Efficacy and Safety of Contact Cryosurgery in the Treatment of Earlobe Keloids.

Earlobe keloids, although not a life-threatening disease, can severely impair a patient's quality of life. Contact cryosurgery is a safe technique for the management of earlobe keloids with tolerable side effects. The aim of this study was to assess the safety and efficacy of contact cryosurgery in the treatment of earlobe keloids through a 1-year prospective study. We treated 15 Caucasian women ranging in age from 8 to 54 years with a total of 18 earlobe keloids. Excellent improvement was noted in 11 earlobe keloids, with a 75%-100% reduction in the volume of the keloids. An improvement ranging from 50% to 75% was observed in the seven other earlobe keloids. During the 18-month follow-up period, there was no evidence of bleeding, infection, recurrence, or permanent hypopigmentation. Two patients had a transient hypopigmentation which disappeared 2 months after the final session of contact cryosurgery. The results of our study highly encourage the use of contact cryosurgery which has tolerable side effects and high safety.

A case of herpes simplex virus reactivation after fractional ablative carbon dioxide laser to treat a burn scar.

Fractional photothermolysis was initially introduced by Manstein in 2004 .Fractional CO2 laser technology introduced has allowed physicians to obtain good cosmetic results with a lower rate of complications than non-fractionated ablative laser treatment. However, adverse effects may still occur.Reported cases of HSV infection after fractional photothermolysis are rare. A 48-year-old woman with Fitzpatrick skin type III presented with a scar in her perioral area desiring esthetic improvement of her burn scar. She didn't have a history of recurrent herpes simplex virus (HSV) infection periorally. A fractionated resurfacing laser Quadralase (Candela) was used to treat her perioral burn scar. Two sessions were performed with a month interval. Five days after the second session of laser therapy even after she took antiviral prophylaxis based on valacyclovir 500mg twice daily 24 hours before the laser session and 3 days after, she presented with a rash on the perioral area preceded by pain. Correlation of the history and the clinical presentation was consistent with HSV reactivation. Treatment was initiated with acyclovir 10mg/kg/8h administered intravenously for 10 days with a clearing of her vesicular eruption. Fractional CO2 laser is a very safe procedure when used with accepted parameters. Early recognition, close monitoring and careful wound care will prevent long term sequelae when complications occur.

Multicentric Anogenital Bowen's Disease Treated with Imiquimod and CO2 Laser.

A 53-year-old white woman presented to our clinic with skin lesions in the anogenital region that had persisted for 1 year. She had a past medical history of total vulvectomy for a vulvar localization of Bowen's disease. She was otherwise in good health, with no evidence of illness or immunosuppression. Physical examination revealed multiple erythematous papular lesions located in the anogenital region (Figure 1). Dermatoscopy of the anogenital papules revealed glomerular vessels on an erythematous background typical of Bowen's disease (Figure 2A). There were no palpable inguinal lymph nodes. Rectosigmoidoscopy was normal. The biopsy specimen showed full thickness keratinocyte atypia with loss of normal stratification and was conclusive for Bowen's disease (Figure 2B).

Significant alleviation of Darier's disease with fractional CO2 laser.

Darier's disease (DD) is a dominantly inherited genodermatosis with highly variable expression. It is characterized by symmetrical hyperkeratotic papules affecting seborrheic areas and extremities. The existence of unsightly lesions could lead to discomfort and social handicap. Conventional treatment consists of topical and systemic steroids and/or retinoids alleviating DD. Ablative lasers also have been used to treat these conditions with variable results and side effects. To the best of our knowledge, fractional CO2 laser has never been used to treat DD. We present a case of a 36-year-old woman with verrucous and hyperkeratotic plaques of the forehead significantly improved after two sessions of fractional CO2 laser treatment. Neither scars nor pigmentary disorders were noted.

Particular Mal de Meleda phenotypes in Tunisia and mutations founder effect in the Mediterranean region.

Mal de Meleda (MDM) is a rare, autosomal recessive form of palmoplantar keratoderma. It is characterized by erythema and hyperkeratosis of the palms and soles that progressively extend to the dorsal surface of the hands and feet. It is caused by mutations in SLURP-1 gene encoding for secreted mammalian Ly-6/uPAR-related protein 1 (SLURP-1). We performed mutational analysis by direct sequencing of SLURP-1 gene in order to identify the genetic defect in three unrelated families (families MDM-12, MDM-13, and MDM-14) variably affected with transgressive palmoplantar keratoderma. A spectrum of clinical presentations with variable features has been observed from the pronounced to the transparent hyperkeratosis. We identified the 82delT frame shift mutation in the SLURP-1 gene in both families MDM-12 and MDM-13 and the missense variation p.Cys99Tyr in family MDM-14. To date, the 82delT variation is the most frequent cause of MDM in the world which is in favour of a recurrent molecular defect. The p.Cys99Tyr variation is only described in Tunisian families making evidence of founder effect mutation of likely Tunisian origin. Our patients presented with very severe to relatively mild phenotypes, including multiple keratolytic pits observed for one patient in the hyperkeratotic area which was not previously reported. The phenotypic variability may reflect the influence of additional factors on disease characteristics. This report further expands the spectrum of clinical phenotypes associated with mutations in SLURP1 in the Mediterranean population.

[Dermatofibrosarcoma protuberans: a study of 18 cases]. / Le dermatofibrosarcome protubérant : Etude de 18 cas.

BACKGROUND: Dermatofibrosarcoma protuberans is the most common mesenchymatous skin tumor. It is often diagnosed late because of its slow development and the lack of symptoms. AIM: To elucidate the epidemio-clinical characteristics of dermatofibrosarcoma in our study. methods: We report a retrospective study of 18 cases of DFS between 1989 and 2009. RESULTS: Mean age at diagnosis was 45.2 years. The average delay before consultation was 45.7 months. Three patients reported a history of trauma. The tumor was mainly located on the trunk or the back. The diagnosis of dermatofibrosarcoma protuberans was histologically confirmed in all cases. Immunohistochemical study was achieved in 6 cases and showed positive staining for CD34. The treatment consisted of surgical excision in all patients. CONCLUSION: Dermatofibrosarcoma protuberans is a low-malignancy potential skin tumor. Treatment of choice is surgery. The main risk is tumor relapse.

Subungueal haemorrhages following docetaxel (taxotere) treatment.

INTRODUCTION: Docetaxel belongs to the taxane group of chemotherapeutic agents used in the management of various malignant diseases. Nail changes as a complication of such treatment are observed in about 44%. Subungual haemorrhages (SH), are very rare following docetaxel therapy and only a few cases have been previously reported. OBSERVATION: An 80-year-old man suffering from prostate adenocarcinoma was treated with a 3-weekly cure of docetaxel started 3 months earlier. Nail changes occurred after the 5th cycle of docetaxel. Clinical examination revealed orange discoloration of the nail plates, subungueal haemorrhages (SH) and onycholysis involving nails of all the digits and toenails of both hands and feet. These features were highly suggestive of nail toxicity following docetaxel therapy. DISCUSSION: Nail changes secondary to Taxane chemotherapy includes nail bed dyschromia, onycholysis, red or orange Beau's lines and subungueal hyperkeratosis. SH, as reported in this case, is related to the cumulative dose of docetaxel and should not be attributed to other systemic diseases. Clinicians should recognize this complication to avoid abusive treatment or investigations and inform the patients about the possibility of nail changes secondary to taxane drugs.

[Cutaneous schwannoma: study of 26 cases]. / Schwannomes cutanés : Etude de 26 cas.

BACKGROUND: Schwannoma is generally a benign tumour developing from Schwann cells. AIM: To study epidemiological, clinical and pathological features of scwannoma. METHODS: Retrospective study about 26 cases of cutaneous schwannoma collected in our department over 24 years. RESULTS: Our patients were 12 females and 14 males. Mean age was 30 years. Clinically, schwannoma was a subcutaneous tumour which was painful in 11 cases. Individual tumours were located on the limbs (10 cases), on the head (9 cases) and the trunk (5 cases). Multiple tumours were seen in two patients. Histopathological examination confirmed the diagnosis of schwannoma in all cases. Treatment was complete surgical excision in all cases. CONCLUSION: Cutaneous location of schwannoma is not rare. Usually, and contrarily to our series, there is no sex predominance. Clinically, schwannoma is located on the limbs and head. The diagnosis is confirmed by histopathological examination. Malignant epitheloid schwannoma diagnosed in one of our patients is a rare tumour. Schwannomas usually grow slowly and are poorly invasive. Malignant degeneration is exceptional. Treatment is complete surgical excision.

[Hyalinosis cutis et mucosae : 5 cases from Tabarka (Tunisia)]. / La hyalinose cutanéo-muqueuse : 5 cas originaires de Tabarka (Tunisie)

BACKGROUND: Hyalinosis cutis et mucosae (HCM), is a rare autosomal recessive genodermatosis. Cutaneous features are characteristic and allow to suspect diagnosis. AIM: To report a series of HCM. METHODS: A retrospective study of all cases of HCM, diagnosed in a dermatology department over a period of 25 years (1983-2007). RESULTS: Over the considered period, 5 new cases of HCM were diagnosed. Patients were aged between 14 and 41 years. They were 3 females and 2 males. All patients were native of Tabarka (northwestern Tunisia). The age of the onset of the disease varied from neonatal period to 5 years. Hoarseness was the first clinical manifestation in all cases. Skin lesions developed between the ages of 3 and 8 years. Vesiculobullous lesions were observed in 2 patients. Moniliform blepharosis was seen in all patients. Warty and hyperkeratotic papules were observed in 3 patients. Diffuse thickening of the skin was seen in 3 patients. Lesions were primarily distributed on the face. All patients presented diffuse scars. Linear palmoplantar keratoderma was seen in one patient. Asymptomatic endocranial calcifications were noted in 4 patients. A pituitary adenoma was noted in one patient. Histopathological examination of a skin lesion revealed a typical pattern of HCM. Two patients were treated with systemic retinoids without improvement. CONCLUSION: We reported five new cases of HCM. All patients were native from Tabarka. We report also one case of linear palmar keratoderma associated with HCM. This association was not reported in the literature. Finally, the association HCM-pituitary adenoma, seen in one patient, may be fortuitous.

Pemphigus vulgaris presenting as an isolated crusted plaque of the cheek.

A healthy 45-year-old man presented with a three-month history of an isolated enlarging eroded plaque on the left cheek. Pemphigus vulgaris was diagnosed by biopsy and positive direct immunofluorescence. The patient was lost to follow up, but returned two months later with generalized cutaneous involvement without mucosal involvement. He responded rapidly to oral corticosteroid therapy.

New mutations of Darier disease in Tunisian patients.

Darier's disease (DD, MIM 124200) also known as Darier-White disease and keratosis follicularis, is a rare autosomal dominant skin disorder characterized by warty papules and plaques in the seborrheic area (central trunk, flexures, scalp, and forehead). Pathogenic mutations in the ATP2A2 gene encoding the sarcoplasmic/endoplasmic reticulum Ca(2+) ATPase (SERCA) 2 gene underlie the disease. In the present study, we performed genetic investigation of three unrelated Tunisian families affected by DD. Mutation screening was performed by direct sequencing of the coding region and exon/intron boundaries of the ATP2A2 gene. Patients in the 3 studied families exhibited classical DD phenotype. DD was associated with neurological and cardiac disorders in one family. Two novel mutations were identified: a missense mutation (R559Q) and a frameshift mutation (1713-1714 del 2A). Both pathogenic mutations are located in exon 13 of the ATP2A2 gene and affected the ATP-binding site of the SERCA2 protein. In one family, no mutation was found within the coding region and exon/intron boundaries of the ATP2A2 gene. Our findings provide further evidence for the genetic heterogeneity of DD in Tunisia and that most mutations involved in this disease are family specific.

[Cutaneous sarcoidosis through a hospital series of 28 cases]. / Sarcoïdose cutanée à travers une série hospitaliére de 28 cas.

BACKGROUND: Sarcoidosis is a systemic disease, of unknown etiology, characterized by noncaseating epithelioid granulomas. It may affect many organs mainly lungs, lymph nodes and skin. AIM: The aim of our study is to evaluate retrospectively the epidemiological, clinical features and therapeutic particularities of cutaneous sarocidosis through a hospital series conducted in the dermatology department of Habib Thameur hospital. METHODS We have included 28 patients (23 females and 5 males) with a mean age of 45.5 years. RESULTS: The most frequent skin lesions were: papular sarcoidosis in 6 cases, nodular sarcoidosis in 8 cases and plaques in 6 cases. The other clinical types observed were: lupus pernio (2 cases), subcutaneous nodules (2 cases) and scar sarcoidosis (2 cases). Three patients presented erythema nodosum. Tuberculin skin test was negative in 77% of cases. Lymph nodes were palpable in 4 patients. Investigative exams revealed intrathoracic involvement in 8 cases and anterior uveitis sequels in 2 cases. Cutaneous manifestations were treated by chloroquin in 13 cases, allopurinol in 3 cases and intralesional betamethasone injections in 1 case. Five patients, with systemic involvement, underwent a therapy with oral corticosteroids. CONCLUSION: Sarcoidosis is a rare affection in Tunisia that affects mainly females. Cutaneous manifestations incite physicians to carry out complete investigations and to uphold a regular follow-up in order to detect systemic involvement.

[Acanthosis nigricans: epidemiologic study of 69 cases]. / Acanthosis nigricans: étude épidémiologique de 69 cas.

BACKGROUND: Acanthosis nigricans is a well-defined skin disorder with a distinctive appearance and an elective topography to the flexural areas that facilitate its diagnosis. In Tunisia, apart from isolated reports of malignant AN, few epidemiologic data are available. AIM: In order to describe the characteristics of this affection, we conducted a retrospective study in the area of Tunis over a 12-year period. METHODS: Retrospective study of all the cases of AN seen in the dermatology department of Habib Thameur teaching hospital between 1991 and 2003, including the cases coming from the north and of the centre of Tunisia. RESULTS: Sixty-nine cases were recorded with a sex-ratio H/F of 0.17. The average age was 24.1 years (extremes of 3 and 66 years) and 57.9% of the patients were aged between 9 and 22 years. The neck (98.6%) and the axillae (75.4%) represented the most common localizations. All the patients presented a benign AN, comprising 51 cases of pseudo-AN, 11 cases of AN associated with endocrine diseases, 5 cases of familial AN and 2 cases of AN associated with an auto-immune disease. Local treatments associated an hypocaloric diet (AN with obesity) were recommended with a light improvement after 3 months an average follow-up. CONCLUSION: The frequency of the AN is probably underestimated because of the usually asymptomatic character of this affection. AN constitutes a double cutaneous marker, being able to reveal either an hyperinsulinemia constituting a cardiovascular risk factor or an ignored subjacent neoplasm engaging the vital prognosis.