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.

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.

A delayed diagnosis of lepromatous leprosy: pitfalls and clues to early recognition.

PURPOSE: To remind special attention to atypical symptoms of Hansen's disease, we report a case of an atypical case due to a delayed diagnosis. BACKGROUND: Clinical features of leprosy are well known, cutaneous lesions and involvement of the peripheral nerves being the cardinal clinical signs. Among these presentations, systemic involvement, including mucous membranes of the upper respiratory tract and eyes, is rarely reported even if it is still commonly seen in endemic areas, in particular lepromatous leprosy. CASE REPORT: We describe here a new case of Hansen's disease in a 51-year-old Tunisian woman with an atypical presentation and a delayed diagnosis. The early symptoms of the disease were different from the main clinical signs of Hansen's disease since they involved the upper respiratory tract and the eyes. A nasal smear was positive for acid-fast bacilli, thus confirming the diagnosis of bacilliferous leprosy. Histological findings suggested the diagnosis of leprosy and were somewhat more characteristic of the borderline lepromatous type. CONCLUSION: Diagnosis of Hansen's disease in patients with neither apparent skin lesions nor neurological signs is still problematic. Clinicians should not only pay attention to the more obvious signs in their own fields of expertise but should be aware of the possible systemic involvement of leprosy.

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.

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.

Chronic linear ulcerations of the inguino-crural and buttocks folds.

Vulvo-perineal Crohn's disease is a rare condition either when it is isolated or associated with digestive manifestations. In the former condition named metastatic Crohn's disease, it may constitute a diagnostic challenge and may be confused especially with other infectious or inflammatory disorders. We report a case of vulvo-perineal Crohn's disease in a 46-year-old woman. A 46-year-old woman was diagnosed with a vulvo-perineal Crohn's disease without digestive involvement. There was a chronic edema of the vulva with linear ulcerations on the inguino-crural regions and the buttocks fold, of 3 years. Treatment with metronidazole (1 g/day for 6 months) led to almost complete healing of the ulcerations with a sustained result. Physicians must be aware of the diverse manifestations and confusing presentations of vulvo-perineal Crohn's disease.

[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.

Endogenous ochronosis: case report and a systematic review of the literature.

BACKGROUND: Endogenous ochronosis (EO) is a rare autosomal recessive disorder due to accumulation of oxidized and polymerized forms of homogentisic acid (HGA) in connective tissues, giving them a deep dark blue pigmentation. AIM: Through a new Tunisian case of EO and a review of the literature, we aimed to define the epidemioclinical features of EO, its diagnostic criteria, and evolution. METHODS: Three hundred and forty patients were enrolled through 54 articles and four abstracts. CASE REPORT: A 35-year-old woman, born in consanguineous parents, presented with blue-grey patches of fingernails, first interdigital spaces, and ears with brown conjunctival pigmentation. Urine specimen turned dark on standing overnight. The diagnosis of EO was confirmed by urinary high levels of HGA. Investigations revealed radiologic signs of ochronotic arthropathy. REVIEW OF THE LITERATURE: EO is ubiquitary. Its prevalence was estimated at almost 6.5 cases/year. The mean age at diagnosis was 55.9 years (M/F: 1.85). Onset symptoms mainly consisted in cutaneous signs. Ochronotic arthropathy was the most frequently reported manifestation. Treatment was mainly symptomatic. DISCUSSION: EO is often revealed in adulthood mainly after the fourth decade. Urinary darkening is the first sign of the disease but is rarely reported as an onset sign. Skin signs are the alerting features. Ochronotic arthropathy is insidious but may be debilitating. No specific medical treatment of EO is available. CONCLUSION: Cutaneous manifestations are the hallmarks of OE. As vital organ involvement has been reported, close monitoring and continuous surveillance is warranted.

Dermatomyositis and breast cancer: a multicenter Tunisian retrospective study of 13 cases.

AIM: To evaluate the epidemiological, clinical, biological features and prognostic factors in patients presenting an association of dermatomyositis (DM) and breast cancer (BC). METHODS: Medical records of 13 patients with DM and BC among 210 DM collected from January 1982 to march 2009 were retrospectively reviewed. RESULTS: Mean age was 47 ± 18 years. DM preceded BC in 3 patients, was concurrent with BC in 2 cases and followed it in 8 cases. Clinically, ulcerative and bullous lesions predominating on limbs were observed in 2 patients. A severe proximal muscular weakness was observed in 4 cases and one patient has presented an amyopathic dermatomyositis. BC was staged IV in 3 pts, IIIA in 2 cases while 3 had stage IIB, 3 stage IIA and 2 stage I according to TNM classification. Nine out of 13 patients had parallel improvement of DM symptoms after treatment of BC. Five patients died of recurrence or distant metastasis (mortality 41.66%). Median survival was 35 months (3-177) after DM diagnostic. CONCLUSION: In Tunisia, DM is associated with an increased incidence of BC. A paraneoplastic course of DM is noted in 70% of patients. In view of the increased risk of BC in our country, in addition to routine examination and laboratory screening, mammography, chest ultrasound, and gynaecological examination, are indicated in women with DM older than 40 years, particularly in case of previous personal or familial history of breast neoplasm.

Late-onset disseminated superficial actinic porokeratosis in an elderly woman.

INTRODUCTION: Disseminated superficial actinic porokeratosis (DSAP) is the most common form of porokeratosis. A case of late-onset DSAP is reported with an uncommon presentation in an elderly patient. METHODS: An 80-year-old woman, with diabetes mellitus treated with metformin, and hypertension treated with metoprolol and captopril, presented with multiple 2 mm-to-7 cm brown patches, for 5 years. The patches were often confluent with an atrophic center and a well-demarcated keratotic border located on the thighs, lower legs, and feet. RESULTS: Histological examination of a cutaneous biopsy showed the presence of rare cornoid lamellae, confirming the clinical diagnosis of DSAP. CONCLUSION: In this report, the atypical presentation of DSAP is discussed in this elderly patient and also the possible triggering factors at this age.

Bullous Darier's disease mimicking Hailey-Hailey disease.

BACKGROUND: The bullous variant of Darier's disease (DD) is a rare type characterized by histological and clinical overlap with Hailey-Hailey disease (HDD). CASE REPORTS: The following case report describes two cases of familial DD; a 51-year-old woman who presented with erythematous plaques, covered by small blisters in axillary and inguinal areas, and the first patient's daughter, who presented with keratotic papules localized on the axillary and inframammary folds. CONCLUSION: These two cases are original by the predominant flexural distribution, and by a bullous form in the first case, clinically and histologically mimicking HHD.

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.