Nelly Rubeiz

Cutaneous leishmaniasis: clinical features and diagnosis.

Leishmaniasis is a protozoan disease of multiple clinical manifestations, present in the Old and New Worlds. The disease is caused by more than 20 Leishmania species. The protozoa are transmitted to humans by the bite of the infected female sandflies belonging to genus Phlebotomus in the Old World and Lutzomyia in the New World. Around 30 species of sandflies are recognized as vectors. The reservoir hosts are usually certain domestic and wild animals. The relative tropism (visceral vs. dermal) of Leishmania organisms is used in the classic classification of the disease into cutaneous (the subject of this review), mucocutaneous, and visceral leishmaniasis. Although each of the Leishmania species may have its peculiar manifestations and areas of endemicity, yet none of the clinical manifestations is unique to a particular species because of considerable clinical diversity and overlap. This is because the clinical picture is dependent on determinants related to the infecting species of Leishmania and the host. These include infectivity, virulence of the parasite, extent of lymphatic and hematogenous spread in addition to immune response, and genetic susceptibility of the host.

A review of Parry-Romberg syndrome

Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, muscles, and sometimes extending to the osteocartilaginous structures. It has been associated with various systemic manifestations, particularly neurologic, ophthalmologic and maxillofacial. In this article, we review Parry-Romberg syndrome with its associated findings (neurologic, ophthalmologic, cardiac, rheumatologic, endocrinologic, infectious, orthodontic and maxillofacial, and autoimmune), underlying cause, differential diagnoses (en coup de sabre, scleroderma, and Rasmussen encephalitis), and therapeutic options.

Pseudoxanthoma elasticum-like papillary dermal elastolysis: a report of two cases.

We report 2 women, aged 83 and 63 years, who presented with multiple asymptomatic, slowly progressive, coalescing, skin‐colored papules affecting the sides of the neck and lower abdomen. Incisional biopsies obtained from both patients revealed elastolysis in the papillary dermis. These 2 cases represent pseudoxanthoma elasticum‐like papillary dermal elastolysis, a recently described entity, the etiology of which remains unclear.

Ashy dermatoses – a critical review of the literature and a proposed simplified clinical classification

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Sweet’s syndrome: retrospective study of clinical and histologic features of 44 cases from a tertiary care center

Background  Sweet’s syndrome (SS) is an uncommon disorder characterized by the abrupt onset of erythematous papules and plaques that histologically exhibit diffuse dermal neutrophilic infiltrate and edema. There are usually associated constitutional symptoms such as fever, neutrophilia, elevated serum inflammatory markers, and associated disorders. The aim of this study was to assess the clinical and histologic features of all patients diagnosed with SS at our institution between 1971 and 2008 and to compare their findings with those published in the literature.

Streptococcus sp. and Staphylococcus aureus Isolates from Patients with Psoriasis Possess Genes That Code for Toxins (Superantigens): Clinical and Therapeutic Implications

Superantigens are powerful T lymphocyte–stimulating agents that are believed to contribute to the pathogenesis of certain diseases such as psoriasis. Toxins produced by Streptococcus pyogenes and Staphylococcus aureus are superantigens. The aim of this study was to detect genes that code for superantigens in Streptococcus and Staphylococcus aureus isolates from psoriatic patients. Primers to amplify streptococcal pyrogenic exotoxin A, B, and C and streptolysin O genes and staphylococcal enterotoxin A, B, C, and D genes were used. Streptococcal exotoxin B was detected in five streptococcal isolates. Staphyloccocus aureus enterotoxin A and/or C genes were detected in nine S. aureus isolates. Isolates from 13 of 22 patients possesed gene(s) that code for toxin(s) (superantigens). These results might support the role of superantigens in the exacerbation of psoriasis.

Vascular anomalies: portwine stains and hemangiomas

In 1995, Dr Martin Mihm, already a renowned dermatopathologist in melanoma, met a desperate mother whose daughter was diagnosed with a hemangioma, the most common benign vascular tumor of infancy. At that time, the literature on the subject was sparse, only a handful of treatment centers existed, and virtually no pathologists took a focused interest in the subject. Dr Mihm’s interest in the area grew not only from meeting the mother but also from his personal experiences with the condition. Dr Mihm himself had been diagnosed with hemangioma as an infant, and his godson also suffered from a recurrent problematic vascular malformation. This was the beginning of a whole new world of tumor research for the brilliant dermatologist and pathologist. At the time, research in hemangiomas was limited: Many physicians were confused about the biological difference between hemangiomas and malformations, and as a result, many affected individuals were inaccurately diagnosed and improperly treated. Although improvements in the treatment of vascular tumors had begun 30 years before, the histopathology remained unchanged. Dr Mihm pioneered the histopathology of vascular anomalies, particularly the understanding of the pathology of these tumors. His self-funded research laid the groundwork for developing an accurate, foolproof diagnosis for hemangiomas. Dr Mihm and his collaborators established that hemangiomas stain positive for glucose transporter isoform 1 (GLUT1).1 Physicians are now able to differentiate hemangiomas accurately from other vascular tumors and thus provide the appropriate treatment. Dr Mihm’s mission focuses on making treatment equally available for all people. He cofounded over a half dozen vascular birthmarks and malformations treatment centers in Albany, Boston, Greece, Italy, Spain and recently one in Vietnam. His personal mission is to bring treatment and the understanding of these tumors to people all over the world. His future plans include multidisciplinary treatment centers in Asia, Canada and Puerto Rico. Classification Vascular anomalies can be divided into two groups: vascular tumors and vascular malformations.2 Vascular tumors usually appear after birth; vascular malformations are usually present at birth. Vascular tumors include: (a) hemangiomas, infantile type and congenital type (RICH: rapidly involuting congenital hemangioma; NICH, noninvoluting congenital hemangioma), (b) kaposiform hemangioendothelioma, (c) tufted angioma, (d) pyogenic granuloma and (e) spindle cell hemangioendothelioma. Vascular malformations include: (a) capillary malformations: portwine stain (PWS) and telangiectasias, (b) lymphatic malformations, (c) venous malformations, (d) arterial malformations and (e) combined malformations. We discuss the most common types, which are infantile hemangiomas (IHs) and PWSs.

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.

Management of ichthyosis in infants and children

Ichthyoses are a group of genetic disorders of keratinization characterized by conspicuous, extensive scaling. Ichthyosis can lead to social discrimination and psychological problems, particularly in childhood, and efficient therapies are necessary that are safe and well tolerated. No cure exists, however, and the management is still unsatisfactory. Treatment is aimed at decreasing the signs and symptoms of these diseases by hydration, lubrication, and keratolysis. Although hyperkeratotic, the ichthyotic skin has a decreased barrier function and increased transepidermal water loss; the pliability of the stratum corneum is also reduced. Thus, the main approach to treatment includes hydration of the skin followed immediately by the application of lubricants to retain the hydration and softening and to prevent evaporation. The stratum corneum can absorb 6 times its weight in water, and oils, ointments, or petrolatum must be applied while the skin is still wet. Hydration also facilitates desquamation by promoting the susceptibility to mechanical forces and by increasing hydrolytic enzyme activity. Mechanical exfoliation may be achieved with a loofah or other mild abrasives while bathing. Increasing the environmental humidity at home is beneficial for all types of ichthyoses. Treatment regimens are variable and are in no way restrictive. They may include topical agents, oral medications, and/or a combination thereof. Old and new remedies exist and are listed below without order or preference.

Vulvar melanosis and lentiginosis: a case report.

Vulvar melanosis and/or lentiginosis (VM/L) is a cause of concern for patients and clinicians, t3 These lesions may be single or multiple and are characterized by large size, irregular borders, and variegated pigmentary patterns: Despite their clinical appearance, the histologic features are benign. t, 3-8 Little is known about the biologic behavior of VM/L. However, during the past few years 17 cases have been reported; malignant melanoma has not occurred in any of these. We report the case of a woman with multiple pigmented macules on her genitalia.