Alopecia areata in Tunisia: epidemio-clinical aspects and comorbid conditions. A prospective study of 204 cases.

BACKGROUND: Alopecia areata (AA) is an autoimmune condition that usually presents as patchy, nonscarring hair loss. Autoimmune disorders and atopy are reported as comorbid conditions. We aimed to investigate the demographics, clinical characteristics, and associations of AA in Tunisian patients. METHODS: Demographic data, pattern of alopecia, age of onset, and associations were evaluated in 204 patients from January 2012 to June 2016. RESULTS: Two hundred and four cases of AA were seen. The male to female ratio was 0.68. The mean age at presentation was 23 years old. Positive family history was noticed in 22.1% of patients. Personal history of atopy was associated with AA in 18.1%. Associated autoimmune diseases were thyroid disorders (12.7%), vitiligo (1.5%), psoriasis (three cases), type 1 diabetes (two cases), autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome (two cases), lichen sclerosus atrophicus (one case), and pemphigus vulgaris (one case). Patchy AA was the most common manifestation (49.5%) followed by alopecia universalis (27.5%), alopecia ophiasis (12.7%), and alopecia totalis (10.3%). Nail changes consisting of pitting, trachyonychia, and longitudinal ridging were reported in 24.8%. AA patterns were more severe in females (P = 0.049). Severe forms showed more persistent disease duration (P = 0.005), earlier onset (P = 0.001), and more recurring episodes (P = 0.002) and were significantly associated with nail involvement (P < 0.001). CONCLUSIONS: Our study aimed to review epidemio-clinical characteristics and comorbid conditions of AA in Tunisian patients. More severe cases with a pejorative value of early-onset AA, long disease duration, and nail involvement were seen in our study.

[A particular type of cicatricial Pemphigoid with unique IgA deposit]. / Forme particulière de Pemphigoide cicatricielle à dépôt unique d'IgA.

Cicatricial Pemphigoid is a subepithelial bullous dermatosis which essentially involves the mucous membranes with cicatricial evolution We report the case of a 66-year old patient hospitalized with erosive gingivitis associated with dysphagia, dyspnea and blurred vision. Dermatologic examination showed erosive lesions involving the palate and the pharynx. Ophthalmologic examination showed symblepharons, ectropion and bilateral cataract. Gingival biopsy revealed a necrotic detachment of the buccal epithelium. Direct immunofluorescence showed linear IgA deposit at the dermo-epidermal junction. Indirect immunofluorescence test was negative. The diagnosis of cicatricial pemphigoid was confirmed. Esophagogastroduodenoscopy objectified double stenosis of the esophagus. Nasopharyngeal and bronchial endoscopy showed ulceration of the epiglottis, hypopharynx, pharynx and bronchial tree. The patient was treated with Solumedrol bolus corresponding to 0.5mg/kg/day prednisone associated with 100mg/day disulone. The patient showed a favorable early clinical outcome complicated because of the aggravation of dysphagia and esophageal stenosis after 2 months. Our case study is singular due to the occurrence of a cicatricial pemphigoid in a male patient with a serious clinical picture due to lesions extending to conjunctival, oral, nasal, esophageal and bronchial mucous membranes associated with direct immunofluorescence only showing IgA deposit.

[Cutaneous leukocytoclastic vasculitis: about 85 cases]. / Vascularites cutanées leucocytoclasiques: à propos de 85 cas.

Clinical manifestation, etiology and outcome of leukocytoclastic vasculitis are little studied. The aim of our study was to examine epidemiological, clinical etiological, and evolutionary characteristics of this entity. We conducted a cross-sectional data collection from medical records of 85 patients with leukocytoclastic vasculitis in the Department of Dermatology at the Farhat Hached University Hospital, Sousse between January 2000 and December 2013. Epidemiological, clinical, paraclinical, etiological data sheets had been completed for each patient. The average age of patients was 47.65 years, ranging between 10 and 78 years. Fifty-three women and 32 men were registered (sex ratio = 0.6). Cutaneous manifestations were dominated by vascular purpura (88.2%). The most common causes of leukocytoclastic vasculitis were systemic diseases (51%), infection (20%) and neutrophilic dermatoses (14.5%). Other causes were drugs (9.1%) and hematologic malignancies (5.4%). The cause of leukocytoclastic vasculitis was not detected in 30 patients (35, 3%). Two predictive factors associated with the acute outcome were retained: the presence of a recent infection (p= 0.014) and drug intake before the rash (p= 0.013). Chronic evolution was positively correlated with antinuclear antibodies (p= 0.009) and cryoglobulinemia (p=0.025). Our study highlights the multitude of causes of leukocytoclastic vasculitis. The search for an underlying disease is an imperative in order to ensure better therapeutic management.

Hemophagocytic lymphohistiocytosis: an unusual complication of leprosy.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition of chaotic uncontrolled immune system stimulation and not fully understood pathophysiology. Most reported cases of hemophagocytic syndrome in patients with mycobacterial infections have been associated with Mycobacterium tuberculosis. As far as we could ascertain, to date, no established HLH case complicating leprosy has been published in the medical literature. CASE REPORT: We describe here a new case of Hansen's disease in a 58-year-old Tunisian man with an unusual complicated clinical course documented as hemophagocytic syndrome. Cutaneous and neurological involvements were the main clinical signs of Hansen's disease. Histological findings suggested the diagnosis of leprosy and were somewhat more characteristic of the lepromatous leprosy type. While on antileprosy treatment, he developed unexplained persistent fever, organomegaly, bicytopenia, and elevated rate of inflammatory markers with bone marrow aspirate showing large macrophages with increased phagocytosis of mature and immature blood elements, typical features of hemophagocytic syndrome. CONCLUSION: A high index of suspicion is essential for prompt diagnosis of hemophagocytic syndrome in the setting of disseminated infection such as leprosy.

Atypical cutaneous fibrous histiocytoma: an unusual and misleading variant of fibrous histiocytoma.

Atypical fibrous histiocytoma is a distinctive variant of cutaneous fibrous histiocytoma, which is often mistaken histologically for sarcoma and which have a tendency to recur locally and a capacity to metastasize, although very rarely. We report a new case of atypical cutaneous fibrous histiocytoma in a 31-year-old man who presented with a recurrent polypoid nodule on the abdominal wall. The diagnosis was made on the basis of morphological and immunohistochemical findings. We discuss through this case and a review of the literature pathological and evolutive features and diagnostic difficulties of this entity.

Childhood linear IgA bullous dermatosis in Tunisia.

The objective was to determine the demographic characteristics, the clinical features, the immuno-histological findings and response to treatment of childhood linear IgA bullous dermatosis (LABD) in Tunisia. We collected all the cases of auto-immune bullous diseases of childhood, diagnosed from January 1987 to December 2006. Based on clinical, histological, and immunofluorescent features, we identified 25 cases of LABD. Sixteen male and nine female children with a mean age of 7.5 years were identified. Clinical manifestations were characterized by a vesiculo-bullous eruption in all cases associated with mucous membrane involvement in two cases. Dapsone was the main therapy in 19 cases, associated with systemic corticosteroids in eight cases. Exclusive antibiotic therapy was successful in five cases. Sixteen of those patients had resolution of disease after a mean period of 15 months and eight patients had severe clinical presentation and required a prolonged follow-up. Childhood LABD is the most frequent bullous dermatosis in Tunisia. The majority of our patients responded rapidly to dapsone treatment and were stabilized for long time. Our cases were characterized by a minimal mucosal involvement and favorable outcome. Treatment with antibiotherapy was interesting. Erythromycin and oxacillin may be considered as an alternative therapy.

Post traumatic amelanotic subungual melanoma.

Subungual melanomas are rare; a delay in the diagnosis is common and is associated with advanced stage. Part of the reason for a delay in presentation to the physician is that patients often attribute the lesion to trauma. Trauma may play a role in the pathogenesis or just draw attention to a skin tumor that may be more susceptible to injury. We report a case of subungual melanoma that was observed in an 86 year old man. The preceding trauma history and misleading clinical appearance delayed the diagnosis slightly. Biopsy of every nodular acral tumor is very important. A direct role of the trauma in the pathogenesis of melanoma remains unclear.

Undifferentiated sebaceous carcinoma: an unusual childhood cancer.

Sebaceous carcinoma is an aggressive, adnexal, rare malignant tumor that may arise in ocular or extra-ocular sites. Extraorbital sebaceous carcinoma is exceptional in childhood. We report a 12-year-old boy with an ocular sebaceous carcinoma who was first seen with an asymptomatic firm, cutaneous nodule on the right eyebrow. The tumor developed slowly within 1 year. Histologically, it was an undifferentiated sebaceous carcinoma. The patient had surgery with wide surgical margins. He was alive and free from disease at a follow-up of 34 months. Close follow-up of this tumor is recommended because of the risk of aggressive behavior.

Netherton's syndrome: the importance of eyebrow hair.

Netherton's syndrome (NS) is a rare autosomal recessive disease associated with variable expressions: congenital ichthyosiform erythroderma, ichthyosis linearis circumflexa, specific hair shaft defects (trichorrhexis invaginata) and atopic diathesis. We report the case of 14-year-old non-identical twins whose diagnosis of NS was established on light microscopy of eyebrow hairs. The sisters consulted for a severe episode of atopic dermatitis. Skin examination revealed an ichthyosiform eruption with generalized, polycyclic erythematous plaques with fine double-edged scaling. The flexural creases were lichenified and multiple eczematoid patches were noted. Blood investigation revealed eosinophilia and high IgE level. Microscopy of scalp hair of the twins was repeatedly normal, but the one of the eyebrows revealed typical trichorrhexis invaginata. The presence of trichorrhexis invaginata is necessary to make the diagnosis of NS, but its identification can be difficult because this defect is variable in time and localization. The examination of eyebrow hairs is especially beneficial for patients first seen in late childhood and adults.

[Lyell's syndrome: a report of 16 cases]. / Le syndrome de Lyell: a propos de 16 observations.

BACKGROUND: Lyell's syndrome (SL) or toxic epidermal necrolysis is a rare mucocutaneous eruption, which is characterised by an acute necrosis of the totality of the epidermis +/- the mucosal epithelium. This is a serious affection considering the severity of systemic manifestations, the unpredictable evolution, and the absence of specific therapy. AIM: To assess epidemiological and clinical features of this condition in our departments. METHODS: This was a retrospective study concerning the cases of Lyell's syndrome carried in the dermatology and the intensive care department of Farhat Hached hospital over a 26 year period. RESULTS: We listed 12 women and 4 with a mean age of 48.9 years. Epidermal detachment varied between 26 and 80% of the body surface and mucus were involved in 87.5% of cases. Systemic manifestations were noted in 11 patients. A drug etiology was found in 87,5% of cases, half of which was due to antibiotics. CONCLUSION: Our results are similar to those in the literature. Our study illustrates the severity of this toxiderma with a high mortality rate (43.75% of cases), conformable with what was predicted by the severity-of-illness score "SCORTEN". Infectious complications were the principal cause of death.

[Fluconazole and treatment of onychomycosis. About 86 cases]. / Fluconazole et traitement des onychomycoses. A propos de 86 cas.

Systemic treatment of onychomycosis is based these last years on the new drug utilization aiming to decrease the length of the treatment and secondary effects particularly with fluconazole. The aim of our study is to determine the efficiency and the tolerance of the fluconazole (Funzol) managed to the dose of 150 mg per week in the treatment of onychomycosis. It is a multicentric and prospective study done to different department of dermatology in Tunisia. Are included adult patients with clinical and mycologic documented onychomycosis. They are treated during 12 to 24 weeks for the fingernail and 24 to 36 weeks for the toenail. The assessment of the efficiency and the tolerance of the drug was clinic and biologic. During this survey, 86 patients are included, 55 female and 31 male with mean age of 43.5 years. Onychomycosis seat to hands in 30 cases (34.8%) with a predominance of Candida species (73%), to toes in 68 cases (79%) witch due in 85% of cases to dermatophytic agent (85%). At 6 months, clinical cure rate is observed in 51% of cases. After 9 months, at the end of therapy, 84% of patients were judged clinical successes and culture was negative in 82% of cases. Some minimal secondary effects are signalled in 11% of cases to 6 weeks and 10% to 12 weeks not justifying the stop of the treatment. So this study confirms the efficiency and the good tolerance of fluconazole in the treatment of onychomycosis.

A rare case of streptomycin-induced toxic epidermal necrolysis in a patient with tuberculosis: a therapeutic dilemma.

OBJECTIVE: To report a case of streptomycin-induced toxic epidermal necrolysis (TEN). CASE SUMMARY: A 55-year-old woman was admitted for treatment of active pulmonary tuberculosis (TB). She was given standard oral anti-TB chemotherapy including isoniazid, rifampin, pyrazinamide, and streptomycin. On the fourth day of therapy, she experienced high fever at 39 degrees C, chills, vomiting, pruritus, and diffuse erythema, followed by extensive bullae formation and skin denudation. Diagnosis of TEN was considered, and all anti-TB drugs were discontinued. Skin biopsy disclosed complete epidermal necrosis with dermal-epidermal cleavage and absence of inflammatory infiltrate, highly suggestive of TEN. The patient was transferred to the intensive care unit. Her general condition and skin lesions improved. A staged-fashion exposure test to the 4 anti-TB drugs allowed the incrimination of streptomycin as the offending agent. DISCUSSION: Anti-TB drugs, mainly rifampin, ethambutol, and isoniazid, have been incriminated in TEN. Streptomycin-induced TEN remains an extremely rare event. However, minor allergic skin reactions (rash, urticaria) have been described with this drug. Our patient presents a rare case of streptomycin-related TEN. Even though dangerous, a step-wise exposure test was necessary to allow safe treatment of active pulmonary TB. It also provided a strong argument of a cause-effect relationship between TEN and streptomycin. An objective causality assessment using the Naranjo rating scale revealed that the adverse drug event was highly probable. CONCLUSIONS: Streptomycin should be added to the list of drugs that induce TEN.

Possible relation of Tunisian pemphigus with traditional cosmetics: a multicenter case-control study.

Pemphigus is a severe, autoimmune, blistering disorder with a high incidence among young women in rural Tunisia. The authors investigated explanatory environmental factors. A multicenter case-control study was conducted prospectively from 1992 to 1996 in Tunisia. Sixty-eight incident female cases of pemphigus and 166 controls matched on age, hospital, and geographic area were included. Data collected concerned socioeconomic status, medical history, drug intakes, lifestyle, and environment. Several factors were significantly associated with pemphigus in multivariate logistic regression analyses: traditional cosmetics (odds ratio (OR) = 4.0, 95% confidence interval (CI): 1.1, 14.8); Turkish baths (OR = 3.2, 95% CI: 1.4, 7.3); cutting up raw poultry (OR = 5.1, 95% CI: 1.3, 19.4); contact with ruminants (OR = 2.7, 95% CI: 1.3, 5.8); and wasp, bee, and spider stings (OR = 3.1, 95% CI: 1.5, 6.4). A dose-dependent relation was observed for traditional cosmetics. All risks except insect bites were higher when analysis was restricted to younger women, the demographic group with higher incidence. The strength of the associations, the dose-dependent relation for traditional cosmetics, and the increase of risk estimates for younger women support a causal relation. Traditional cosmetics widely used by Tunisian women could play a major role in excess of cases of pemphigus.