Cylindroma: an update

This paper reviews the recent literature on cylindroma, a rare tumor originating from the cutaneous adnexal structures of the scalp and face. Benign cylindroma is more frequent in women, and there are solitary and multiple forms, which are autosomal. Malignant cylindroma can occur in the Brooke–Spiegler syndrome, and malignant transformation is more frequent in multiple variants than in solitary tumors. Its histopathological basis is controversial, and its origin is unknown, although it is known that there is a genetic basis for onset of these tumors in Brooke–Spiegler syndrome.     

Update on the current state of melanoma incidence

The incidence of melanoma is rising worldwide, and in the United States has increased by approximately 2.8% annually since 1981. Melanoma is more common in whites, and is generally more prevalent in men. However, there is a 6.1% annual increase in US incidence of melanomas in white women younger than age 44, with growing concern that increases in skin cancer in younger women may reflect recent trends in indoor tanning. Melanoma incidence is also greater in higher economic groups. Globally, melanoma incidence is highest in Australia, followed by the United States and parts of Europe.     

Training for integration

Training is often suggested as the solution to the inadequacies of the health care system, and there is little doubt that without it, service quality would suffer and new techniques and technologies would be difficult to introduce; clearly it is an important component in any drive to achieve quality of care. However, in this era of cost-effectiveness and cost cutting, which is part of the reason for integration, it is surprising that training is often not well planned and is rarely evaluated in a rational manner. This paper relies on recent discussions within ILEP about training and the use of training materials for leprosy in the present environment--one in which most programmes are being integrated into the general health services. The development of a National Training Plan for Leprosy is proposed, with clear objectives, in order to best utilize the resources available.     

Cause and management of lipedema‐associated pain

Lipedema is a painful, underdiagnosed adipose tissue disorder, characterized by symmetrical swelling of the extremities due to subcutaneous fat deposition in the buttocks, thighs, legs, and arms, sparing the most distal part of the extremities. Although etiology and pathogenesis of lipedema is unclear, possible role of hormonal and genetic factors have been proposed previously. Patients with lipedema suffer from pain, easy bruising, tenderness, and disfigurement. Pain is the leading symptom in lipedema. Since the pain is associated with depression and impaired quality of life, reduction of pain is the major therapeutic approach. Pain in lipedema is attributed to allodynia, exaggerated sympathetic signaling, and estrogens. Although the mechanism of pain in lipedema is uncertain, effective treatment of lipedema should provide a satisfactory pain reduction. Efficacy of the conservative treatment is a matter of debate. Microcannular tumescent liposuction is the most effective therapeutic option for lipedema. There is a large body of evidence that this procedure significantly reduces pain in patients with lipedema.     

Imported Tropical Infectious Ulcers in Travelers

Skin ulcers are a commonly encountered problem at departments of tropical dermatology in the Western world. Furthermore, the general dermatologist is likely to be consulted more often for imported chronic skin ulcers because of the ever-increasing travel to and from tropical countries. The most common cause of chronic ulceration throughout the world is probably pyoderma. However, in some parts of the world, cutaneous leishmaniasis is one of the most prevalent causes. Mycobacterium ulcerans is an important cause of chronic ulcers in West Africa. Bacterial infections include pyoderma, mycobacterial infections, diphtheria, and anthrax. Pyoderma is caused by Staphylococcus aureus and/or beta-hemolytic streptococci group A. This condition is a common cause of ulcerative skin lesions in tropical countries and is often encountered as a secondary infection in travelers. The diagnosis is often made on clinical grounds. Antibacterial treatment for pyoderma should preferably be based on culture outcome. Floxacillin is generally active against S. aureus and beta-hemolytic streptococci. Infection with Mycobacterium ulcerans, M. marinum, and M. tuberculosis may cause ulcers. Buruli ulcers, which are caused by M. ulcerans, are endemic in foci in West Africa and have been reported as an imported disease in the Western world. Treatment is generally surgical, although a combination of rifampin (rifampicin) and streptomycin may be effective in the early stage. M. marinum causes occasional ulcerating lesions in humans. Treatment regimens consist of combinations containing clarithromycin, rifampin, or ethambutol. Cutaneous tuberculosis is rare in travelers but may be encountered in immigrants from developing countries. Treatment is with multiple drug regimens consisting of isoniazid, ethambutol, pyrazinamide, and rifampin. Cutaneous diphtheria is still endemic in many tropical countries. Cutaneous diphtheria ulcers are nonspecific and erythromycin and penicillin are both effective antibacterials. Antitoxin should be administered intramuscularly in suspected cases. Anthrax is caused by spore-forming Bacillus anthracis. This infection is still endemic in many tropical countries. Eschar formation, which sloughs and leaves behind a shallow ulcer at the site of inoculation, characterizes cutaneous anthrax. Penicillin and doxycycline are effective antibacterials. Cutaneous leishmaniasis is caused by different species belonging to the genus Leishmania. The disorder is one of the ten most frequent causes of skin diseases in travelers returning from (sub)tropical countries. The clinical picture is diverse, ranging from a painless papule or nodule to an ulcer with or without a scab. Treatment depends on the clinical manifestations and the species involved.Sporotrichosis, chromo(blasto)mycosis, and mycetoma are the most common mycoses that may be accompanied by ulceration. Infections are restricted to certain regions and often result from direct penetration of the fungus into the skin. Anti-mycotic treatment depends on the microorganism involved. The most common causes of infectious skin ulceration encountered in patients from tropical countries who present at a department of tropical dermatology are reviewed in this article.     

Soft tissue myoepithelioma of the scalp in a 11-year-old girl: a challenging diagnosis

Myoepithelioma is a well-known tumor in the salivary glands and breasts in adults. It is exceptionally rare in soft tissue and in children. We present a case of subcutaneous scalp myoepithelioma in an 11-year-old girl. On clinical examination, it appeared as a dermoid cyst. Myoepithelioma is uncommon in the subcutaneous tissue. Clinically, the neoplasm is nonspecific. Because of the variable appearance of myoepithelial cells and their phenotype, the pathological diagnosis is challenging. We report a case of subcutaneous myoepithelioma in a child and discuss the literature.     

Griseofulvin

Griseofulvin is a metabolic product of Penicillium spp. It was the first available oral agent for the treatment of dermatophytoses and has now been used for more than forty years. Griseofulvin is fongistatic, the exact mechanism in witch it inhibits the growth of dermatophytes is doubtful. Several ways are invoked: inhibition of fungal cell mitosis and nuclear acid synthesis, probable interference with the function of microtubules. Griseofulvin is poorly absorbed from the gastrointestinal tract. Absorption is enhanced by administration with fatty meal. Peak plasma occurs four hours after oral administration. Griseofulvin is detected in the outer layer of the stratum corneum soon after it is ingested, it is diffused from the extracellular fluid and sweat. There is no information regarding the mechanism by witch the drug is delivered to nails and hair. Deposition in the newly formed cells could be the major factor. Griseofulvin has also anti-inflammatory properties and some direct vasodilatory effects when it is used in high doses. It is metabolised by the liver microsomial enzyme system and excreted in the urine. The half-life is 9 to 21 hours. Griseofulvine has been used in the therapy of dermatophyte onychomycosis, treatment periods from 6 to 18 months were necessary with disappointing results and numerous relapses. Newer oral antifungal agents are now preferred especially in toenail infections. For many authors griseofulvin is still the treatment of choice of tinea capitis. Doses are 15-20 mg/kg/d for 6 to 8 weeks in children with the microsized form. Clinical response rates have been reported between 80 and 90 p. 100 in controlled studies. Griseofulvin is well-tolerated particulary in children. More frequent side effects are minor: headaches, gastrointestinal reactions and cutaneous eruptions. The major drug interactions has been noted with phenobarbital, anticoagulants and oral contraceptives.     

雌激素β受体在恶性黑素瘤中的研究进展

越来越多的研究提示,恶性黑素瘤是一种激素依赖性肿瘤,其发病率男性高于女性,女性患者预后较好.β受体是黑素瘤中主要的雌激素受体,可以抑制细胞增殖,具有抑癌作用.随着肿瘤的进展,其表达量逐渐下降,与恶性黑素瘤的发生发展密切相关.女性雌激素β受体水平明显高于男性,可能与恶性黑素瘤女性患者预后更好相一致.未来雌激素受体β及其激动剂可作为评价预后的重要标志及新的治疗策略.     

Retrospective analysis of the treatment of psoriasis of the palms and soles

In this retrospective analysis, the effect of currently used treatments in 26 patients with psoriasis of the palms and soles were analyzed. In general, patients are treated initially with topical medications including superpotent topical corticosteroids in combination with calcipotriene ointment or tazarotene gel or both. If satisfactory improvement is not achieved in 4-8 weeks, systemic retinoids are added, formerly etretinate and currently acitretin, except in women of childbearing potential. If the latter regimen is not effective within two months, soak PUVA is added to the regimen of oral retinoids and topical medications. If improvement is inadequate, or if the treatment regimen is not tolerated, methotrexate or cyclosporine have been added in the past. The availability of the excimer laser has recently modified our approach so that this therapy is used in combination with acitretin before soak PUVA. With the availability of biologic agents, methotrexate is avoided because of its hepatotoxicity and bone marrow toxicity and cyclosporine is avoided because of its nephrotoxicity. If oral acitretin plus topical therapy is not adequate to control the disease and the excimer laser is not an option because of its limited availability, alefacept, etanercept and infliximab are added when possible. Other biologic agents are likely to be added to this list in the future.     

Erysipelas: recognition and management

Erysipelas is an acute bacterial infection of the dermis and hypodermis that is associated with clinical inflammation. It is a specific clinical type of cellulitis and, as such, it should be studied as a specific entity. Erysipelas is generally caused by group A streptococci; it is highly probable that streptococcal toxins also play a role, which could, in part, help explain the clinical inflammation. Erysipelas of the leg is the main clinical type encountered. The face, arm, and upper thigh are the other most common sites for the occurrence of erysipelas. After a sudden onset, areas of erythema and edema characteristically enlarge with well-defined margins. Athlete's foot is the most common portal of entry for the disease. Erysipelas is generally associated with high fever, and adenopathy and lymphangitis are sometimes present. At the time of diagnosis, it is important to look for clinical markers of severity (local signs and symptoms, general signs and symptoms, co-morbidity, social context) which would necessitate hospitalization. There are many differential diagnoses, particularly in the case of atypical dermo-hypodermitis. Some bacterial infections may have specific clinical aspects or may lead to a diagnosis of cellulitis. Necrotizing cellulitis or fasciitis are life-threatening diseases and a rapid diagnosis is important. Other noninfectious types of cellulitis have been reported in many diseases, both localized or generalized. The biology of typical erysipelas is of little value in diagnosis and a laboratory workup is usually not required. There are few local complications associated with erysipelas; abscess can occur in some patients and septicemia is rare. Recurrence is the more distressing complication. Treatment of patients with erysipelas has been evaluated in a small number of studies. In most of them, erysipelas has been included in therapeutic studies of 'severe cutaneous infections'. This is not justified as in fact erysipelas is usually sensitive to penicillin G. Amoxicillin and macrolides are also effective. However, comparative, cost-analysis studies need to be performed to determine the best therapeutic option. Bed rest with the leg elevated is also important. Anticoagulants are indicated in patients at risk of venous thromboembolism. The portal of entry will also require treatment. Long-term antibacterial therapy is required for patients with recurrence.     

The multifunctional role of leukaemia inhibitory factor in cutaneous biology

Leukaemia Inhibitory Factor (LIF) is a polyfunctional cytokine, that belongs to the family of haemopoietic growth factors. LIF plays a role in growth-promotion and differentiation, regulates calcium and bone metabolism, induces acute phase proteins and causes cachexia in organisms with neoplastic disorders. LIF is also to be found in normal skin, where it may be involved in the differentiation process of keratinocytes. In addition, recent data in medical literature indicates that LIF is engaged in the pathogenesis of some skin disorders as well. It has been clearly demonstrated that LIF may act as a proinflammatory cytokine. In allergic contact dermatitis, the expression of LIF mRNA is augmented to a significant degree, indicating that LIF may play a role in the early phase of allergic contact dermatitis. LIF also plays an important role in psoriatic lesions. As the mechanism is not yet fully understood, however, it is hypothesized that the LIF function in psoriatic processes is solely connected with IL-8, as it is known that LIF is able to induce the release of IL-8. Also, some reports have suggested that LIF may also play a role in the carcinogenesis of the skin.     

Tyrosinemia II: Lessons in Molecular Pathophysiology

Tyrosinemia II is caused by a deficiency of hepatic tyrosine amino-transferase. With the deficiency of this key enzyme of tyrosinc catabolism there is an increase in plasma tyrosine and then an increase in tyrosinc metabolites in the urine. The increased plasma tyrosinc causes tyrosine to crystallize in the cornea, producing corneal ulcerations and sometimes proliferation of corneal epithelium. In the epiderm is of the palms and soles, tyrosine leads to erosions, crusting, and then hyperkeratosis. The human disease is due to an autosomal recessive gene, and similar genetic diseases have been found in mink and in dogs. A nutritional model for the disease, in which a high-tyrosine low-protein diet is ted to rats, produces almost identical features. The features of this disorder and some of the implications of this disease for the study of other genetic diseases is discussed in this review.     

Two mouse mutations mapped to chromosome 11 with differing morphologies but similar progressive inflammatory alopecia

Alopecia is a common dermatological condition in humans and other mammals. Here, we present two similar but histologically distinct mouse models of scarring alopecia. Both mutant lines were generated using random genome-wide N-ethyl-N-nitrosourea mutagenesis, and both harbor dominant mutations on chromosome 11. In both mutants, there is an early onset of alopecia that progresses to nearly complete pelage hair loss in both males and females by 20 weeks of age. Histologically, there is an increased dermal cellularity due to inflammatory cell infiltration at 7-10 days of age. By 3 weeks of age, the epidermis is acanthotic and the dermis is approximately twice as thick as in control mice due to a substantial, mostly mononuclear, inflammatory cell infiltrate. This infiltrate becomes more perifollicular by 4-5 weeks of age but is localized differently in the two mutants. In alopecia 1 (Alo-1), the perifollicular infiltrate is confined to the portion of the follicle within the dermis, whereas in Alo-2, the infiltrate extends the full length of the follicle. Expression of major histocompatibility complex (MHC) class I on the follicular epithelium in the two mutants is much greater than that in non-mutants. Furthermore, MHC class I expression is localized differently in the two mutant lines and mirrors the pattern of the inflammatory infiltrate. Despite these differences, the clinical progression of alopecia is identical in both mutants. The early onset of the disease, predictable progression, and differences in inflammatory cell localization between the two mutants make these mice particularly useful models for inflammatory hair loss and autoimmune diseases in general.     

Cutaneous artifactual disease. A review, amplified by personal experience

Cutaneous artifactual disease is part of the general syndrome of contrived disease. While classical examples are easy to recognize, it can present in unusual ways. Difficulties in recognition include the unusual doctor-patient relationship, the doctor's fear of missing organic disease, and the reality of the disease to the patient's family and the family doctor. The diagnosis depends upon finding lesions whose morphology is consistent and an emotionally immature patient, whose personality shows, or has shown, hysterical and masochistic traits. Lesions "arrive" fully developed. Once there they begin to heal, so that a continuous supply of new ones is necessary if the illness is to continue. High intelligence is compatible with the diagnosis, but a mature personality is not. The differential diagnosis is extensive. Investigations prove negative or equivocal; in the latter case, the investigator is led even further into unfamiliar territory. It is suggested that the essence of management is to keep in contact with the patient.     

Controversies about the decubitus ulcer

Knowledge of the decubitus ulcer has made great progress in recent decades. No longer is the diagnosis of a bedsore, as it was in the iron lung days, almost a death warrant. One cannot cause to heal a patient who is in organ failure, nor can one necessarily prevent ulcers in a new spinal cord-injured patient. Reflecting on the common cold, one realizes that prevention is limited to covering the face when sneezing, and treatment is symptomatic. There is no way of eradicating the virus of the common cold, nor is there an appropriate treatment. The decubitus ulcer should be conceived of in a similar vein, until understanding is more complete and specific treatments are available.     

Tumoral calcinosis

Tumoral calcinosis is a distinct entity, which is rarely seen in Europe and North America but much more common in black Africans. Typical symptoms are calcified nodules, which grow while remaining asymptomatic and are found in the tissues adjacent to the large joints of the body. Histologically there is collagen necrobiosis initially, which results in aggregates of densely calcified material. The aetiology is unknown, but the condition is probably a form of dystrophic calcification caused by mechanical injury. A patient with tumoral calcinosis is presented, and the clinical and histological findings are described.     

A new form of cellular arrangement in guinea-pig ear epidermis

A new type of squame arrangement is described in guinea-pig ear epidermis. It is characterized by overlapping obliquely-angled outer cells and squames, which thus form a roof-tile pattern. This pattern develops in epidermis that is thicker than about 42 μm; in thinner epidermis the outer cells and squames form the usual columnar stacks. It is concluded that the roof-tile pattern, which is usually directed towards the ear edge, is typical of slight to moderate hyperplasia. In extreme hyperplasia, when the strata granulosum and corneum are destroyed, only cellular disorder is seen.     

The role of psychoneuroimmunology in the pathogenesis of psoriasis

Although it is well known that stress can trigger and exacerbate psoriasis, the exact mechanism is unknown. An explanation is presented based on recent findings in psychoneuroimmunology. The number of cutaneous sensory nerves known to release neuropeptides, such as substance P, is increased in patients with psoriasis. Preliminary data indicate altered concentrations in psoriatic lesions of the same neuropeptides known to be altered in the brain during stress. An anatomical pathway is suggested to explain how descending information from the brain could cause release of neuropeptides in the skin, which would then induce psoriasis. Biochemical and clinical evidence is presented to support the relationship between stress and psoriasis.     

Staphylococcal scalded skin syndrome: diagnosis and management

Staphylococcal scalded skin syndrome (SSSS) is a common disorder that is usually seen in infants and children and rarely seen in adults. SSSS usually presents with a prodrome of sore throat or conjunctivitis. Extremely tender flaccid bullae, which are Nikolsky sign-positive, develop within 48 hours and commonly affect the flexures; occasionally, large areas of the skin may be involved. The bullae enlarge and rupture easily to reveal a moist erythematous base, which gives rise to the scalded appearance. SSSS in adults is a rare disorder, though there are now over 50 documented cases. Usually SSSS occurs in predisposed individuals, but not all adults have an underlying illness. Whereas mortality in childhood SSSS is approximately 4%, the mortality rate in adults is reported to be greater than 60%. SSSS is caused by an infection with a particular strain of Staphylococcus aureus, which leads to blistering of the upper layer of the skin, by the release of a circulating exotoxin. It has recently been demonstrated that the exfoliative exotoxin responsible for SSSS leads to the cleavage of desmoglein 1 complex, an important desmosomal protein. The same toxins that are responsible for causing SSSS also cause bullous impetigo. There appears to be a relationship between the disease extent, the amount of toxin produced and whether the toxin is released locally or systemically. As a result there is likely to be a spectrum of disease and there are likely to be a number of milder cases of adult SSSS that go undiagnosed. Social improvements and hygiene have led to a dramatic fall in the number of cases of SSSS. Treatment is usually straightforward, when there is no coexistent morbidity and the presentation is mild, but can be demanding if the patient is particularly ill. SSSS is still associated with mortality, particularly when it occurs in adults.     

Lipoid proteinosis

Lipoid proteinosis is an infrequent disease characterized by the deposition of a PAS-positive diastase-resistant hyaline material in the skin and respiratory tract, although it can also be deposited in internal organs, in a generally asymptomatic manner. The earliest clinical manifestation is hoarseness. Clinical cutaneous manifestations come later, in the form of hyperkeratotic lesions located on the trunk, elbows, axillae, groins, backs of hands, palms and soles. A lesion typical of the disease is moniliform blepharosis, which consists of beaded papules along the eyelid margins. Also characteristic is the presence of comma-shaped intracranial calcifications in the temporal lobes. The course of the disease is progressive, with a normal life expectancy. It affects men and women equally, with worldwide distribution. The diagnosis is based on the clinical symptoms and the histology. At this time, there is no effective treatment for the disease. We present a case of lipoid proteinosis in a 23-year-old woman, with typical clinical and histological characteristics.