Papulonodular mucinosis in a patient with systemic lupus erythematosus and antiphospholipid syndrome.

The skin is one of the target organs most commonly affected in lupus erythematosus (LE) and a wide range of cutaneous changes have been described in LE patients. Papulonodular mucinosis (PNM) in particular is an uncommon cutaneous manifestation of LE. We discuss the case of a 26-year-old Senegalese woman with systemic LE and antiphospholipid syndrome (APS) who presented with pruritic papules on her back and extremities that appeared when she was on vacation in Africa and non-compliant with medications. Histopathologic examination was consistent with PNM. The patient was treated with mycophenolate mofetil and hydroxychloroquine, with subjective relief in pruritis at 6-week follow-up. To our knowledge, this is the first case of PNM presenting in a patient with both SLE and APS. Whether APS contributes to the pathogenesis of PNM is currently unknown.

Is it time to re-evaluate the treatment of pemphigus?

Pemphigus vulgaris, foliaceous, and vegetans are potentially fatal, autoimmune, vesiculobullous mucocutaneous diseases. In order to prevent potentially fatal infection and other complications, most patients with pemphigus require treatment with systemic corticosteroids and immunosuppressive agents, although these medications often cause chronic and serious adverse effects. Many case reports and several trials have documented remissions and clinical improvement in cases of pemphigus recalcitrant to standard therapy, who were treated with either intravenous immunoglobulin (IVIG) or rituximab, alone or in combination with each other. Collectively, the body of evidence from these reports is large enough to spark consideration of these treatments early in the management of pemphigus. Among the potential benefits of a therapeutic strategy that includes these biologic agents are more rapid induction of remission, prevention of corticosteroid-related adverse effects, and decreased cost of therapy. Considering the outcomes from recent trials with these novel therapies, reevaluation of the best-practice treatment of pemphigus seems prudent and timely.

Verrucous lymphangioma circumscriptum.

A 19-year-old woman with a seven-year history of pruritic, grouped, verrucous papules and plaques on the left arm presented for consultation. The lesion slightly flattened in appearance with topical glucocorticoid treatment. The histopathological features were consistent with lymphangioma circumscriptum. This entity is the most common subtype of lymphangioma and presents with grouped, clear vesicles that may appear pink to purple. However, a rare verrucous type can present a diagnostic challenge. Whereas the definitive treatment option is surgical excision, other treatments, which include sclerotherapy, radiotherapy, and laser therapy, have been reported to cause resolution or improvement. In the future, propranolol may hold promise as a medical therapy for lymphangioma.

Is chronic cutaneous discoid lupus protective against severe renal disease in patients with systemic lupus erythematosus?

OBJECTIVE: The aim was to assess the level of systemic involvement and character of renal disease in patients with chronic cutaneous lupus erythematosus of the discoid lupus variety (hereafter referred to as 'discoid lupus') and features of systemic lupus erythematosus (SLE). Clinical confusion with other types of cutaneous lupus erythematosus complicates interpretation of some previously reported studies. METHODS: Over three years, sixteen patients met the diagnostic criteria of discoid lupus, positive anti-nuclear-antibody, and at least one extracutaneous manifestation. RESULTS: Most patients (14/16) were female, between 26 to 66 years old. Arthritis was the most common extracutaneous manifestation followed by Raynaud's phenomenon. The anti-nuclear-antibody was speckled in ten patients with titers ranging from 1:40 to 1:1280 IU/mL. Elevated levels of double-stranded-DNA in low titers were found in four patients, anti-Smith-antibody in four; anti-Sjogren-syndrome-A-antibody in seven, and anti-ribonucleoprotein-antibody in seven. Renal function markers were transiently high in some patients but normalized over time. Hematuria and/or proteinuria were present at some time in seven patients. The highest BUN and creatinine levels were 42 mg/dL and 1.5 mg/dL, respectively. One patient had membranous glomerulonephropathy class 5; however, discoid lupus developed well after the onset of renal disease during a time when renal function had returned to normal. CONCLUSION: Our observational data supports previous reports suggesting that patients with active discoid lupus rarely have progressive renal insufficiency. The mechanism for the development of discoid lupus may involve an immunologic mechanism that differs from that which produces severe organ involvement, especially advanced immune-complex-mediated renal disease. Patients with discoid lupus rarely have sustained high levels of antibodies to double-stranded-DNA. Discoid lupus appears to be a marker for a more benign lupus course. This clinical observation lays the groundwork for a larger prospective, longitudinal cohort study for further validation.

Thalidomide and analogues: potential for immunomodulation of inflammatory and neoplastic dermatologic disorders.

Thalidomide and analogues are a class of immunomodulatory drugs or IMiDS. Thalidomide was initially approved by the U.S. Food and Drug Administation for treatment of erythema nodosum in leprosy and is now approved for multiple myeloma as well. A second generation IMiD, lenalidomide, is also approved for multiple myeloma and refractory myelodysplastic syndrome. Discovery of this class of drugs has been serendipitous and empirical, as the drug targets have been unknown. In this review, the authors integrate recent identification of drug targets of IMiDS, which include the inducible form of nitric oxide synthase (iNOS), Rho GTPase and caspase-1, with the developments in the understanding of the molecular biology of human inflammatory, infectious and neoplastic skin disorders. Because thalidomide reemerged through leprosy, the original disease classified by the T cell, the authors have also emphasized advances in the understanding of T-cell subsets in human skin disorders.

Atypical mycobacteria infection in an immunocompromised patient.

A 61-year-old woman with systemic lupus erythematosus and Sjögren syndrome presented with a two-month history of symptomatic nodules on the buttocks and thighs that progressed to involve the dorsal aspects of the hands. On examination, infiltrative papules, nodules, and plaques were present in these regions. Biopsy specimens demonstrated granulomatous inflammation and acid-fast bacilli with the use of a Fite stain, although a culture and polymerase chain reaction analysis were negative. The patient continues to improve on long-term clarithromycin therapy. Atypical mycobacterial infections are becoming more common, especially in immunocompromised patients. Antimicrobial therapy, either with a single agent or multiple agents, often is prolonged. A high index of suspicion is warranted in immunocompromised patients, which includes those with connective-tissue diseases that are active or that require immunosuppression. In these patients, the differential diagnosis includes infectious as well as inflammatory, reactive, or neoplastic processes.

Cutaneous umbilical endometriosis.

A 35-year-old woman presented with a four-month history of a tender umbilical nodule that bleeds during her menstrual period. Physical examination showed a hyperpigmented umbilical nodule. A biopsy specimen showed fibrotic dermis with increased numbers of blood vessels and scattered glandular structures with areas of hemosiderin deposition consistent with a diagnosis of endometriosis. Cutaneous umbilical endometriosis is rare, with an estimated incidence of 0.5 to 1.0 percent. Although anti-gonadotropin medications, such as danazol, have been used for symptomatic control, but surgical excision is the treatment of choice owing to the possibility of malignant degeneration of cutaneous endometriosis.

Invasive squamous-cell carcinoma and arsenical keratoses.

A 42-year-old man presented with a six-month history of a slowly-enlarging ulcer on his right sole, a 30-year history of altered pigmentation of the trunk and extremities, and hyperkeratotic papules of the palms and soles. Histopathologic examination showed an invasive squamous-cell carcinoma of the right sole and hyperkeratosis with keratinocyte atypia of the left finger and left lateral foot. The clinical and histopathologic findings are consistent with chronic arsenicism, which most commonly occurs in the setting of drinking contaminated water or after occupational exposure. Evaluation should include a physical examination, basic laboratory work-up, and measurement of a 24-hour urine arsenic concentration. Vigilant surveillance for the development of cutaneous malignancies is required. Oral retinoids may be helpful in reducing hyperkeratosis secondary to chronic arsenicism.

Cutaneous diseases in Latinos.

The knowledge of many specific skin diseases that predominantly affect Latinos has been increasing; however, the understanding of common skin disease in Latinos is superficial. There are few prospective studies done in the Latino population and none that differentiate between diverse Latino backgrounds. In view of the expanding Latino population in this country, such research is timely.

New and re-emerging cutaneous infectious diseases in Latin America and other geographic areas.

Due to environmental factors and inadequate public health measures in many developing countries, new tropical infections, as well as infections that were previously isolated to remote locales, are becoming more prevalent in several areas of Latin America. This article discusses some tropical infections and infestations with predominantly cutaneous manifestations. Previously uncommon diseases such as gnathostomiasis, mycobacteria ulcerans infection, paederus dermatitis, Balamuthia mandrillaris infection, and human T-lymphotrophic virus 1 dermatitis are increasingly being reported. Well-known tropical infections such as bartonellosis, leishmaniasis, chromomycosis, larva migrans, and larva currens are also becoming more prevalent. On the other hand, the incidence of Hansen's disease, the quintessential tropical infection, is dwindling all over the globe thanks to a highly effective eradication campaign launched by the World Health Organization. Because of increased immigration and tourist travel, the number of cases of these diseases in the United States may escalate.

Cultural aspects in the treatment of patients with skin disease.

As the cultural distance between patient and physician widens, so does miscommunication. Negotiation between the belief systems of the physician and the patient may create a therapeutic environment of mutual trust and respect that increases the possibility of successful clinical outcomes.

Skin conditions of baseball, cricket, and softball players.

Each year in the United States over 80 million people participate in bat-and-ball sports, for example baseball and softball. Cricket, the world's second most popular sport, is enjoyed by hundreds of millions of participants in such countries as India, Pakistan, Australia, New Zealand, Bangladesh, South Africa, West Indies, Sri Lanka, United Kingdom, and Zimbabwe. Although any player can develop skin disease as a result of participation in these bat-and-ball sports, competitive team athletes are especially prone to skin problems related to infection, trauma, allergy, solar exposure, and other causes. These diseases can produce symptoms that hinder individual athletic performance and participation. In this review, we discuss the diagnosis and best-practice management of skin diseases that can develop as a result of participation in baseball, softball, and cricket.

Skin conditions in figure skaters, ice-hockey players and speed skaters: part I - mechanical dermatoses.

Figure skaters, ice-hockey players and speed skaters experience a range of dermatologic conditions and tissue-related injuries on account of mechanical trauma, infectious pathogens, inflammatory processes and environmental factors related to these competitive pursuits. Sports medicine practitioners, family physicians, dermatologists and coaches should be familiar with these skin conditions to ensure timely and accurate diagnosis and management of affected athletes. This review is Part I of a subsequent companion review and provides a comprehensive review of mechanical dermatoses experienced by ice-skating athletes, including skater's nodules and its variants, pump bumps, piezogenic pedal papules, talon noir, skate/lace bite, friction bullae, corns and calluses, onychocryptosis, skater's toe and skate blade-induced lacerations. These injuries result from friction, shear forces, chronic pressure and collisions with surfaces that occur when athletes endure repetitive jump landings, accelerated starts and stops and other manoeuvres during rigorous training and competition. Ill-fitting skates, improper lacing techniques and insufficient lubrication or protective padding of the foot and ankle often contribute to the development of skin conditions that result from these physical and mechanical stresses. As we will explain, simple measures can frequently prevent the development of these conditions. The treatment of skater's nodules involves reduction in chronic stimulation of the malleoli, and the use of keratolytics and intralesional steroid injections; if malleolar bursitis develops, bursa aspirations may be required. Pump bumps, which result from repetitive friction posteriorly, can be prevented by wearing skates that fit correctly at the heel. Piezogenic pedal papules may be treated conservatively by using heel cups, compressive stockings and by reducing prolonged standing. Talon noir usually resolves without intervention within several weeks. The treatment of skate bite is centred on reducing compression by the skate tongue of the extensor tendons of the anterior ankle, which can be accomplished by use of proper lacing techniques, increasing pliability of the skate tongue and using protective padding, such as Bunga Pads™. Anti-inflammatory medications and cold compresses can also help reduce inflammation. Friction bullae are best managed by careful lancing of painful blisters and application of petrolatum or protective dressings to accelerate healing; preventative measures include the use of well fitting skates, proper lacing techniques and moisture-wicking socks. Corns and calluses are similarly best prevented by the use of well fitted skates and orthotic devices. Symptomatic, debridement reduces the irritant effect of the thick epidermis, and can be accomplished by soaking the area in warm water followed by paring. Application of creams with high concentrations of urea or salicylic acid can also soften callosities. Cases of onychocryptosis benefit from warm soaks, antibiotic ointments and topical steroids to reduce inflammation, but sometimes chemical or surgical matricectomies are required. Preventative measures of both onychocryptosis and skater's toe include cutting toenails straight across to allow for a more equal distribution of forces within the toe box. Finally, the prevention and treatment of lacerations, which constitute a potentially fatal type of mechanical injury, require special protective gear and acute surgical intervention with appropriate suturing. The subsequent companion review of skin conditions in ice skaters will discuss infectious, inflammatory and cold-induced dermatoses, with continued emphasis on clinical presentation, diagnosis, treatment and prevention.

Skin conditions in figure skaters, ice-hockey players and speed skaters: part II - cold-induced, infectious and inflammatory dermatoses.

Participation in ice-skating sports, particularly figure skating, ice hockey and speed skating, has increased in recent years. Competitive athletes in these sports experience a range of dermatological injuries related to mechanical factors: exposure to cold temperatures, infectious agents and inflammation. Part I of this two part review discussed the mechanical dermatoses affecting ice-skating athletes that result from friction, pressure, and chronic irritation related to athletic equipment and contact with surfaces. Here, in Part II, we review the cold-induced, infectious and inflammatory skin conditions observed in ice-skating athletes. Cold-induced dermatoses experienced by ice-skating athletes result from specific physiological effects of cold exposure on the skin. These conditions include physiological livedo reticularis, chilblains (pernio), Raynaud phenomenon, cold panniculitis, frostnip and frostbite. Frostbite, that is the literal freezing of tissue, occurs with specific symptoms that progress in a stepwise fashion, starting with frostnip. Treatment involves gradual forms of rewarming and the use of friction massages and pain medications as needed. Calcium channel blockers, including nifedipine, are the mainstay of pharmacological therapy for the major nonfreezing cold-induced dermatoses including chilblains and Raynaud phenomenon. Raynaud phenomenon, a vasculopathy involving recurrent vasospasm of the fingers and toes in response to cold, is especially common in figure skaters. Protective clothing and insulation, avoidance of smoking and vasoconstrictive medications, maintaining a dry environment around the skin, cold avoidance when possible as well as certain physical manoeuvres that promote vasodilation are useful preventative measures. Infectious conditions most often seen in ice-skating athletes include tinea pedis, onychomycosis, pitted keratolysis, warts and folliculitis. Awareness, prompt treatment and the use of preventative measures are particularly important in managing such dermatoses that are easily spread from person to person in training facilities. The use of well ventilated footgear and synthetic substances to keep feet dry, as well as wearing sandals in shared facilities and maintaining good personal hygiene are very helpful in preventing transmission. Inflammatory conditions that may be seen in ice-skating athletes include allergic contact dermatitis, palmoplantar eccrine hidradenitis, exercise-induced purpuric eruptions and urticaria. Several materials commonly used in ice hockey and figure skating cause contact dermatitis. Identification of the allergen is essential and patch testing may be required. Exercise-induced purpuric eruptions often occur after exercise, are rarely indicative of a chronic venous disorder or other haematological abnormality and the lesions typically resolve spontaneously. The subtypes of urticaria most commonly seen in athletes are acute forms induced by physical stimuli, such as exercise, temperature, sunlight, water or particular levels of external pressure. Cholinergic urticaria is the most common type of physical urticaria seen in athletes aged 30 years and under. Occasionally, skaters may develop eating disorders and other related behaviours some of which have skin manifestations that are discussed herein. We hope that this comprehensive review will aid sports medicine practitioners, dermatologists and other physicians in the diagnosis and treatment of these dermatoses.