Fractional laser for vitiligo treated by 10,600 nm ablative fractional carbon dioxide laser followed by sun exposure.

BACKGROUND: Vitiligo is an acquired disorder of the skin and mucous membranes. Many patients with vitiligo remain in the refractory state despite the availability of numerous potential treatments. To the best of our knowledge, only one trial considers ablative fractional CO2 laser in the treatment of vitiligo. OBJECTIVE: To investigate the effects of fractional CO2 laser followed by systemic sun exposure on non-segmental vitiligo (NSV). METHODS: Ten patients presenting refractory NSV were enrolled in this study. The patients underwent three sessions, one month apart, of fractional CO2 laser therapy on the affected areas of the skin (L-group). Five days after each laser treatment, patients were asked to expose themselves to the sun for 2 hours on a daily basis. Objective and subjective clinical assessments were performed at the beginning and at the end of the treatment. The L-group was then compared to a control group (C-group) that consisted of vitiligo lesions in the same patients but with sun exposure as the exclusive therapy. RESULTS: Compared to the C-group, the L-group showed better improvement in both objective and subjective assessments. There were no noticeable adverse events in terms of scarring and Koebner phenomenon among others. CONCLUSIONS: All patients treated with both, laser sessions and sun exposure, improved their chronic NSV lesions. Improvement was less significant in patients who exhibited vitiligo lesions over articular surfaces such as elbows and underarms. The best results were observed in vitiligo plaques located on the face, neck and legs. Consequently, fractional CO2 laser followed by sun exposure could be considered as an alternative modality for the treatment of refractory vitiligo, especially in sunny regions.

Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome.

Netherton syndrome (NS) is a rare and severe autosomal recessive ichthyosis. We report the case of a patient with NS treated successfully with narrowband ultraviolet B (NB-UVB) phototherapy after failure of low-dose oral isotretinoin. A 16-year-old girl born to consanguineous parents presented with severe ichthyosis. On examination of her hair, she was found to have trichorrhexis invaginata. Genetic analysis revealed a SPINK5 mutation specific for NS. The patient's cousin had the same mutation. The patient developed Cushing syndrome after long-term application of topical steroids. She was treated with low-dose oral isotretinoin with no benefit. NB-UVB phototherapy was started, which resulted in marked improvement after 2 months. In conclusion, we report a case of NS with failure of low-dose oral isotretinoin, for which NB-UVB phototherapy, through its apoptosis-inducing and immunomodulatory effects, was effective. However, long-term use of UVB is limited by its potential side-effects.

[Oral lesions in psoriasis]. / Manifestations buccales du psoriasis.

BACKGROUND: The literature contains little information about the incidence or prevalence of oral signs in psoriasis. The pustular forms are the most commonly incriminated, with geographic tongue being the most frequently cited oral sign. The aim of our study was to determine the various types of oral signs seen in a large population of psoriasis patients. PATIENTS AND METHODS: Four hundred psoriasis patients were recruited consecutively and a detailed oral examination performed by two clinicians. Similar examinations were performed over the same period in 1000 nonpsoriasis subjects comprising a control group in order to assess the incidence of the same oral signs in the general population. RESULTS: Comparing the two groups, fissured tongue was seen in 33.2% of psoriasis patients versus 9.9% of control subjects (P<0.0001). There was thus a highly significant (P<0.0001) correlation between geographic tongue and psoriasis (7.7% of psoriasis patients versus 1% of controls). In addition, a strong correlation was seen between the presence of pustular psoriasis and fissured tongue (83.3% of patients with pustular psoriasis versus only 30% of patients with other forms of psoriasis). DISCUSSION: Our study shows a strong correlation between psoriasis and fissured and geographic tongue, although these features are not pathognomonic for the disease. As regards geographic tongue, our results are consistent with the data in the literature. However, the prevalence of fissured tongue was considerably higher among our patients than in series published to date. These two types of sign involving the tongue, and which can occur in all forms of psoriasis, appear to be particularly strongly associated with pustular psoriasis. Patients are generally unaware of this sign and rarely complain of it.

Cutaneous granular bacteriosis, a rarely diagnosed infection of the head and the neck.

A young man presented with a large multilobulated and mutilating tumour of the scalp, which had been relapsing for years. Histological examination of a biopsy from the lesion revealed chronic inflammation with granulation tissue and presence of granules with eosinophilic periphery, which was positive for Gram, Grocott and periodic-acid-Schiff stains. A large excision was performed. Cultures grew Staphylococcus aureus. The patient was treated with penicillin G, but 4 weeks after the start of treatment, new small nodules appeared over the same area. All these new nodules disappeared within 2 weeks the addition of clindamycin and cotrimoxazole. This triple antibiotic treatment was carried on for 18 months, and the patient remained disease-free after a follow-up of 4 years. Although the lesions were clinically and histologically suggestive of actinomycosis, culture revealed that they were caused by a completely different organism. We suggest grouping such lesions under a single term "granular bacteriosis" and combining surgery with broad-spectrum antibiotics covering Actinomyces species and botryomycosis-causing organisms (mainly Staphylococcus).

[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.

[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.

[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.

[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.

[Cutaneous nodular lesions and antiphospholipid syndrome]. / Nodules cutanés douloureux et syndrome des antiphospholipides.

BACKGROUND: Antiphospholipid antibodies are a group of autoimmune antibodies directed against a broad panel of phospholipids and their cofactors. Antiphospholipid antibody syndrome is defined by the presence of a hypercoagulable clinical state associated with antiphospholipid antibodies. We report a case with very unusual cutaneous nodular lesions. PATIENTS AND METHODS: A 37-year-old man presented with painful recurrent cutaneous lesions of the nape of the neck. The lesions appeared four years earlier and regressed with oral prednisone. The previous year, cerebral ischaemia revealed antiphospholipid syndrome and anticoagulant therapy was initiated. The patient's cerebral ischaemia was successfully treated but the skin lesions recurred in spite of good anticoagulation therapy. The nodules were strictly confined to the back of the neck. Histopathological examination of a nodular lesion from this patient revealed dilated arterioles and venules with luminal blockage due to fibrinoid thrombosis consistent with the thrombotic microangiopathy seen in antiphospholipid syndrome. DISCUSSION: We describe a case of primary antiphospholipid syndrome with cutaneous lesions as the initial sign of the disease. These "Sweet-like" lesions have been described only once in the literature. Therapeutic strategy in the event of failure of anticoagulants is not well codified.

[Plaque-type scleroderma associated with linear and oesophageal features and facial and extra-facial hemiatrophy]. / Sclérodermie en plaques et linéaire avec hémiatrophie faciale et extra-faciale et atteinte oesophagienne.

BACKGROUND: Association of scleroderma with hemiatrophy is rare. The case we describe is unusual because of the combination in the same patient of several sub-types of scleroderma with oesophageal involvement and facial and extra-facial hemiatrophy. CASE REPORT: A 38-year-old women suffering from plaque-type morphea presented oesophageal dysfunction during the course of her disease with positive anti-Scl70-antibodies and progressive right-sided hemiatrophy of the face, sternocleidomastoid, thumb and thenar eminence. Linear hyperpigmentation of the right arm and a "coup de sabre" appearance on the face were also noted. DISCUSSION: Facial and extra-facial hemiatrophies are usually associated with or originate secondary to linear scleroderma. Only two cases with systemic involvement have been reported but hemiatrophy was localised to the face. The present case is unusual because of its onset as morphea in plaque form, because of the oesophageal involvement and the additional association of morphea in a linear form and facial and extra facial hemiatrophy. The relationship between sclerodermic facial hemiatrophy and Romberg facial hemiatrophy is also discussed. CONCLUSION: The combination of several sub-types of scleroderma and facial and extra-facial hemiatrophy in the same patient may indicate that these entities actually represent different spectra of the same disease.

[Junctional epidermolysis bullosa. Identification of a new mutation in two Lebanese families]. / Epidermolyse bulleuse jonctionnelle: identification d'une nouvelle mutation dans 2 familles libanaises.

BACKGROUND: Junctional epidermolysis bullosa (JEB) represents a genetically heterozygous group of bullous disorders characterized by dermo-epidermal separation resulting from mutations affecting the main dermo-epidermal adhesion factor, laminin-5, its cellular receptor, integrin alpha6B4, or collagen XVII. We report the identification of a new mutation of LAMA3, encoding laminin-5 alpha3 subunit in two unrelated Lebanese families. PATIENTS AND METHODS: Two female newborn, descending from 1st degree consanguineous marriages, presented a lethal form of EBJ-Herlitz. Histologic, ultrastructural and immunofluorescence studies were performed in order to ascertain the diagnosis and to direct genetic analysis. Mutation search was conducted through direct DNA sequencing of patients and ascendants. RESULTS: Immunohistology of frozen skin samples revealed an extremely reduced immunoreactivity for the alpha3 laminin-5 subunit. The two patients were homozygous carriers (parents heterozygous) of a new missense mutation of LAMA3 gene (exon 32: 4300 insA) encoding the alpha3 subunit of laminin-5. Resulting messenger RNA, rapidly degraded, induced an extremely reduced synthesis of alpha3-polypeptide, truncated in its Cterminal domain. DISCUSSION: LAMB3 gene recurrent mutations R636X and R42X account for about 50p. 100 of EBJ cases affecting Caucasians while mutation Q1083X, affecting the same gene, is recurrent in Arab populations. The newly identified mutation results in extremely reduced synthesis of alpha3 chain and truncation of its C-terminal domain, which is crucial for the intermolecular interactions of laminin-5. Our data are in accordance with recent reports suggesting geographical specificity of EBJ mutations linked to founder effects which are amplified by consanguineous marriages in genetically isolated populations. Otherwise, the observation of other unexplored cases of bullous dermatoses with early demise originating from the same region of the two families herein reported highlights the need for the implementation of a prenatal and postnatal diagnostic strategy regarding these genodermatoses. These studies should target LAMA3 and other genes involved in JEB too.

Allergic contact dermatitis from safety clothes and individual protective devices.

Safety clothing and individual protective devices may induce contact dermatitis. Typical clinical patterns cannot always be found. Patch testing must be performed with articles actually used and with specific allergens. In some cases, it is necessary to analyze chemically the article itself.