Histiocytosis X revealed by diabetes insipidus and skin lesions.

Langerhans cell histiocytosis is part of a larger group of syndromes described as histiocytoses. The disease may involve single or multiple systems including skin and nervous system. Here we report an adult case where Langerhans cell histiocytosis presented with diabetes insipidus and cutaneous ulcers.

Postvaccination morphea profunda in a child.

We report a new case of postvaccination morphea profunda (MP) in a child and discuss its different clinical presentations, prognosis, and therapy and its relationship with "solitary morphea profunda." A 2-year-old healthy girl presented with an induration of the anterior aspect of the left thigh of 9 months duration. The lesion had appeared 3 months after a third dose of diphtheria-tetanus-pertussis vaccine. Cutaneous examination showed an induration of 7 × 7 cm with an "orange peel" texture after pinching the skin. Histologic examination confirmed the diagnosis of MP. Systemic steroids (1 mg/kg/day) led to the stabilization of the lesion. After 4 months of treatment, we began the concomitant use of oral methotrexate (10 mg/wk) for 2 months. Methotrexate was then continued alone for 10 months, leading to a significant regression of the induration with no relapse.

[Treatment of psoriasis by methotrexate in the era of biotherapies: a study in 21 Tunisian patients]. / Traitement du psoriasis par méthotrexate à l'ère des biothérapies : étude chez 21 patients tunisiens.

INTRODUCTION: We present here results of methotrexate, in term of efficacy and tolerance, administrated in 21 Tunisian patients with severe psoriasis. METHODS: It was a retrospective study conducted between january 2002 and december 2009 in the department of dermatology of Charles Nicolle Hospital of Tunis. We have included 21 patients with severe psoriasis treated by methotrexate. RESULTS: Patients were 53 year-middle aged with a sex-ratio = 6. Psoriasis evolved for a mean of 10 years (1month-60 years). Patients had: plaque psoriasis (n = 18, 85.8%) with 63% body surface involvement, erythrodermic psoriasis (n = 2, 9.5%) and psoriatic arthritis (n = 1, 4.7%). Methotrexate was orally administrated at an initial dose of 5-7.5 mg/week. The maximum dose was of 7.5 mg-12.5 mg/week. Complete remission was achieved in 62% of cases and partial remission in 28.5% of cases. Haematological and hepatic toxicities were observed in 2 patients (9.5%). Mean remission period was of 14 months (3 months-3 years). Seven patients had severe relapses. CONCLUSION: Our study concluded to the efficacy of methotrexate in severe psoriasis with a high rate and long term remission, despite lower doses than those classically used in the literature.

[Not Available]. / Traitement du psoriasis par méthotrexate à l'ère des biothérapies : étude chez 21 patients tunisiens.

INTRODUCTION: We present here results of methotrexate, in term of efficacy and tolerance, administrated in 21 Tunisian patients with severe psoriasis. METHODS: It was a retrospective study conducted between january 2002 and december 2009 in the department of dermatology of Charles Nicolle Hospital of Tunis. We have included 21 patients with severe psoriasis treated by methotrexate. RESULTS: Patients were 53year-middle aged with a sex-ratio=6. Psoriasis evolved for a mean of 10years (1month-60years). Patients had: plaque psoriasis (n=18, 85.8%) with 63% body surface involvement, erythrodermic psoriasis (n=2, 9.5%) and psoriatic arthritis (n=1, 4.7%). Methotrexate was orally administrated at an initial dose of 5-7.5mg/week. The maximum dose was of 7.5mg-12.5mg/week. Complete remission was achieved in 62% of cases and partial remission in 28.5% of cases. Haematological and hepatic toxicities were observed in 2 patients (9.5%). Mean remission period was of 14months (3months-3years). Seven patients had severe relapses. CONCLUSION: Our study concluded to the efficacy of methotrexate in severe psoriasis with a high rate and long term remission, despite lower doses than those classically used in the literature.

A case of multiple epithelioid angiomatous nodules.

Cutaneous epithelioid angiomatous nodule (CEAN) is a distinct type of epithelioid vascular tumor that is usually solitary. Herein we present a 31-year-old man with multiple, rapidly growing nodules on the scalp.

Cutaneous adverse drug reactions in children. A series of 90 cases.

BACKGROUND: Cutaneous adverse drug reactions (CADR) are frequent in children. They have different clinical presentations and may be caused by several drugs. AIM: To evaluate the epidemioclinical features of cutaneous adverse drug reactions (CADR) and the different causative drugs in a Tunisian paediatric series. METHODS: We have retrospectively included 90 children (under 16 years old) with a well documented cutaneous drug reaction, seen in the Department of Dermatology of Charles Nicolle hospital of Tunis over 18 years (1991-2008). Age, gender, duration of skin disorders, type of cutaneous lesions, incriminated drugs, delay between drug consumption and eruption, validation by the national pharmacovigilance centre, treatment and outcome were recorded. RESULTS: Our patients were 6.9 year-aged (sex-ratio M/F 1.19). They had maculopapular eruption (MPE) (57.7%), acute urticaria (16.6%), fixed drug eruption (14.4%), erythema multiform (2.2%), photosensitization (1.1%) or severe cutaneous drug reactions (10%).Incriminated drugs were: Antibiotics (55.5%), non-steroidal antiinflammatory drugs (18.8%), antiepileptics (11.1%), and analgesics (5.5%). Betalactamins were the most commonly incriminated antibiotics (32 out of 50 patients; 64%). Barbiturates were the most commonly incriminated anti-epileptics (7/90 cases, 7.7%). Favourable outcome was noted in all patients, even those with severe drug reactions. CONCLUSION: MPE to antibiotics were the most common kinds of CADR in children. Drug responsibility should be based on solid criteria given the frequency of MPE of infectious origin and the frequent prescription of antibiotics in paediatric population.

Chronic actinic dermatitis: two patients with successful management using narrowband ultraviolet B phototherapy with systemic steroids.

BACKGROUND: Chronic actinic dermatitis (CAD) is a debilitating photodermatosis with characteristic clinical, histological and photobiological features (reduced minimal erythema dose: MED). Its management involves various therapeutic approaches, among them there is phototherapy. Efficacy of psoralen ultraviolet therapy (PUVA therapy) was previously demonstrated but there are no current data on the use of narrowband ultra violet B (UVB) therapy (NB-UVB) in CAD. NB-UVB has already been proven to be effective and safe in several other photodermatoses. CASE REPORTS: We report here two dark-skinned patients (skin type IV and V) with CAD, successfully treated with an incremental regimen of NB-UVB phototherapy coupled to a 3 month-course of systemic steroids (1mg/Kg/day). CONCLUSION: Our protocol of NB-UVB with steroids seems to be effective for the management of CAD with a good short term safety profile.

[Kindler syndrome: clinical and ultra-structural particularities, a propos of three cases]. / Le syndrome de Kindler, particularités cliniques et ultrastucturales à travers trois nouvelles observations familiales.

INTRODUCTION: Kindler's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia. We report three new sibling cases of this rare syndrome. CASES REPORTS: The condition was seen in three sisters aged 12, 16 and 20 years, born of a first-degree consanguineous marriage with no family history of Kindler's syndrome. The three patients presented spontaneously regressive bullous eruptions, poikilodermia of gradual onset, major cutaneous atrophy on the back of the hands and the feet, photosensitivity and gingival hypertrophy. Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage. DISCUSSION: Diagnosis of Kindler's syndrome is based upon clinical evidence. Kidler's syndrome is a well defined clinical entity. Ultra-structural studies show intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and other bullous hereditary poikilodermas.

Pigmented lesion in the inguinal region.

Pigmented Bowen disease (PBD) is a rare tumor characterized by increased melanin pigment in the epidermis or papillary dermis in addition to the typical findings of Bowen disease. We report the case of a 60-year-old woman who presented with a 6-month history of a gradually enlarging solitary dark brown plaque in her right inguinal region. Histopathology showed hyperkeratosis with parakeratosis, acanthosis, disorganization of epidermal architecture, atypical keratinocytes, and increased melanin pigment of the papillary dermis. Considering the clinical and the histological evidence, a diagnosis of PBD was established. Complete resection confirmed the diagnosis. Pigmented Bowen disease is an unusual form of squamous carcinoma in situ. Other tumors in the differential diagnosis include pigmented basal cell carcinoma and superficial spreading melanoma.

Erythema elevatum diutinum: an "idiopathic" case.

Erythema elevatum diutinum (EED) is a rare condition with an unclear pathogenesis. Initially classified within neutrophilic dermatoses, it is now considered as a leukocytoclastic vasculitis accordingly to its histopathologic pattern. Several clinical presentations as well as many associated diseases are reported in the literature. We report a new case of EED in a 58-year-old man who presented with a three-month history of plaques and nodules on the extensor surfaces of hands, elbows, knees, ankles, forearms, and buttocks. Histology showed a leucocytoclastic vasculitis, suggestive of the diagnosis of EED. Screening for an associated pathology, namely a paraproteinemia or a solid cancer, was negative. Treatment with dapsone leads to amelioration within few weeks.

Giant trichoblastoma of the scalp.

BACKGROUND: Trichoblastoma is a rare and benign adnexial tumor with characteristic histological features. It occurs on any hair folliclebearing location, and usually presents as a solitary lesion most often less than 2 cm in size. Giant trichoblastoma has been rarely reported in the literature. AIM: To report a new case of giant trichoblastoma, misleading for malignancy. CASE REPORT: A 57-year-old woman presented with a 5 cm-solitary asymptomatic nodular lesion of the scalp, of 28 years. It had been previously excised with recurrence and progressive regrowth. On examination, it was a dome-shaped, erythematous, firm, papillomatous, non infiltrated nodule. Full body work up revealed no metastases. Cutaneous biopsy concluded to trichoblastoma but failed to eliminate malignancy. After excision with secondary skin graft, histological examination confirmed the benignity with clear margins. There was no evidence of recurrence after a 5 year-follow-up period. CONCLUSION: This case illustrates a rare clinical variant of trichoblastoma with an unusual important size. This can be misleading for malignancy, but the slowly progressive course of the tumour in our patient, together with histological benignity led to the correct diagnosis. This tumour is considered as a distinct entity by some authors.

[Hydroxyurea induced-leg ulcer in polycythemia vera]. / Ulcère de jambe induit par l'hydroxyurée dans la cadre d'une thrombocythémie essentielle.

BACKGROUND: Hydroxyurea (HU) is an antineoplastic drug commonly used to treat chronic myeloproliferative disorders. Dermatological side effects are frequent and usually benign. Leg ulceration following HU therapy is less common. AIM: To describe epidemioclinical and therapeutic features of leg ulcers induced by HU. CASE REPORT: A 70-year-old woman is treated with hydroxyurea for polycythemia vera. One year later; she presented with a malleolar painful ulcer, initially healed without discontinuation of the treatment, but has been recurred 2 months later, becoming multiple and bilateral. HU has been discontinued and ulcers were completely cured. CONCLUSION: Leg ulcers induced by hydroxyurea are rare. Pathogenesis of HU-induced ulcers remains unknown and is multi factorial. Discontinuation of treatment is still the option of choice for complete recovery.

Xeroderma pigmentosum skin: an immune privilege site for tumor development.

A unique feature of the skin immune system is its proximity to cells continuously exposed to sun rays, as it is located in the interface between the body and the environment. In this study, we aimed to determine the impact of DNA damaged keratinocytes on the expression of apoptotic-related molecules, in T-cells of the inflammatory component of the tumor environment. Immunohistochemistry was performed on tissue sections derived from skin biopsies of basal cell carcinomas (BCCs) of xeroderma pigmentosum (XP) patients, non-XP patients and nevoid basal cell carcinoma syndrome (NBCCS) patients, using antibodies against B-cell lymphoma/leukemia-2 (Bcl-2), Bcl-2 associated X protein (Bax), CD95, CD3, CD8 and CD56. Our results showed significantly lower levels of expression of the antiapoptotic Bcl-2 molecule, in XP, in comparison with non-XP and NBCCS T-lymphocytes, leading to the highest Bax/Bcl-2 ratio for XP T-cells. For the CD95 receptor expression levels, there were significant differences among T-cells of the three patient subgroups as well. The higher propensity of XP T-cells to undergo apoptosis may have evolved in individual XP patients, apparently during the course of their disease, to maintain a special skin as an immune privilege site for tumors' development.

Early onset childhood cicatricial pemphigoid: a case report and review of the literature.

Cicatricial pemphigoid is an extremely rare condition in children, since only 17 cases have been reported in the English literature, so far. We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation.

Vulvoperineal Crohn's disease: response to metronidazole.

A 46-year-old woman with a medical history of chronic juvenile arthritis with bilateral prosthetic hips presented with vulvoperineal ulcerations of 3 years' duration. There was no diarrhea or recent weight loss. Cutaneous examination showed asymmetrical vulvar edema of the labia minora and labia majora with deep and linear ulcerations having verrucous borders located on the inguinocrural regions and the buttocks fold (Figure 1). On physical examination there was bilateral limited mobilization of the hips. A biopsy specimen was taken from the border of the vulvar ulceration and histologic examination showed under a hyperplasic epidermis an epithelioid granuloma with multinucleated giant cells of the dermis without caseification. Laboratory analyses and results from chest x-ray were normal. Results for Koch bacilla in the spittle, microbiologic studies (staining for microorganisms and cultures), and tuberculin intradermoreaction were negative. There was no Crohn's disease aspect on colonoscopy, and there was normal small bowel enterography. Systematic intestinal biopsies were also with normal aspect. Based on the clinical data and granulomatous histologic characteristics, the diagnosis of metastatic Crohn's disease without digestive involvement was obtained. The patient was started on metronidazole 1 g/d. After 6 months of treatment, there was an almost-complete healing of ulcerations (Figure 2). Treatment was well-tolerated.

Multiple keratoacanthoma centrifugum marginatum.

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacanthoma characterized by a progressive peripheral growth with concomitant central healing. We report here a case of multiple KCM of the lower legs in a 48-year-old man. The lesions had progressively evolved over 3 years. They were multiple asymptomatic and confluent annular plaques of 5 to 20 cm, having papulo-nodular with hyperkeratotic and crusted borders and cicatricial center. Within the centers were numerous firm and pigmented minipapules of 1 to 2 mm. The typical clinical aspect, together with characteristic histological features confirmed the diagnosis of KCM. Herein we will highlight the clinical and histological features of KCM, as well as the different effective treatments. We will also briefly discuss KCM among the other types of keratoacanthomas.

Linear IgA bullous dermatosis: the more frequent bullous dermatosis of children.

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.

Generalized peeling skin syndrome: Case report and review of the literature.

Peeling skin syndrome (PSS) is a rare form of ichthyosis with a probable autosomal recessive inheritance that exhibits superficial, painless, continual, or seasonal cutaneous exfoliation. The syndrome generally appears at birth or in infancy. We report a case of generalized PSS and provide a literature review. A 34-year-old woman reported a lifelong history of generalized and painless peeling of the skin that worsened in summer. Her parents were third degree cousins. Her twin sister and her two cousins presented with the same condition. Physical examination showed widespread superficial sheets of variable size that could be easily removed without bleeding or pain. No underlying erythema was noted. Otherwise, the patient was in good health. Histological findings showed an epidermal cleavage within the stratum corneum. The generalized form of PSS is classified into 3 types, A, B, and C, according to the classification system of Traupe and Mevorah. We have tried to classify the cases of generalized PSS already reported in the literature into one of these three types. Thirteen reported cases probably presented PSS-type A. Sixteen patients are best described as PSS-type B. Two patients exhibit PSS-type C. Fifteen reported patients had an acral form of peeling skin syndrome. The classification of the eleven remaining patients was difficult to determine. Our patient presented clinical and histological features of generalized PSS-type A.

Cutaneous, pulmonary and sinusal aspergillosis in a diabetic patient.

BACKGROUND: Cutaneous aspergillosis is rarely reported in diabetic patients. AIM: The objective of our study is to report a case of lethal disseminated aspergillosis revealed by multiples skin necroses, with pulmonary and sinusal involvement in a diabetic patient. CASE REPORT: A 60-year-old diabetic woman, presented with one month -rapidly -extensive, 1 to 10 cm skin necroses of the trunk, limbs and eyelids. Few days after her admission, she developed dyspnoea. Chest X-ray showed an interstitial and alveolar syndrome with multiple excavated anfractuous-edged-opacities. Facial CT scan showed a right orbital cellulitis with Pansinusitis. The methaminesilver stains on a cutaneous biopsy showed filamentous septate fungal hyphae with branches at right angles. The immunofluorescence with an anti-aspergillus serum was positive. The diagnosis of secondary disseminated aspergillosis to a primary pulmonary focus with cutaneous, sinusal, and upper airway's dissemination was made. The patient died despite an intravenous amphotericin B therapy. CONCLUSION: This report emphasizes the importance of evoking and seeking for a mycosis in every skin necrotic and ulcerative lesions occurring in an immunocompromised patient. The prognosis depends on the diagnosis and treatment institution delay.

[Chronic lupus erythematosus: 104 Tunisian cases]. / Le Lupus Erythémateux Chronique: 104 cas tunisiens.

BACKGROUND: Chronic lupus erythematosus is a cutaneous form of lupus erythematosus, usually involving photoexposed areas. AIM: The aim of our study is to evaluate the epidemio-clinical trends, therapeutic features and outcome of patients with chronic lupus erythematosus, through a Tunisian hospital series. We have tried to compare our results with those of other African and western series. METHODS: It is a retrospective study, concerning all patients with chronic lupus erythematosus, attending the Dermatology department of Charles Nicolle Hospital over an 11 years period. The patients' age, sex, clinical features, explorations results, treatment and evolution were recorded. RESULTS: A total of 104 patients were included. Chronic lupus erythematosus represented 0,1 % of all the dermatitis seen over 11 years. Chronic lupus erythematosus affects young women with a ratio F/M of 1,97 and an average age of 42 years. The discoid form was the most frequent clinical shape, observed in 73 % of cases (76 patients). The face was the most frequent localization of the lesions (91%). Five patients (4.8%) presented a generalized chronic lupus erythematosus. The direct immunofluorescence in sick skin was positive in 54 % of cases. Treatment included sun avoidance and oral antimalarials drugs in the majority of cases (95%). The progression from chronic lupus erythematosus to systemic lupus erythemetosus was observed in two cases (2%). CONCLUSION: Chronic lupus erythematosus is a relatively rare disorder in Tunisia, compared to other African countries, where prevalence is 7 to 10 times more frequent. As shown in our study, the most common clinical feature is the discoid form. The diagnosis can be confirmed by histopathological and direct immunofluorescence examination. Progression of chronic lupus erythematosus to systemic lupus erythematosus is possible, as observed in two of our patients. Thus, Patients with chronic lupus erythematosus should be continually followed up.