[Extra-cutaneous systemic manifestations in generalized pustular psoriasis]. / Manifestations systémiques extracutanées du psoriasis pustuleux généralisé.

Generalized pustular psoriasis of the von Zumbusch type is a severe form of psoriasis characterized by disseminated pustular skin lesions with high fever and hyperleukocytosis. We report a 32-year-old woman who presented a generalized pustular psoriasis with extra-cutaneous manifestations of the disease that included pulmonary involvement, aseptic arthritis, jaundice, and liver abnormalities. The extra-cutaneous manifestations of generalized pustular psoriasis should be known to physicians caring for patients with psoriasis in order to avoid diagnostic delay.

Results of a public awareness campaign in Morocco regarding the sun's deleterious effects.

OBJECTIVE: To investigate knowledge of the various sun radiation-related risks and awareness of photoprotection measures using a sample of the Moroccan population. METHODS: An investigation of prevalence, which was administered while a public awareness campaign on the sun's harmful effects, organized by the department of dermatology of Ibn Sina's hospital in Rabat, was running. The collection of the data was made on the basis of a questionnaire that was filled by the team of the doctors of the department. The logistic regression allowed to identify the explanatory factors for the prevalent knowledge and behaviours related to the subject in hand. RESULTS: Four hundred eleven subjects were included in the survey. The subjects' average age was 40 +/- 10 years. Eighty-four per cent were female. The analysis of the total score of knowledge related to the sunrays' deleterious effects demonstrated low levels of knowledge among 160 participants (38.9%). Females were clearly more knowledgeable than the opposite sex (P = 0.003) and also those having a higher educational level (P = 0.006) scored high. The use of sunscreens was recognized by 52.8% of the patients and was associated with female gender and higher educational level. CONCLUSION: Our study reports the results of the first public awareness campaign on the sun's deleterious effects on the Moroccan population, inclusive and relevant to all ages. Awareness of the sun's radiation-related risks in a country as sunny as ours is low, whereas different measures of photoprotection are rather well known. Messages adapted to the characteristics of our population are a necessity.

Childhood cutaneous leishmaniasis: Experience of a Moroccan unit of dermatology.

OBJECTIVE: We undertook a monocentric retrospective analysis of childhood leishmaniasis in order to describe the epidemio-clinical profile, therapeutic characteristics and clinical outcomes of affected patients. PATIENTS AND METHODS: The files of all children treated for cutaneous leishmaniasis (CL) in the Dermatology Department of Ibn Sina University Hospital over an 11-year period were reviewed. RESULTS: A total of thirteen children were included. The mean age was 10.2 years and the sex ratio (F/M) was 5.5. All patients lived or had stayed in an endemic area. The average delay of diagnosis was 11.8 months. The most frequent clinical finding consisted of ulcers (61.5%), usually located on the face (69.2%). The clinical diagnosis was confirmed by parasitologic smear in 76.9 percent of cases. The treatment used was intralesional meglumine antimoniate in twelve cases. The outcome was favorable in all cases. CONCLUSION: Our series is characterized by a female predominance and the patients presented with a single lesion. The lesions were frequently located on the face and a favorable outcome was obtained after treatment with intralesional meglumine antimoniate.

Pemphigus and HLA in Morocco.

PURPOSE OF STUDY: Pemphigus is a group of autoimmune bullous dermatosis diseases characterized by autoantibodies against keratinocyte adhesion molecule. A significant association with HLA class II genes, particularly DR4 and DR14 has been described in many ethnic groups and countries. We have investigated, for the first time in Morocco the relationship between different pemphigus subtypes and HLA genes. PATIENTS AND METHODS: Fifty-two unrelated patients were compared to 178 healthy controls matched by age, sex and ethnic origin. HLA typing was performed by standard complement dependent microlymphocytotoxic method for class I and by sequence-specific primer amplification method for class II. RESULTS: No significant association was observed with any of the HLA-A or -B antigens. Generic typing showed a significant increase of DRB1*04 (p=0.002), DRB1*14 (p=0.003) and DQB1*03 (p=0.02) allele frequencies and significant decrease of DRB1*15 (p<0.0001) and DQB1*06 (p=0.01) allele frequencies. HLA-DRB1*15-DQB1*06 haplotype seems to confer a protective effect in our population while DRB1*04-DQB1*03 and DRB1*14-DQB1*05 haplotypes induced susceptibility to the disease. CONCLUSION: Taken together, our results confirmed the genetic predisposition to pemphigus. However, genetic factors are not sufficient to explain the high prevalence of pemphigus observed in the Moroccan population since alleles of susceptibility were similar to those commonly described in other populations throughout the world.

[Pseudoxanthoma elasticum and nephrocalcinosis]. / Pseudoxanthome élastique et néphrocalcinose.

INTRODUCTION: Pseudoxanthoma elasticum (PXE) is a hereditary disease that presents clinically as cutaneous, ocular and cardiovascular problems. The associated clinical signs are due to calcification of elastic fibres and blood vessels. The case we describe below involves a 24 year-old woman hospitalised for pseudoxanthoma elasticum and also diagnosed with asymptomatic renal nephrocalcinosis. OBSERVATION: A diagnosis of pseudoxanthoma elasticum was suspected in this patient based upon yellowish papules on the head and neckline together with angioid streaks. This diagnosis was confirmed by histological examination of the skin biopsy sample. Levels of calcium and phosphorus in blood and urine were normal, as were the cardiovascular test results. Abdominal ultrasound revealed bilateral nephrocalcinosis. DISCUSSION: This case is original in terms of the association of pseudoxanthoma elasticum with nephrocalcinosis. There are in fact increasing reports of diffuse visceral calcifications in pseudoxanthoma elasticum and the literature contains reports of association with mammary and testicular calcifications. To our knowledge, associated nephrocalcinosis has only been described in two subjects in a series of 11 PXE patients. The question is whether this association was entirely fortuitous or if it in fact constitutes a rare manifestation of the disease.

[Skin and menopause]. / Peau et ménopause.

Important changes related to declining level of several hormones occur during menopause: vasomotor instability, bone loss, anxiety, sexual dysfunction, skin aging... Our objective was a review of the literature concerning the histological and clinical changes seen in post menopausal skin, and also an analysis of the effect of hormonal replacement therapy in slowing down the aging process. Decline in progesterone increases the impact of androgen on the sebaceous glands and hair. Decreased estrogen slows down mitotic activity in the epidermal basal layer, reduces the synthesis of collagen and contributes to thickening of the dermo-epidermal junction. This hypoestrogenemia may be spontaneously attenuated by local synthesis of oestradiol in peripheral target tissues according to the intracrine process. This new hormonal pattern is associated with skin atrophy, hyperseborrhea, increased pilosity on the cheeks and upper lip, loss of scalp hair, increase in degeneration of elastic tissue, atrophy and dryness of the vaginal mucosa. Estrogen treatment in post menopausal women has been shown to increase collagen content, dermal thickness and elasticity. Biophysical properties are also significantly improved for the parameters reflecting hydration and sebum secretion. However, numerous side effects such as increased incidence of cancer and cardiovascular morbidity limit the use of this treatment. So non hormonal alternatives are proposed. Laser and lifting remain the most important options.

[Pseudoainhum and peripheral neuropathy]. / Pseudo-aïnhum et neuropathie axonale.

BACKGROUND: Pseudoainhum is a rare disease characterised by gradual fibrous constriction of the fingers and/or toes eventually resulting in their amputation. In this article, we report the first case seen in Morocco, highly unusual in terms of its severity. CASE REPORT: A 46-year-old woman with no toxic habits was hospitalised for spontaneous amputation of the fingers and toes. This condition began when the patient was 12 years old with the appearance of a circular constriction band at the base of the fifth toe, eventually resulting in its loss. The patient gradually lost all her other toes and fingers except for the first joint of her left index finger. There was no family history of any similar condition. Clinical examination also revealed perforating plantar disease in two of the stumps and peripheral neuropathy in all four limbs, comprising predominantly axonal disease responsive to electromyogram. Amputation of the index finger was completed and histological examination of the removed section showed nothing unusual, with no signs of diabetes. DISCUSSION: The peculiarity of our case resides in the exceptional severity of these amputations and their association with isolated polyneuropathy. Pseudoainhum has been described chiefly in patients with congenital keratoderma, certain systemic diseases, diabetes and alcohol dependence. A number of etiopathogenic hypotheses have been suggested: traumatic, infectious, vascular, neurological and genetic processes.

[Idiopathic hypereosinophilic syndrome and bullous pemphigoid]. / Syndrome hyperéosinophilique idiopathique et pemphigoïde.

BACKGROUND: We report the case of bullous pemphigoid associated with hypereosinophilic syndrome. This association has only been report only once in the literature. CASE REPORT: A 58 year-old man was admitted for a surinfected, pruriginous and generalized bullous dermatosis. Physical examination revealed bronchial rales. The cutaneous histology showed a junctional and intradermic cleavage associated with massive dermal infiltration by eosinophils. The diagnosis of a pemphigoid was confirmed by immunology. In parallel, the idiopathic hypereosinophilic syndrome was evoked in view of persisting hypereosinophilia without detected aetiology and associated with pulmonary infiltration. The skin lesions disappeared under symptomatic treatment, but the patient was rehospitalized 4 months later for severe relapse of dermatosis associated with medullary infiltration by eosinophils. Oral corticosteroid therapy gave spectacular results on both skin and blood formula. DISCUSSION: This association is rare, but seems to be related by the same immunological factors highlighting eosinophils. In addition, the presence of hypereosinophilic syndrome gives the pemphigoid some particular clinical, histological and evolutive characteristics.