Linear IgA bullous dermatosis following Oxford AstraZeneca COVID-19 vaccine.

A case of linear IgA bullous dermatosis developing 3 days after the second dose of Oxford AstraZeneca COVID-19 vaccine in an adult patient, suggesting a possible causal association. It is worth keeping in mind that COVID-19 vaccination could induce immune-mediated bullous disease in susceptible people.

Risk factors in facial hyperpigmentation in Maghrebian population - a case-control study.

Triggering factors seem to be multiple in the pathogenesis of facial hyperpigmentation (FH), as dark skin types, pregnancy, sun exposure… The aim of this study was to investigate the risk factors for FH in Maghrebian population. Patients with FH were included in a case-controlled study. Following data were recorded: age, sex, parity, phototype, FH and hormonal-related history, endocrinological features, sunlight exposure, specific cultural behaviours, use of topic corticosteroids and sunscreens. One hundred women (mean age 29.5 ± 13.7 years) were included with 200 age-matched controls. Duration of FH was 32.9 ± 42.2 months. There was a statistically significant relation between FH and hirsutism (P = 0.009), troubles of menstruations (P = 0.008), but not with acne (P = 0.23) and contraceptive oral (P = 0.06) drugs or with history of thyroid disorders (P = 0.13). For cultural factors, there were a statistical significant relation with rubbing by flannel glove (P < 0.05), use of dark soap (P = 0.009) and traditional masks (P < 0.05) but both groups were used to go to hammam. A strong relation was observed between FH and use of topical corticosteroids: 40% in the FH group vs. 5% in the control group (P < 0.05). Both groups used to apply sunscreens: 70% in the FH group and 67% in the control group where as there were no differences in the two groups for sun exposure. No statistical differences were observed regarding to age, phototypes and grade of parity. Our study demonstrated a relation between FH and hyperestrogenemia, rubbing with a friction glove, use of moroccan traditional masks and application of topical steroids. The eviction of all these triggering factors could be an adjuvant recommendation in the assessment of FH.

[Malignant pyoderma with cranial osteolysis]. / Pyoderma malin compliqué d'ostéolyse crânienne.

BACKGROUND: Malignant pyoderma or pyoderma gangrenosum with cephalic involvement is a rare disease characterized by chronic aggressive local progression. We report a case of malignant pyoderma with cranial osteolysis. PATIENTS AND METHODS: A 42-year-old man had been presenting chronic ulcerative dermatosis involving the entire scalp since the age of sixteen. Clinical examination, histopathological analysis of the skin sample and negative aetiological tests suggested a diagnosis of cephalic pyoderma gangrenosum. Different treatments were ineffective. The patient was subsequently admitted with convulsions revealing cranial osteolysis. A cranial scan confirmed the osteolysis and histopathological analysis of the bone sample revealed non-specific suppurative osteitis. Treatment with corticosteroids and thalidomide was effective. Skin and bone grafting was proposed but was not carried out due to the chronic progression and absence of stable wound healing. DISCUSSION: Malignant pyoderma is a rare destructive, ulcerating dermatosis. It is characterized by a chronic course and resistance to treatment. Our case report is original in terms of the involvement of underlying cranial bone revealed by convulsions. This bone involvement makes treatment of the dermatosis more difficult.

[Prevalence of syphilis and HIV infection in female prison population in Morocco]. / Prévalence de la syphilis et de l'infection à VIH dans une population carcérale féminine au Maroc.

We studied the prevalence of syphilis and HIV infection in 217 female prisoners at Oukacha prison, Casablanca and associated risk factors. The mean age was 32.0 years and mean length of imprisonment 22.8 months. As regards risk factors, 9% of the women said they used condoms, homosexuality was not admitted, 1.8% were intravenous drug users and the average number of sexual partners was 5.17. Fifty-one women (23%) were positive for syphilis and 4 (2%) were HIV-positive. Multivariate analysis found that being married was protective (OR = 0.75, 95% CI: 0.57-0.97), while high number of partners was a positive risk factor (OR = 2, 95% CI: 1.2-3.51).

[Childhood cutaneous tuberculosis from Morocco: a study of 30 cases]. / Tuberculose cutanée de l'enfant au Maroc: étude de 30 cas.

UNLABELLED: In Morocco, tuberculosis is still endemic. Cutaneous tuberculosis is ranged the fifth after the pleuro-pulmonary, lymphe node, urogenital and digestive tuberculosis. It mainly affects young people. Few studies of cutaneous tuberculosis are available in this age group. The aim of our study is to emphasize its epidemiological features. PATIENTS AND METHODS: It is a retrospective study including all cases of childhood cutaneous tuberculosis observed between January 1981 and December 2004. The diagnosis was based on the confrontation of clinical, immunological, bacteriological and histological data. RESULTS: Thirty cases were collected. The mean age was 11 years. The clinical features are as follows: gumma 46.6%, scrofuloderma 36.6%, lupus vulgaris 13.3% and skin tuberculosis chancre 3.3%. The Mantoux test was positive in 67%, tuberculoid granuloma was observed in 78% and the search of Mycobacterium tuberculosis was positive in 13%. DISCUSSION: Gumma and scrofuloderma were the most frequent forms as in other Moroccan series. These results attest the endemicity of this affection in our country. The diagnosis relies on the discovery of Mycobacterium tuberculosis; as this situation is rare for the cutaneous location, the diagnosis relies then on the association of clinical and paraclinical criteria.

[Precocious mediastinal lipomatosis: a rare complication of systemic corticosteroid therapy]. / Lipomatose médiastinale précoce: une complication exceptionnelle de la corticothérapie generale.

BACKGROUND: Corticosteroid-induced lipomatosis results from hypertrophy within adipose tissue; the condition is frequently asymptomatic and its incidence is underestimated. We report a case of mediastinal lipomatosis that is rare in terms of both site and presenting symptoms. CASE REPORT: A 46-year-old woman with no disease history other than obesity with a weight of 90 kg had been treated since 2002 for mixed connective tissue disease (profound lupus and dermatomyositis). She had been treated with oral corti costeroids (1 mg/kg/d). Two months after the start of treatment, she presented chest pains, resting dyspnea particularly aggravated in dorsal decubitus, chest edema in the subclavicular space and jugular turgescence. Chest x-ray revealed widening of all levels of the mediastinum. The chest CT scan showed lipomatosis throughout the entire mediastinum with no associated chest abnormalities or pericardial effusion. Rapid downward dosage adjustment ofcorticosteroids to 10 mg/d coupled with synthetic antimalarials resulted in gradual reduction of symptoms. The chest scan performed two months later short stabilization of the patient's mediastinal lipomatosis. DISCUSSION: The effects of long-term of glucocorticosteroid therapy are well-known, in particular Cushing's syndrome. Lipomatosis has been described more recently and affects different axial regions. Mediastinal localization is seen in 15% of patients treated. This presentation is less common than orbital and epidural localizations. Although often asymptomatic, as in our own report, it may present with worrying symptoms that pose real diagnostic problems. The diagnostic examinations of choice are CT scan or MRI. Regression following discontinuation or reduction of corticosteroids is inconsistent and often gradual.

[Intertoe space cutaneous epidermoid carcinoma]. / Carcinome épidermoïde interorteil.

BACKGROUND: Localisation of epidermoid carcinoma in the intertoe space is very rare. We report a case occurring in a setting of chronic intertoe intertrigo. CASE: A 66 year-old woman presenting intertoe intertrigo for 30 years consulted for painful ulceration of the 3rd intertoe space of the left foot present for 4 years and refractory to antifungal treatment. The examination revealed ulceration with infiltrated edges occupying the entire 3rd intertoe space, with no satellite adenopathy. Histological examination of the skin biopsy sample revealed epidermoid carcinoma. Wide excision of the lesion was performed with a safety margin of 5 mm, with removal of the 3rd and 4th left toes. The outcome was favourable with no relapse after 12 months. DISCUSSION: A single publication reported a series of 22 cases of intertoe epidermoid carcinoma in which the key aetiological feature of tumour was continual maceration. In this patient, maceration was maintained by a number of factors, including cultural factors: ritual ablutions performed 5 times daily, with no drying of the intertoe spaces, and domestic cleaning involving large quantities of water several times weekly.

[Terbinafine-induced Stevens-Johnson syndrome and aggravation of systemic lupus erythematosus]. / Syndrome de Stevens-Johnson et aggravation d'un lupus systémique induits par la terbinafine.

BACKGROUND: There have been published reports of induction and aggravation of subacute systemic lupus erythematosus of lupus by terbinafine. We report the case of female patient with systemic lupus erythematosus who, after treatment with terbinafine, presented a Stevens Johnson syndrome eruption, together with renal exacerbation of her connective tissue disease. OBSERVATION: A 25 - year-old woman was followed for 4 years for systemic lupus erythematosus was no renal involvement. The patient was stable under corticosteroids (20 mg/d) and chloroquine (200 mg/d). She was treated with terbinafine onychomycosis caused by Trichophyton rubrum. Seven days after trunk and limbs, with cheilitis affecting the mucous membrane and bilateral conjunctivitis. This rash was followed by epidermolysis involving 10% of the cutaneous area. Stevens-Johnson syndrome was diagnosed. Laboratory tests indicated massive hematuria and proteinuria, and the renal needle biopsy sample showed signs of class III lupus glomerulonephritis. The anti-histone antibodies were highly positive. The patient was treated with systemic corticosteroids (1 mg/kg/d), chloroquine was recommenced and cyclophosphamides were given in a bolus. The outcome of the patient's eruption and lupus was favorable. DISCUSSION: Although induction and worsening of lupus by terbinafine have already been reported, the present case differs through the concomitant occurrence of eruption on treatment with terbinafine and severe relapse of lupus, thus suggesting the involvement of a shared immunological mechanism.

[Ocular manifestation of Behçet disease (study of 50 patients consulting in dermatology department)]. / Les manifestations oculaires de la maladie de Behçet (étude de 50 patients consultant en dermatologie).

INTRODUCTION: Behçet disease is a multisystemic vascularitis. Ocular affection is one of the major criteria of this disease. The aim of this study is to specify the clinical, therapeutical characteristics and the prognosis factors of the ocular affection in patients having BD and admitted to the dermatology department. PATIENTS AND METHODS: It is a retrospective investigation carried out in the dermatology department of Ibn Rochd university hospital center of Casablanca, Morroco, from Jannuary 1990 until December 2003. Two patient groups have been distinguished. The first one involved 50 patients (44,2%) having BD with ocular affection, and the second group involved 63 patients having BD without ocular affection. RESULTS: The mean age was 29 +/- 8 years in the first group VS 30 +/- 7,9 years in the 2(nd) group. The ocular affection was more frequent in males than in females (P < 0.05). The ocular manifestations were marked by uveitis and retinal periphlebitis. The frequency of the cutaneomucosal and joint manifestations was similar in both groups, whereas neurologic and vascular with ocular affections. The choice of the treatment depended on the type of ocular affection. Evolution was marked by blindness in 6 patients (12%). DISCUSSION: The ocular affection comes second after the cutanous mucuous affection. Males are more clearly affected than females. This allows saying that there is a marked effect of the sexual hormones on the ocular affection. Age is not predictive of this ocular affection. The ocular affection was severe in our series and was dominated by uveitis and vascularitis. We insist on the severity of ocular Behçet and its evolution? Risk toward blindness especially concerning young man. Currently, the treatment is not codified; however, the encouraging outcome obtained with some immunosuppressive therapies would be better if this treatment was set up early. CONCLUSION: This study enebed us to re-examine the ocular manifestations of the Behçet disease in the Maroccan population by the means of a consultation of Dermatology. Il should be noted that it is worse forecast because on the one hand of its frequent association to vascular and neurological affections and other share of the delay of consultation noted at the majority of our patients.

[Quality of life and pemphigus]. / Qualité de vie et pemphigus.

INTRODUCTION: Pemphigus is a frequent chronic bullous dermatosis in Morocco that predominantly affects young women. It requires serious therapeutic management and often repeated hospitalizations. The purpose of this study was to assess the particular impact of pemphigus on quality of life by comparing the quality of life in patients suffering from pemphigus with that of the general population using a generic instrument of measure of health related quality of life: the SF-36, in association with a questionnaire exploring the impact of the disease on self perception, social relationship and behaviour. PARTICIPANTS AND METHODS: A French version of the SF-36 underwent 2 translations from French to Moroccan dialect, then two retrograde translations. After a pre-test, the questionnaire survey was administered by the same investigator to 30 patients who suffered from pemphigus, followed-up in the Ibn Rochd UHC in Casablanca and to 60 healthy adults. The two samples were paired according to age and sex. RESULTS: In the pemphigus group, there was a significant decrease of mean scores of all the SF-36 dimensions, except for physical pain and alteration in general status of health. The greatest alteration concerned the impact on physical and emotional status (p<0.00001) and the smallest alteration concerned the perception of general health (p=0.02). The factors influencing this impact were: profession, face involvement and extent of lesions. Other results concerning the psychosocial impact of pemphigus are presented. CONCLUSION: Our study confirmed that pemphigus is responsible for great alterations in health related quality of life. Therefore, the management of this disease must take into account its impact on various fields of life of the patients.

Lamellar ichthyosis: further narrowing, physical and expression mapping of the chromosome 2 candidate locus.

Lamellar ichthyosis (LI) is an autosomal recessive genodermatosis which has been shown to be both clinically and genetically heterogeneous. Keratinocyte transglutaminase (or transglutaminase 1: TGM1) has been demonstrated to be the disease-causing gene in some families, whilst in others, a second unidentified LI gene was mapped to chromosome 2q33-35 (ICR2B locus). In this study, we present a physical map that encompasses the ICR2B locus, including the mapping of new microsatellite markers. Based on this new map, genotyping additional families highly suggests a reduction in size of the candidate interval. The final interval is covered by a single yeast artificial chromosome (937-H-3) which is 2.2Mb in length. Fine mapping of potential candidate transcripts was also focused on this region.

Novel recurrent nonsense mutation causing neurofibromatosis type 1 (NF1) in a family segregating both NF1 and Noonan syndrome.

Neurofibromatosis type 1 (NF1), a genetic disorder with neuroectodermal involvement, demonstrates phenotypic overlap in some patients with Noonan syndrome (NS), ultimately resulting in the so-called neurofibromatosis-Noonan syndrome (NF-NS). A strong association of the two phenotypic traits was recently illustrated by a four-generation family, although NF1 and NS were eventually demonstrated to segregate independently on the basis of polymorphic DNA markers [Bahuau et al., 1996: Am J Med Genet 66:347-355]. Identification of the causal NF1 mutation seemed a prerequisite to further dissecting this singular familial association. Using the protein truncation assay, a nonsense mutation (C2446T-->R816X) of the neurofibromin gene was evidenced. This mutation occurred on a CpG dinucleotide within exon 16 and 5' to the GAP domain-specifying region of the gene. R816X creates a recognition site for endonuclease HphI, absent in 2 individuals with NS only. Screening 184 unrelated NF1 patients, three novel occurrences of the mutation were found in individuals diagnosed with classical NF1. Based on the assumption of genotype-phenotype correlation in these individuals, clinical and molecular analyses of this four-generation family demonstrated that the NF-NS phenotype was additive, being the result of both classical NF1 and NS. This particular observation also suggests the presence of an NS locus on 17q, which might be of interest for further linkage studies.

[Sunscreens: use in pregnant women at Casablanca]. / Photoprotecteurs externes. Utilisation chez la femme enceinte à Casablanca.

BACKGROUND: Photoprotection with sunscreens is recommended in hyperpigmentation, particularly in pregnancy-related form. The aim of this study was to evaluate the use of sunscreens in pregnant women and to determine whether melasma was correlated to sunscreen use. PATIENTS AND METHODS: Four hundred and fifteen pregnant women were surveyed by a standardized questionnaire on the presence of melasma and their use of sunscreens. The study took place in Casablanca, Morocco, in two public maternity hospitals and two private gynaecologic centres, from April 1999 to July 1999, during antenatal consultations. RESULTS: The mean age of the 415 pregnant women was 29 +/- 6 years. One hundred and fifty-one women (37 p. 100) had a melasma and 72 (17 p. 100) used a sunscreen at their inclusion in the study. Women with melasma used sunscreens in 18.8 p. 100 and the others in 16.6 p. 100. Users of sunscreens differed from non-users in their higher level of instruction, professional activity, presentation in private practice and of urban origin. DISCUSSION: This study showed a low incidence of sunscreen use in our population of pregnant women. Melasma wasn't a reason for using sunscreens. However, access to medical information, especially to photoprotection advice, and the financial possibility of buying the product led to larger use of sunscreens. Up to now no study has confirmed that sunscreens can prevent melasma in dark phototype women with pregnancy. It would therefore be difficult to recommend them to pregnant women.

[Comparison of diagnostic criteria in Moroccan patients with Behçet's disease]. / Comparison des critères diagnostiques de la maladie de Behçet chez des malades marocains.

BACKGROUND: Behçet's disease is a vasculitis with diverse systemic manifestations usually involving the skin, eyes, joints and nerves. Clinical diagnosis is based on several signs, generally using the criteria established by the International Group for the Study of Behçet's disease, by O'Duffy, by Hamza, by Mason and Barnes and by the Japanese Committee. The purpose of this retrospective study was to assess the frequency of clinical manifestations in Behçet's disease and to apply the five diagnostic criteria scorings cited above to our patients to determine their sensitivity. PATIENTS AND METHODS: Between January 1988 and December 1997, 110 cases of Behçet's disease were seen at our Dermatology Unit in Casablanca as inpatients or outpatients. Complete history and clinical data including results of the ophthalmology exam and skin tests were available for 70 patients (29 men and 41 women, mean age 31 years). RESULTS: All 70 patients had buccal aphtosis and 88 p. 100 had genital aphtosis. Ocular signs were found in 36 p. 100 of the patients and the skin test was positive in 57 p. 100. According to the criteria of the International Group for the Study of Behçet's disease, 65 patients (93 p. 100) had Behçet's disease. For the Japanese Committee criteria there were 63 (90 p. 100), for the O'Duffy criteria 48 (68 p. 100), for Hamza criteria 45 (64 p. 100) and for Mason and Barnes criteria 35 (50 p. 100). DISCUSSION: Our series is comparable to other Mediterranean series observed in dermatology units, but our patients had fewer systemic manifestations compared with another Moroccan series reported from an internal medicine ward. This could be due to recruitment bias and different medical education. For positive diagnosis of Behçet's disease, the criteria established by the International Group for the Study of Behçet's disease, the Japanese Committee, O'Duffy and Hamza enabled the diagnosis in the majority of the cases. Fifty percent of the patients had all the criteria described in these scores. These criteria would appear to be both overly descriptive, with the risk of confounding other disease states such as enterocolopathy, or underly descriptive, making it impossible to classify certain patients as having Behçet's disease.

[Lichen planus and hepatitis C]. / Lichen plan et hépatite virale C.

Cases associating lichen planus and hepatitis C are increasingly being reported. We observed three new cases, two males and one female aged 46, 63 and 56 years respectively. In all three cases, the lichen planus was limited to the oral cavity with erosion in two cases. Hepatitis C was confirmed by 2nd generation ELISA. Two of the patients consulted for lichen planus during the recovery phase after hepatitis C. In the third patient, lichen planus preceded the hepatitis. These three cases are further evidence favouring the hypothesis of a non-fortuitous association between lichen planus and hepatitis C. The hepatitis C virus might induce an immune reaction leading both to lichen planus and chronic hepatitis.

[Juvenile hyaline fibromatosis: 2 twin brothers affected]. / Fibromatose hyaline juvénile. Atteinte de 2 jumeaux.

INTRODUCTION: Juvenile hyaline fibromatosis is a rare hereditary genetic disease characterized by a cutaneous tumoral fibroblastic proliferation with joint involvement. CASE-REPORT: We report the case of 2 homozygous twin brothers both presenting a diffuse and invalidating form of juvenile hyaline fibromatosis. The 2 boys' born of a twin pregnancy' presented at the age of 2 months a clinical picture made up by the classic association: infiltrated papulo-nodules, gingival hyperplasia and joint contracture. The diagnosis of juvenile hyaline fibromatosis suggested clinically was confirmed by the histological study of the skin. DISCUSSION: These observations of juvenile hyaline fibromatosis's are particular in the involvement of 2 twin brothers, which to our knowledge has never been reported, the resemblance of the clinical features, and the severity of the functional handicap.

[Tabetic arthropathy: "a forgotten disease"]. / Arthropathie tabétique: "une maladie oubliée"

INTRODUCTION: With the current sparcity of syphilitic manifestations, the once obvious aspects of the affection have been forgotten or are little known. We report 4 cases of tabetic arthropathy admitted to the Dermatology Department Ibn Rochd University Hospital of Casablanca. OBSERVATIONS: The mean age of the patients was 50 years, all were males. The diagnosis was based on clinical, radiological, and biological criteria. There was a large contrast between the extent of the articular malformation and the indolence. The radiological aspect associated destructive and constructive lesions. The syphilis serology was positive in the articular liquid, the blood and/or the cerebro-spinal fluid. Treatment was essentially medical. DISCUSSION: Tabetic arthropathy, an exceptional affection, is one of the complications of the tabes dorsalis. Its prevention relies on the treatment of the syphilis at an early stage, before the onset of the articular and neurologic forms, the management of which remains very difficult.

[Herpetic superinfection of pemphigus: 6 cases]. / Surinfection herpétique du pemphigus: six cas.

BACKGROUND: Herpes simplex infection should be searched for in patients who experience unexplained aggravation of acantholytic dermatosis. Epidermal alterations appear to play an important favoring role. PATIENTS AND METHODS: We report 6 cases of Herpes simplex infections in patients with pemphigus observed at the Ibn Rochd hospital dermatology unit, Casablanca, over a 3-year period (1995-1998). RESULTS: There were 4 men and 2 women, age range 27-76 years. Superficial pemphigus was observed in 5 cases and pemphigus vulgaris in 1. In all cases, histology showed cytopathogenic Herpes simplex infection. The Herpes simplex infection appeared when the patients were given general corticosteroid therapy at doses from 1 to 1.5 mg/kg/d for 15 days to 1 month. In two cases, the corticosteroids were associated with an immunosuppressor (azathioprine, cyclophosphamide). Cure was rapid with systemic aciclovir, achieved in 5 to 20 days. Recurrence was observed in 2 cases. DISCUSSION: Herpes simplex superinfection in patients with pemphigus is classically described but uncommonly observed. In our experience, it has become more frequent over recent years since, before 1990, we observed no cases. Its prevalence may be underestimated. The diagnosis of Herpes simplex superinfection is sometimes difficult and should be suggested in case of relapse or resistance to treatment. The Herpes virus simulates most cases of pemphigus relapse, with a preferential localization on the bullous lesions. The painful nature of the eruption is highly suggestive of Herpes simplex infection as is the presence of vesicles grouped in bouquets. Both type 1 and type 2 viruses are observed. Cure is rapidly achieved with aciclovir in 5 to 15 days. Spontaneous cure has been reported in the literature as well as recurrent herpetic infection. In two cases in the literature, the Herpes eruption was fatal due to severe herpetic hepatitis and disseminated intravascular coagulation. Herpes simplex infection must be rapidly recognized in pemphigus patients so curative aciclovir treatment can be given early.

[Loose anagen hair syndrome: a familial case with fetal hair in meconium]. / Syndrome des cheveux anagènes caducs. Une observation familiale avec emission de cheveux foetaux dans le meconium.

INTRODUCTION: Loose anagen hair syndrome is a recently described hair disorder. We report a familial case. CASE REPORT: Two sisters were examined, 4 years-old and 8 months old, from a consanguineous marriage. At birth, they presented diffuse alopecia of the scalp; the meconium contained black and silky hair. Clinical examination noted the short, fine hair with easy and painless uprooting. No clinical associated symptoms were observed. The trichogram showed 70 p. 100 of anagen hair and 30 p. 100 of telogen hairs. DISCUSSION: Our familial case is original by this clinical symptom: discharge of probably dystrophic foetal hair in the meconium. Loose anagen hair is characterised by easily pluckable hair. The trichogram confirms the diagnostic. It is composed of anagen hairs (80 to 100 p. 100) devoid of sheaths. The loose anagen hair is usually isolated, but associations have been reported. It may occur in families. The precise pathogenesis and treatment of this hair disorder are not known.