Samer Ghosn

Successful treatment of refractory skin manifestations of systemic lupus erythematosus with rituximab: report of a case.

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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.

Acrodermatitis Continua of Hallopeau Is a Clinical Phenotype of DITRA: Evidence that It Is a Variant of Pustular Psoriasis

Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, sterile, pustular eruption that predominantly affects the fingertips with nail involvement. While some consider ACH a distinct entity, many believe it to be a variant of pustular psoriasis, especially as cases of ACH progressing to generalized pustular psoriasis (GPP) have been reported. Recently, recessively inherited mutations in the IL36RN gene, which encodes interleukin-36 receptor antagonist (IL-36Ra), have been demonstrated to be the cause of familial GPP, a condition termed DITRA (deficiency of IL-36Ra). Here, we identified a homozygous missense mutation c.338C>T (p.Ser113Leu) in the IL36RN gene in a male patient with ACH, as well as in his sister who had a history of GPP.

Takayasu disease presenting as malignant pyoderma gangrenosum in a child with relapsing polychondritis

A 7-year-old Lebanese girl with recently diagnosed relapsing polychondritis had a 1-month history of several painful ulcerations involving her entire body. Skin biopsy was consistent with the diagnosis of pyoderma gangrenosum. She was hospitalized and started on intravenous steroids with partial improvement. During her hospital stay, she developed right wrist drop. Radiologic studies revealed a large aortic aneurysm and an axillary aneurysm compressing the right brachial nerve plexus. Pathology of the resected aortic anyeurism confirmed the diagnosis of Takayasus arteritis. The patient was started on infliximab therapy with complete resolution of her skin lesions and improvement in hand function.

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.

Cutaneous manifestations of liver diseases

The cutaneous manifestations of liver disorders are protean. In addition to the well-recognized stigmata of liver cirrhosis, various infectious, metabolic, autoimmune, hereditary, developmental, and neoplastic liver disorders can result in various cutaneous and mucosal changes as well as in pruritus and abnormal hair and nail growth. Although many of these changes are nonspecific, some are associated with distinct liver diseases and correlate with the severity of hepatic pathology. Familiarization with the spectrum of these manifestations is important for early detection and treatment of hepatic diseases. We review the medical literature and discuss the spectrum of dermatologic manifestations of liver cirrhosis and of important liver diseases, their etiopathogenesis, significance, and treatment.

Bilateral aberrant axillary breast tissue mimicking lipomas: report of a case and review of the literature

Abstract:  A 31 year old Indian woman presented with bilateral axillary masses that became noticeable with the onset of puberty. The masses exhibited similar consistency to the adjacent normal breast tissue but lacked an associated nipple complex. The clinical impression was lipoma; however, mammography, ultrasonography and skin biopsy revealed ectopic breast tissue. These findings were consistent with the diagnosis of aberrant breast tissue. A subset of ectopic mammary tissue, aberrant breast tissue may constitute a diagnostic challenge and is often misdiagnosed as lipoma, hidradenitis, follicular cyst, or lymphadenopathy. In addition, some studies have suggested that aberrant breast tissue may be at higher risk of malignant degeneration. Therefore, it’s important that physicians be familiar with this condition as this may contribute to the early detection of ectopic breast cancer.

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.

Late Evolution of Giant Verruciform Xanthoma in the Setting of CHILD Syndrome

Abstract:  Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) syndrome is a rare X‐linked dominant disorder that is characterized by peculiar cutaneous features commonly associated with skeletal and internal organ involvement. Cutaneous verruciform xanthoma is an uncommon cutaneous manifestation of congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome. We present a case presenting with a late onset of a large verruciform xanthoma of the right foot, in addition to the more typical cutaneous features of congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome.

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.