Primary cutaneous B-cell lymphoblastic lymphoma (PB-LBL) is an exceedingly rare malignant neoplas... more Primary cutaneous B-cell lymphoblastic lymphoma (PB-LBL) is an exceedingly rare malignant neoplasm in children. Skin lesions may represent the initial sign. Herein, we report a case of a PB-LBL with cutaneous lesions as a primary extranodal involvement, occurring in a 5-year-old infant.
Journal of Cosmetic and Laser Therapy, Jul 18, 2018
Fractional photothermolysis was initially introduced by Manstein in 2004 .Fractional CO2 laser te... more 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.
Linear distribution of psoriasis is rare. This presentation offers to physicians some diagnostic ... more Linear distribution of psoriasis is rare. This presentation offers to physicians some diagnostic difficulty, especially in the absence of a history of pre-existing psoriasis or in the presence of any other linear dermatosis. In this study, we report cases of 3 girls, ages 4 yr, 5 yr and 10 yr, admitted to our dermatology department. The clinical features and differential diagnosis of this skin disease which, in children, can be easily mistaken for inflammatory verrucous epidermal nevus, are discussed.
Lichen sclerosus is a chronic inflammatory mucocutaneous disease which origin remains unknown. It... more Lichen sclerosus is a chronic inflammatory mucocutaneous disease which origin remains unknown. Its prevalence ranges from one in 300 to one in 1000 of all patients referred to a dermatology clinic in the seventeenth. Through the analysis of a hospital survey, we outline the epidemio-clinical aspects of this dermatosis. Over a 19-year period (1984-2002), we have conducted a retrospective and monocentric study of all patients with genital lichen sclerosus were examined at the dermatology department of Habib Thameur hospital. Thirty-four patients suffered from lichen sclerosus. There were 33 female and only one male (sex-ratio: 0.03). All patients underwent topical corticosteroid therapy (level I, II or IV). The recovery rate of lichen sclerosus was about 20% (7/34). An epidermoid carcinoma occurred in three patients. The frequency of lichen sclerosus in our study is estimated at 1,8 new cases per year. This frequency is probably under-estimated because of some patients' reluctance to seek help. A relatively low recovery rate of genital lichen sclerosus was found in our study. This may be related to an inadequate follow up added to an insufficient treatment adherence.
Background: Cutaneous adverse drug reactions correspond to adverse effects with cutaneous express... more Background: Cutaneous adverse drug reactions correspond to adverse effects with cutaneous expression resulting from the systemic penetration of a drug in the body. The aim of this study is to evaluate the various clinical pictures of RCM, their epidemiologic characteristics as well as the different causative drugs, through a retrospective hospital series. Methods: It is about a retrospective study about all the patients consulting and/or hospitalized for suspicion of an adverse cutaneous drug reaction led to the service of dermatology of the teaching hospital Habib Thameur of Tunis over a 3-year period (from January 2002 to December 2004). The diagnosis was based on a beam of clinical and anamnestic arguments. Only the patients having a positive pharmacovigilance investigation were retained. Results: 28 patients were retained for this study. The macular and papular exanthema represented the most frequent clinical aspects followed by acute urticaria and fixed drug eruption. The antibiotics represented the most causative drugs followed by analgesics and non steroidal anti-inflammatory. Conclusion: We record a lower frequency of cutaneous adverse drug reactions in comparison with the literature. We also find a high frequency of severe forms and stress on the difficulties encountered in the identification of the causativele drugs. Though, a close cooperation between the various hospital structures and pharmacovigilance centers is mandatory.
Cutaneous leishmaniasis (CL) is a widespread parasitic disease that represents a major public hea... more Cutaneous leishmaniasis (CL) is a widespread parasitic disease that represents a major public health problem in several countries including Tunisia. The aim of this study was to determine the characteristics of childhood CL in a university hospital center in Tunis. The files of all children treated for of CL in the Dermatology Department of Habib Thameur Hospital over a 23-year period were reviewed. A total of 60 children were included. The mean age was 9.2 years and the sex ratio was 0.93. All patients lived or had stayed in an endemic area. Infectious risk was highest in autumn (41.6%). The most frequent clinical finding consisted of crusty nodules (53.3%) that were most often the only manifestation (63.3%) and usually located on the face (65%). Clinical diagnosis was confirmed by either peripheral blood smear (56.6%) or histological examination (35%). The treatment of choice was Glucantime administered by the intralesional route in 70% of patients or by intramuscular route in 30%. The outcome was favorable in all cases within 2 weeks to 6 months. Sequels were noted in 9 cases (18%) in the form of pigmented (7 cases) or atrophic (2 cases) residual scars. The frequency of childhood CL is relatively high especially during the second decade of life. Childhood CL is characterized by a generally favorable outcome and lack of distinguishing clinical characteristics form adult CL. If possible, therapeutic abstention is more widely in the children due to the high risk of adverse reactions.
Annales De Dermatologie Et De Venereologie, Jun 1, 2015
Introduction Le rhinophyma apparait a la phase tardive de la rosacee. Il touche principalement le... more Introduction Le rhinophyma apparait a la phase tardive de la rosacee. Il touche principalement les hommes, en general apres l’âge de 50 ans. Les modalites therapeutiques sont variables incluant la chirurgie et l’electrocoagulation avec des resultats cosmetiques souvent decevants. L’avenement des lasers CO2 ablatifs a constitue une excellente alternative therapeutique avec des resultats cosmetiques satisfaisants et des effets indesirables moindres. Patients et methodes Le but de cette etude est de demontrer l’interet du laser CO2 continu dans le traitement du rhinophyma. Etude retrospective menee au service de dermatologie de l’hopital Habib Thameur de Tunis incluant deux patients ayant un rhinophyma severe traites par laser CO2 continu. Resultats Nos deux patients âges respectivement de 54 et 60 ans avaient des rhinophyma severes. Ils ont ete traites par laser CO2 10 600 nm continu (Sharplan et Limmer 1 cas, Candela 1 cas). Le premier patient avait necessite 4 seances et le deuxieme patient deux seances. Aucune complication n’a ete notee. Le resultat cosmetique etait excellent et les deux malades etaient tres satisfaits des resultats. Un patient a presente une recidive apres 5 ans. Discussion Le traitement par laser CO2 du rhinophyma a ete evalue par plusieurs etudes. Il est considere par la plupart des auteurs comme le gold standard dans le traitement du rhinophyma. Dans ce travail, la revue de la litterature et nos deux cas montre que les avantages de cette methode etaient la precision de la rhinosculpture, le champ operatoire exsangue, la cicatrisation rapide et le faible risque cicatriciel. Les inconvenients en le comparant a la chirurgie etaient la duree plus longue du temps operatoire et le cout plus onereux. Conclusion Le traitement du rhinophyma par laser CO2 continu donne de bons resultats esthetiques mais les recidives sont toujours possibles necessitant la repetition des seances.
Earlobe keloids, although not a life-threatening disease, can severely impair a patient's quality... more 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.
Médecine tropicale : revue du Corps de santé colonial, 2005
Dermatophytic disease is a rare chronic infection caused by common dermatophytes and characterize... more Dermatophytic disease is a rare chronic infection caused by common dermatophytes and characterized by cutaneous and subcutaneous invasion. It is observed mainly in North Africa where it is presumably inherited via an autosomal recessive trait. Cellular immunodeficiency could account for the chronic nature of the disease and for its refractoriness to drug treatment. The purpose of this report is to describe the occurrence of dermatophytic disease in three sisters of consanguineous parents in a rural region of northern Tunisia. To our knowledge, this is the first familial case reported in Tunisia. The most salient features in these patients were early age of disease onset in three females, isolation of Microsporum canis at the beginning of the disease, relatively favorable course with long remissions in two cases, and absence of visceral involvement. Various drugs including antifungal agents and immunostimulants were used for treatment with disappointing results. Surgical excision ass...
Périneuriome extraneural ; Nez ; Nodule ; Storiforme Résumé Introduction.-Le périneuriome est une... more Périneuriome extraneural ; Nez ; Nodule ; Storiforme Résumé Introduction.-Le périneuriome est une tumeur rare des gaines nerveuses périphériques, composée de cellules périneuriales. On en distingue deux grandes variétés : le périneuriome intraneural et le périneuriome extraneural. Nous rapportons un cas de périneuriome extraneural des tissus mous développé dans une localisation atypique, au niveau de l'aile du nez. Observation.-Un homme de 24 ans présentait sur l'aile gauche du nez un nodule de 1 cm évoluant depuis quatre ans. Histologiquement, il s'agissait d'une prolifération tumorale fusocellulaire d'architecture storiforme occupant le derme. Après exérèse, la lésion n'a pas récidivé. Commentaires.-Le périneuriome extraneural des tissus mous est une tumeur d'origine périneuriale, le plus souvent bénigne. Il s'agit en général d'un nodule cutané unique, siégeant le plus souvent au niveau des extrémités ou du tronc. La localisation au nez, comme chez notre patient, est rarement décrite. La tumeur est constituée histologiquement d'une prolifération fusocellulaire dermique bien limitée, d'architecture storiforme. Les cellules tumorales expriment l'epithelial membrane antigen (EMA) et non la protéine S100. Le traitement est chirurgical.
Background: Digital necrosis is a frequent vascular disease. Its very often reflects arterial dis... more Background: Digital necrosis is a frequent vascular disease. Its very often reflects arterial disorders of macro or mirocirculation. Paraneoplastic digital necrosis is rare, however. Aim: Report a new case of paraneoplastic digital necrosis Case: Our patient was a 62-year old diabetic female patient with a history of total thyroid ablation at 50 year old. She presented with bilateral digital necrosis of the second, third and fourth fingers. During her recovery, we have found a biological inflammatory syndrome and abdominal echography revealed a metastatic liver. Radiological investigations in search for the primitive tumour were negative. The patient was referred to a specialized center for a palliative chemotherapy. Conclusion: Etiologies of digital necrosis are various, mainly iatrogen, professional, toxic, atheroma or systemic disease. In case of no evident cause, explorations should be gone to detect underlying malignancies.
Background: Hyalinosis cutis et mucosae (HCM), is a rare autosomal recessive genodermatosis. Cuta... more 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.
Primary cutaneous B-cell lymphoblastic lymphoma (PB-LBL) is an exceedingly rare malignant neoplas... more Primary cutaneous B-cell lymphoblastic lymphoma (PB-LBL) is an exceedingly rare malignant neoplasm in children. Skin lesions may represent the initial sign. Herein, we report a case of a PB-LBL with cutaneous lesions as a primary extranodal involvement, occurring in a 5-year-old infant.
Journal of Cosmetic and Laser Therapy, Jul 18, 2018
Fractional photothermolysis was initially introduced by Manstein in 2004 .Fractional CO2 laser te... more 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.
Linear distribution of psoriasis is rare. This presentation offers to physicians some diagnostic ... more Linear distribution of psoriasis is rare. This presentation offers to physicians some diagnostic difficulty, especially in the absence of a history of pre-existing psoriasis or in the presence of any other linear dermatosis. In this study, we report cases of 3 girls, ages 4 yr, 5 yr and 10 yr, admitted to our dermatology department. The clinical features and differential diagnosis of this skin disease which, in children, can be easily mistaken for inflammatory verrucous epidermal nevus, are discussed.
Lichen sclerosus is a chronic inflammatory mucocutaneous disease which origin remains unknown. It... more Lichen sclerosus is a chronic inflammatory mucocutaneous disease which origin remains unknown. Its prevalence ranges from one in 300 to one in 1000 of all patients referred to a dermatology clinic in the seventeenth. Through the analysis of a hospital survey, we outline the epidemio-clinical aspects of this dermatosis. Over a 19-year period (1984-2002), we have conducted a retrospective and monocentric study of all patients with genital lichen sclerosus were examined at the dermatology department of Habib Thameur hospital. Thirty-four patients suffered from lichen sclerosus. There were 33 female and only one male (sex-ratio: 0.03). All patients underwent topical corticosteroid therapy (level I, II or IV). The recovery rate of lichen sclerosus was about 20% (7/34). An epidermoid carcinoma occurred in three patients. The frequency of lichen sclerosus in our study is estimated at 1,8 new cases per year. This frequency is probably under-estimated because of some patients' reluctance to seek help. A relatively low recovery rate of genital lichen sclerosus was found in our study. This may be related to an inadequate follow up added to an insufficient treatment adherence.
Background: Cutaneous adverse drug reactions correspond to adverse effects with cutaneous express... more Background: Cutaneous adverse drug reactions correspond to adverse effects with cutaneous expression resulting from the systemic penetration of a drug in the body. The aim of this study is to evaluate the various clinical pictures of RCM, their epidemiologic characteristics as well as the different causative drugs, through a retrospective hospital series. Methods: It is about a retrospective study about all the patients consulting and/or hospitalized for suspicion of an adverse cutaneous drug reaction led to the service of dermatology of the teaching hospital Habib Thameur of Tunis over a 3-year period (from January 2002 to December 2004). The diagnosis was based on a beam of clinical and anamnestic arguments. Only the patients having a positive pharmacovigilance investigation were retained. Results: 28 patients were retained for this study. The macular and papular exanthema represented the most frequent clinical aspects followed by acute urticaria and fixed drug eruption. The antibiotics represented the most causative drugs followed by analgesics and non steroidal anti-inflammatory. Conclusion: We record a lower frequency of cutaneous adverse drug reactions in comparison with the literature. We also find a high frequency of severe forms and stress on the difficulties encountered in the identification of the causativele drugs. Though, a close cooperation between the various hospital structures and pharmacovigilance centers is mandatory.
Cutaneous leishmaniasis (CL) is a widespread parasitic disease that represents a major public hea... more Cutaneous leishmaniasis (CL) is a widespread parasitic disease that represents a major public health problem in several countries including Tunisia. The aim of this study was to determine the characteristics of childhood CL in a university hospital center in Tunis. The files of all children treated for of CL in the Dermatology Department of Habib Thameur Hospital over a 23-year period were reviewed. A total of 60 children were included. The mean age was 9.2 years and the sex ratio was 0.93. All patients lived or had stayed in an endemic area. Infectious risk was highest in autumn (41.6%). The most frequent clinical finding consisted of crusty nodules (53.3%) that were most often the only manifestation (63.3%) and usually located on the face (65%). Clinical diagnosis was confirmed by either peripheral blood smear (56.6%) or histological examination (35%). The treatment of choice was Glucantime administered by the intralesional route in 70% of patients or by intramuscular route in 30%. The outcome was favorable in all cases within 2 weeks to 6 months. Sequels were noted in 9 cases (18%) in the form of pigmented (7 cases) or atrophic (2 cases) residual scars. The frequency of childhood CL is relatively high especially during the second decade of life. Childhood CL is characterized by a generally favorable outcome and lack of distinguishing clinical characteristics form adult CL. If possible, therapeutic abstention is more widely in the children due to the high risk of adverse reactions.
Annales De Dermatologie Et De Venereologie, Jun 1, 2015
Introduction Le rhinophyma apparait a la phase tardive de la rosacee. Il touche principalement le... more Introduction Le rhinophyma apparait a la phase tardive de la rosacee. Il touche principalement les hommes, en general apres l’âge de 50 ans. Les modalites therapeutiques sont variables incluant la chirurgie et l’electrocoagulation avec des resultats cosmetiques souvent decevants. L’avenement des lasers CO2 ablatifs a constitue une excellente alternative therapeutique avec des resultats cosmetiques satisfaisants et des effets indesirables moindres. Patients et methodes Le but de cette etude est de demontrer l’interet du laser CO2 continu dans le traitement du rhinophyma. Etude retrospective menee au service de dermatologie de l’hopital Habib Thameur de Tunis incluant deux patients ayant un rhinophyma severe traites par laser CO2 continu. Resultats Nos deux patients âges respectivement de 54 et 60 ans avaient des rhinophyma severes. Ils ont ete traites par laser CO2 10 600 nm continu (Sharplan et Limmer 1 cas, Candela 1 cas). Le premier patient avait necessite 4 seances et le deuxieme patient deux seances. Aucune complication n’a ete notee. Le resultat cosmetique etait excellent et les deux malades etaient tres satisfaits des resultats. Un patient a presente une recidive apres 5 ans. Discussion Le traitement par laser CO2 du rhinophyma a ete evalue par plusieurs etudes. Il est considere par la plupart des auteurs comme le gold standard dans le traitement du rhinophyma. Dans ce travail, la revue de la litterature et nos deux cas montre que les avantages de cette methode etaient la precision de la rhinosculpture, le champ operatoire exsangue, la cicatrisation rapide et le faible risque cicatriciel. Les inconvenients en le comparant a la chirurgie etaient la duree plus longue du temps operatoire et le cout plus onereux. Conclusion Le traitement du rhinophyma par laser CO2 continu donne de bons resultats esthetiques mais les recidives sont toujours possibles necessitant la repetition des seances.
Earlobe keloids, although not a life-threatening disease, can severely impair a patient's quality... more 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.
Médecine tropicale : revue du Corps de santé colonial, 2005
Dermatophytic disease is a rare chronic infection caused by common dermatophytes and characterize... more Dermatophytic disease is a rare chronic infection caused by common dermatophytes and characterized by cutaneous and subcutaneous invasion. It is observed mainly in North Africa where it is presumably inherited via an autosomal recessive trait. Cellular immunodeficiency could account for the chronic nature of the disease and for its refractoriness to drug treatment. The purpose of this report is to describe the occurrence of dermatophytic disease in three sisters of consanguineous parents in a rural region of northern Tunisia. To our knowledge, this is the first familial case reported in Tunisia. The most salient features in these patients were early age of disease onset in three females, isolation of Microsporum canis at the beginning of the disease, relatively favorable course with long remissions in two cases, and absence of visceral involvement. Various drugs including antifungal agents and immunostimulants were used for treatment with disappointing results. Surgical excision ass...
Périneuriome extraneural ; Nez ; Nodule ; Storiforme Résumé Introduction.-Le périneuriome est une... more Périneuriome extraneural ; Nez ; Nodule ; Storiforme Résumé Introduction.-Le périneuriome est une tumeur rare des gaines nerveuses périphériques, composée de cellules périneuriales. On en distingue deux grandes variétés : le périneuriome intraneural et le périneuriome extraneural. Nous rapportons un cas de périneuriome extraneural des tissus mous développé dans une localisation atypique, au niveau de l'aile du nez. Observation.-Un homme de 24 ans présentait sur l'aile gauche du nez un nodule de 1 cm évoluant depuis quatre ans. Histologiquement, il s'agissait d'une prolifération tumorale fusocellulaire d'architecture storiforme occupant le derme. Après exérèse, la lésion n'a pas récidivé. Commentaires.-Le périneuriome extraneural des tissus mous est une tumeur d'origine périneuriale, le plus souvent bénigne. Il s'agit en général d'un nodule cutané unique, siégeant le plus souvent au niveau des extrémités ou du tronc. La localisation au nez, comme chez notre patient, est rarement décrite. La tumeur est constituée histologiquement d'une prolifération fusocellulaire dermique bien limitée, d'architecture storiforme. Les cellules tumorales expriment l'epithelial membrane antigen (EMA) et non la protéine S100. Le traitement est chirurgical.
Background: Digital necrosis is a frequent vascular disease. Its very often reflects arterial dis... more Background: Digital necrosis is a frequent vascular disease. Its very often reflects arterial disorders of macro or mirocirculation. Paraneoplastic digital necrosis is rare, however. Aim: Report a new case of paraneoplastic digital necrosis Case: Our patient was a 62-year old diabetic female patient with a history of total thyroid ablation at 50 year old. She presented with bilateral digital necrosis of the second, third and fourth fingers. During her recovery, we have found a biological inflammatory syndrome and abdominal echography revealed a metastatic liver. Radiological investigations in search for the primitive tumour were negative. The patient was referred to a specialized center for a palliative chemotherapy. Conclusion: Etiologies of digital necrosis are various, mainly iatrogen, professional, toxic, atheroma or systemic disease. In case of no evident cause, explorations should be gone to detect underlying malignancies.
Background: Hyalinosis cutis et mucosae (HCM), is a rare autosomal recessive genodermatosis. Cuta... more 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.
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