. The clinical answer was 75% of the level of the tumor. A standard surgery mastectomy with axillary lymph node dissection was realized, followed by external radiotherapy. The anatomopathologic and the immuno-histochemical study of the operational part confirmed the diagnosis of neuroendocrine carcinoma with large cells expressing the progesterone receptor. The patient is subjected to adjuvant hormonal treatment. After a 12 months retreat, a complete remission is maintained. Considering the scarcity of neuroendocrine carcinoma with large cells of the breast, the therapeutic standard is not yet available and the forecast remains difficult to determine.

[Fatal progression of Bannayan-Zonana syndrome]. / Syndrome de Bannayan-Zonana d'évolution fatale.

Bannayan-Zonana syndrome is a rare entity characterized by macrocephaly and multiple soft tissue and visceral hamartomas and lipomas. Knowledge of this syndrome is important given the risk of sometimes fatal bleeding and visceral neoplasia in adulthood. We report on a new case, original in its sporadic occurrence and rapidly fatal progression.

[Intertoe space cutaneous epidermoid carcinoma]. / Carcinome épidermoïde interorteil.

BACKGROUND: Localisation of epidermoid carcinoma in the intertoe space is very rare. We report a case occurring in a setting of chronic intertoe intertrigo. CASE: A 66 year-old woman presenting intertoe intertrigo for 30 years consulted for painful ulceration of the 3rd intertoe space of the left foot present for 4 years and refractory to antifungal treatment. The examination revealed ulceration with infiltrated edges occupying the entire 3rd intertoe space, with no satellite adenopathy. Histological examination of the skin biopsy sample revealed epidermoid carcinoma. Wide excision of the lesion was performed with a safety margin of 5 mm, with removal of the 3rd and 4th left toes. The outcome was favourable with no relapse after 12 months. DISCUSSION: A single publication reported a series of 22 cases of intertoe epidermoid carcinoma in which the key aetiological feature of tumour was continual maceration. In this patient, maceration was maintained by a number of factors, including cultural factors: ritual ablutions performed 5 times daily, with no drying of the intertoe spaces, and domestic cleaning involving large quantities of water several times weekly.

[Fahr syndrome associated to hypoparathyroidy, revealed by dermatologic manifestation]. / Syndrome de Fahr secondaire à une hypoparathyroïdie à révélation dermatologique.

BACKGROUND: Fahr syndrome is defined by the presence at the brain base of strio-pallido-notched non-arteriosclerotic bilateral and symmetric calcifications. We report an observation of Fahr syndrome revealed by a growing pyodermitis in direct relation with hypocalcemia. OBSERVATION: A 17 year-old girl was admitted to the Dermatology Department for a growing pyodermitis of the large folds. The patient was known to be epileptic and had undergone surgery for a left eye cataract a year before. Microbiologic and mycologic examinations of the pus and of the cutaneous fragments were negative. During hospitalization, the patient had an attack of tetany, a laryngeal spasm and a generalized erythema thrust studded with pustules. The phosphocalcic assessment showed severe hypocalcemia and hyperphosphoremia. Parathormone serum rate was decreased. The brain tomodensitometry conveyed bilateral calcifications of the central gray nucleus. The diagnosis of a growing amicrobial pustulosis secondary to an idiopathic hypoparathyroidism and responsible for the Fahr syndrome was maintained. Treatment was composed of calcium (2 g/day) and vitamin D3. The evolution was favorable with a relapse at 2 years. DISCUSSION: We report an idiopathic hypoparathyroidism associated with Fahr syndrome conveyed by growing amicrobial pustulosis. We discuss the dermatologic manifestations of Fahr syndrome associated with hypoparathyroidism and the physiopathologic mechanisms of the dermatologic lesions during hypocalcemia.