[Pregnancy-specific dermatoses]. / Dermatoses de la grossesse.

Pregnancy-specific dermatoses include polymorphic eruption of pregnancy, atopic eczema of pregnancy, and pemphigoid gestationis. Intrahepatic cholestasis of pregnancy and impetigo herpetiformis are not real pregnancy-specific dermatoses but they are important to know considering the fetal and maternal risks. Polymorphic eruption of pregnancy is a pruritic disease that usually occurs in primiparous women during the last trimester of pregnancy. Atopic eczema of pregnancy is still controversial as an entity covering conditions with eczematous lesions, prurigo, or folliculitis, and inconstantly associated with a personal history of atopy. Skin biopsy with direct immunofluorescence or search for serum anti-BPAg1 (180kD) NC16a antibodies is mandatory in pruritic dermatoses of pregnancy in order to rule out pemphigoid gestationis. Serum bile salts levels should be tested whenever a generalized pruritus develops during pregnancy in order to rule out intrahepatic cholestasis.

[Neutrophilic dermatoses]. / Les dermatoses neutrophiliques.

Neutrophilic dermatoses are inflammatory disorders where normal neutrophils infiltrate the skin without infection. Sweet's syndrome, pyoderma gangrenosum, subcorneal pustulosis, erythema elevatum diutinum and a few other conditions are included in the spectrum of neutrophilic dermatoses. In addition to their typical presentation, atypical and overlap forms of these disorders may be observed. According to the location of the neutrophilic infiltrate, three clinical forms are distinguished: superficial (epidermal), en plaques (dermal) and deep (dermal and hypodermal). During the disease course, other tissues can be affected by the same neutrophilic infiltrate. An association with other systemic diseases including malignant blood disorders, inflammatory bowel diseases and autoimmune disorders is frequent. The mechanisms leading to the invasion of the skin by neutrophils remains not completely elucidated. Treatment depends on the intensity and the acute or chronic form of the disease. In acute and severe forms, systemic corticosteroids are first-line therapy, whereas colchicine, dapsone, and even topical corticosteroids could be used in milder presentations of the disease.

[Cutaneous side effects of EGF receptor inhibitors]. / Manifestations cutanées associées aux inhibiteurs du récepteur de l'EGF.

EGFR receptor inhibitors are used for the treatment of a number of solid tumors. Their cutaneous toxicity is a specific and frequent side effect that has shown to be correlated to the antitumoral effect. Here, we present an overview of the cutaneous manifestations and management of EGF receptor inhibitors toxicity.

[Novel adverse effects of laser-assisted axillary hair removal]. / Nouveaux effets indésirables du laser dépilatoire axillaire.

BACKGROUND: Several adverse effects of depilatory laser may now be commonly expected in relation to skin type and anatomic location. We report and analyse unusual events in patients undergoing laser-assisted axillary hair removal, including hyperhidrosis, bromhidrosis and leukotrichia. OBJECTIVE: The aim of this study was to examine a large patient population, assess the frequency of these adverse effects, and establish a correlation with different hair-removal laser protocols. PATIENTS AND METHODS: A retrospective case-control study of patients undergoing laser-assisted axillary hair removal was conducted to determine the incidence of these adverse effects in relation to skin type and types of laser used. RESULTS: Hyperhidrosis, bromhidrosis and leukotrichia were seen in 11, 4, and 2% of patients respectively. Hyperhidrosis was significantly less frequent in patients with skin types III and IV than in those with skin type II or V. Combined diode and alexandrite laser sessions were associated with a significantly higher incidence of hyperhidrosis compared to diode or alexandrite sessions alone. Regarding bromhidrosis and leukotrichia, no significant correlation with age, skin type or laser settings was revealed by the statistical analysis. CONCLUSION: Hyperhidrosis, bromhidrosis and leukotrichia are likely new adverse effects of laser-assisted axillary hair removal.

[Plasma cell panniculitis: a histopathological variant of morphea profunda]. / Hypodermite à plasmocytes, variante histologique de la morphée profonde.

BACKGROUND: Plasma cell panniculitis is a rare histopathological variant of morphea profunda. Its occurrence in two siblings has been reported once. We report a new case in which a brother and a sister with a family history of consanguinity presented plasma cell panniculitis of long duration. CASE REPORTS: A man and his sister, 38 and 28 years old respectively, presented thick hyperpigmented indurations of the trunk and thighs since puberty. Previous investigations did not lead to any definitive diagnosis. Deep cutaneous specimens taken from both patients exhibited indistinct histological features of plasma cell panniculitis. DISCUSSION: Morphea profunda is a deep-localized variant of scleroderma. The presence of predominant plasma cell infiltrate suggests a rare type of morphea profunda named plasma cell panniculitis. Subcutaneous plasma cell infiltrate has been described in many diseases such as lupus profundus and lymphoplasmocytic panniculitis of Lyme disease. The family history of consanguinity, similar age of onset and identical clinicopathological presentation suggest a genetic role in this disorder. This is the second report of plasma cell panniculitis in two siblings with a history of consanguinity.

[Anhidrotic ectodermal dysplasia. Report of a rare mutation in EDA1]. / Dysplasie ectodermique anhidrotique familiale: une mutation rare du gène EDA1.

BACKGROUND: Anhidrotic ectodermal dysplasia is a rare inherited disorder seen mainly in the X-linked recessive form. We report the case of a Lebanese family in which the mother transmitted an uncommon missense mutation to three of her sons. PATIENTS AND METHODS: A 23-year-old patient presented with keloids in nodular acne. The physical examination showed fine and sparse hair, thick everted lips and dental defects. A detailed history revealed congenital anhidrosis. The patient's, seven-year-old and four-year-old brothers had the same characteristic facial morphology and were also presenting anhidrosis. The mother had hypodontia. The parents, though not consanguineous, were from the same village. Genetic testing with sequencing of the EDA1 gene revealed a missense mutation affecting codon 155. DISCUSSION: Ectodermal dysplasias are currently found in more than 150 syndromes. The patient's history and the clinical signs suggest the X-linked recessive form of anhidrotic ectodermal dysplasia due to a mutation in EDA1 gene encoding the ectodysplasin. The mutation found in this family is very rare and was mentioned once in a study on splicing forms that permit detection of all EDA1 mutations. Besides, this patient tolerated oral isotretinoin perfectly well, unlike another case reported once in the literature. Finally, genetic counselors must inform carrier mothers of the high recurrence rate among male offspring.

[Keratosis lichenoides chronica in two siblings with marked response to UVB phototherapy]. / Deux cas de kératose lichénoïde chronique dans une même fratrie. Réponse favorable à la photothérapie UVB.

BACKGROUND: Keratosis lichenoides chronica (KLC) is a rare chronic keratinisation disorder. Out of almost 60 published cases of KLC, only two report its occurrence in siblings. We report a new case in which a brother and sister present KLC that improved markedly with UVB phototherapy. PATIENTS AND METHODS: A 10-year-old girl presented with hyperkeratotic papules symmetrically arranged in a reticular pattern on the face and the extremities. Onset occurred at the age of six months. For a number of years, minimum sunlight was allowed owing to a diagnosis of lupus. However, KLC was our first diagnostic assumption, confirmed by an elbow lesion biopsy sample. The patient's brother, one and a half years old, had been presenting similar lesions since the age of two months. Sibship was demonstrated by DNA analysis using short tandem repeat markers. No consanguinity was found. After one month of narrow-band UVB phototherapy, most of the papules had flattened. DISCUSSION: KLC is uncommon in childhood and familial occurrence is very rare. Clinically, thick keratotic papules arranged in parallel lines or small networks cover the dorsal aspects of the limbs symmetrically. The face may be affected by a seborrhoea-like dermatitis. Histology typically shows alternating acanthosis and atrophy, with focal parakeratosis. An extensive lichenoid lymphohistiocytic and plasmocytic infiltrate is demonstrated in the dermis. The course is chronic. Spontaneous resolution may occur. Sunlight has been shown to be effective in the few paediatric cases reported. Narrow-band UVB phototherapy appears to be an effective therapeutic option.