Maurice J. Dahdah

Videodermoscopy of the hyponychium in nail bed psoriasis

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Atrophoderma of Pasini and Pierini: a clinical and histopathological study

Background:  Idiopathic atrophoderma of Pasini and Pierini (IAPP) usually manifests as one or multiple depressed and hyperpigmented patches, with a predilection to the trunk. No diagnostic changes are usually seen on histology. Elastic stains often reveal no abnormalities.

Retronychia: Report of two cases

Retronychia is an incomplete form of nail shedding that leads to embedding of the nail into the proximal nail fold and subsequent inflammation. Patients present with persistent paronychia in the setting of disrupted nail growth. Other nail changes may be present. Simple avulsion is curative, and unlike other forms of ingrown nails, it does not tend to recur. We report 2 cases of retronychia that were associated with distal onycholysis and subungual hematoma, respectively. Both were successfully treated with nail avulsion.

Primary localized cutaneous amyloidosis: a sign of immune dysregulation?

The manifestations of primary localized cutaneous amyloidosis (PLCA) are usually limited to the skin. The exact etiopathogenesis of PLCA has not been clearly elucidated yet. An increasing number of reports in the literature that associate PLCA with various autoimmune/immune disorders suggest that underlying immune‐mediated factors may be implicated.

GJB6, of which mutations underlie Clouston syndrome, is a potential direct target gene of p63

BACKGROUND Clouston syndrome is a rare autosomal dominant condition characterized by hypotrichosis, nail dystrophy, and occasionally palmoplantar keratoderma. The disease is caused by mutations in GJB6 gene, which encodes a gap junction protein connexin 30 (Cx30). OBJECTIVE To disclose the molecular basis of Clouston syndrome in a Lebanese-German family, and also to determine precise expression of Cx30 in normal skin of humans and mice, as well as transcriptional regulation for the GJB6 expression. METHODS We searched for mutations in the GJB6 gene using DNA of the family members with Clouston syndrome. We performed immunostaining to localize the Cx30 expression in normal human skin and mouse embryos. In addition, we did a series of in vitro studies to investigate if the GJB6 could be a direct transcriptional target gene of p63. RESULTS We identified a recurrent heterozygous mutation c.31G>C (p.Gly11Arg) in the GJB6 gene in the Lebanese-German family with Clouston syndrome. Immunostaining in normal human skin sections demonstrated predominant expression of Cx30 in hair follicles, nails, and palmoplantar epidermis, which partially overlapped with p63 expression. We also showed co-expression of Cx30 and p63 in developing mouse hair follicles and nail units. In cultured cells, the GJB6 expression was significantly upregulated by ΔNp63α isoform. Further in vitro analyses suggested that ΔNp63α was potentially involved in the GJB6 expression via binding to the sequences in intron 1 of the GJB6 gene. CONCLUSION Our data further underscore the crucial roles of Cx30 in morphogenesis and development of skin and its appendages.

Treatment of pachydermoperiostosis pachydermia with botulinum toxin type A.

BACKGROUND Pachydermoperiostosis (PDP) is a rare hereditary disorder characterized by digital clubbing, periostosis, and pachydermia. Pachydermia results in leonine facies, a major cause of cosmetic and functional morbidity in these patients. Its treatment is usually surgical. So far, no medical treatment has been suggested to alleviate this morbidity. OBJECTIVE We sought to assess the role of botulinum toxin type A (BTX-A) in improving the cosmetic appearance of pachydermia in patients with PDP. METHODS Three patients with PDP were treated with BTX-A for their leonine facies. A total of 70 to 80 U were used to treat the upper third of the face. Photographs were taken at baseline and at 2 and 6 weeks after the injections. The patients were followed up periodically for at least 6 months. Wrinkle severity was assessed at relaxation using the 4-point facial wrinkle scale at baseline, week 6, and month 6. In addition, a subjective assessment of the improvement of the extent and depth of the facial rhytides/furrows over the upper third of the face was performed by the same investigator at week 6 and month 6. RESULTS Using the subjective assessment of the improvement of wrinkles, all 3 patients exhibited a fair to excellent response at week 6 that started manifesting 1 week after the BTX-A treatment. All patients demonstrated a residual effect 6 months after the treatment. One patient exhibited a mild exacerbation of his ptosis. LIMITATIONS Major limitations were the small number of patients and the administration of BTX-A injections and assessment of their response by a single unblinded physician. CONCLUSION BTX-A is a simple procedure that may be of value in temporarily improving the cosmetic appearance of pachydermia in patients with PDP.

The Role of Hair Restoration Surgery in Primary Cicatricial Alopecia

Primary scarring alopecias are a group of inflammatory conditions in which the main target of the inflammation is the hair follicle. They represent up to 7.3% of the hair loss cases presenting to specialty hair clinics. They are poorly understood, their pathogeneses are mostly unknown and their management is guided at best by the results of small case series and expert opinions. These alopecias are frequently referred to as a trichologic emergency as their progression can be rapid and the hair loss they induce is permanent. In view of the suboptimal solutions offered by the pharmacological treatments available and the significant emotional distress caused by these conditions, it is only normal for patients with scarring alopecias and their treating physicians alike to explore the prospects of surgical correction. This review attempts to summarize what is known about hair restoration surgery in scarring alopecia.

Mutilating Lupoid Leishmaniasis: Twelve Years to Make the Diagnosis!

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Pachyonychia congenita tarda : very late onset

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