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.

Pseudotumoral cutaneous aspergillosis in chronic granulomatous disease, report of a pediatric case.

Invasive aspergillosis is a life-threatening condition in patients with chronic granulomatous disease (CGD). Skin invasion by Aspergillus occurs most commonly by contiguity to a neighboring cavity. We describe an unusual case of invasive cutaneous aspergillosis presented as a large burgeoning tumor in a 4-year-old girl with CGD who underwent surgical treatment for bifocal osteomyelitis of the left leg.

Oral HPV infection and MHC class II deficiency (A study of two cases with atypical outcome).

Background: Major histocompatibility complex class II deficiency, also referred to as bare lymphocyte syndrome is a rare primary Immunodeficiency disorder characterized by a profondly deficient human leukocyte antigen class II expression and a lack of cellular and humoral immune responses to foreign antigens. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections. The infections begin in the first year of life and involve usually the respiratory system and the gastrointestinal tract.

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.

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.

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.

Transient symptomatic zinc deficiency in a breast-fed infant: relevance of a genetic study.

Objective: We present a case of acrodermatitis enteropathica in a full-term, breast-fed, 7-mo-old infant born from consanguineous parents with a family history of acrodermatitis enteropathica.

Methods: The patient presented with periorificial and symmetric acral lesions, which prompted us to review the clinical features of acrodermatitis enteropathica and its pathogenesis. Laboratory investigations showed low zinc levels in the infant's and mother's sera and in the mothers' milk.

[Kindler syndrome: clinical and ultra-structural particularities, a propos of three cases].

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.

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.

Acrodermatitis enteropathica: a review of 29 Tunisian cases.

Introduction: Acrodermatitis enteropathica is a rare autosomal recessive disease due to an abnormality in a zinc transporting molecule.

Methods: We conducted a retrospective monocentric study on 29 Tunisian cases of Acrodermatitis enteropathica (AE) treated in our Department of Dermatology in Tunisia, between January 1981 and June 2008.

Results: The age of onset of disorders was between 15 d and 12 months (mean 6.

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.

[Bullosis diabeticorum: report of ten cases].

Background: Bullosis diabeticorum is a rare characteristic complication of diabetes mellitus; it affects 0.5% of diabetics. Bullosis diabeticorum is a manifestation of complicate and longstanding diabetes mellitus.

[Localized scleroderma: a retrospective study about 92 cases].

Background: Sclerodermas are rare affections which can be located or generalized. Localized form is the most frequent.

Aim: The purpose of this study was to describe epidemiologic, clinics, biological, immunological, therapeutic, evolutionary characteristics of the localized scleroderma through a personal series and the data of the literature.

[Orf of the hand].

Introduction: The orf is a zoonotic infection which can be transmitted to humans.

Objective: The aim of our report is to describe this often misdiagnosed viral infection in a man with an orf of the hand appearing after the feast of the sacrifice.

Case Report: A 40-year-old man with no past medical history, presented with a nodular lesion on the fifth right finger evolving for 3 weeks which had appeared 2 weeks after the feast of the sacrifice.

[What is new in the treatment of vitiligo].

Background: Vitiligo is a frequent hypomelanosis that affects 1% of the world population, and 0,95% of the tunisian population.

Objective: The objective of our study is to describe through a recent review of the literature, the different therapeutic modalities, now used in vitiligo. We have used a clinical approch to guid therapeutic indications.

[Hereditary ichthyosis in Tunisia: epidemiological study of 60 cases].

Background: Ichthyosis are a group of inherited keratinizing disorders. The cutaneous abnormalities may be isolated or associated with extra-cutaneous symptoms.

Aim: To report the epidemiological and clinical profiles of patients with these genodermatoses from a hospital tunisian study.

[Isolated cutaneous pigmentation: adrenal insufficiency may be the cause].

Introduction: Addison's disease is a primary global deficiency in adrenocortical hormones resulting from the progressive total destruction of the adrenal glands.

Case: A 15-year-old girl consulted after four months of pigmentation on sun-exposed areas, with photosensitivity. She reported no weakness, no decline in her general status, and no menstrual disorders.

[Scleredema of Buschke. A case report].

Scleredema of Buschke is a rare disease whose aetiology remains unknown. It is characterised by symetric skin induration of the upper part of the body which usually resolve spontaneously. It can be associated with extra-cutaneous symptoms.

[Foot and diabetic neuropathy: about 205 cases].

Diabetic neuropathy is a serious and disabling complication of diabetes mellitus. It occurs in all types and at any moment of the evolution of diabetes. It can be worsened by arteriopathy or an associated cutaneous infection.

[Multiple basal cell carcinomas following radiation therapy for nasopharyngeal cancer].

Introduction: Basal cell carcinoma (BCC) is a cutaneous, generally primary malignancy, most common among the elderly. We report the case of a patient presenting numerous BCCs several years after radiation therapy for nasopharyngeal cancer and discuss the risk factors for this tumor and the role played by radiation in its genesis.

Case: A 30-year-old woman presented with pigmented facial lesions that had been developing over the past year.

[Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 49 Tunisian cases].

This is a retrospective study about 49 cases of xeroderma pigmentosum (XP). The goal was to determine the clinical features of XP in Tunisia. Our study revealed the predominance of a moderate form.