A Case of Post-kala-azar Dermal Leishmaniasis

Only a few cases of Post-kala-azar dermal leishmaniasis have been reported in Japan, especially recently. We describe a case of a 32-year-old woman who developed rose-colored nodules on her forearms two years after Kala-azar. A skin biopsy specimen from a nodule revealed not only granulomatous changes but also many amastigotes of Leishmania donovani in macrophages. Rose-colored nodules were also distributed on her face and neck. Treatment with antimony compound was very effective.     

Cataract and retinal detachment in patients with severe atopic dermatitis who were withdrawn from the use of topical corticosteroid.

Many patients with severe atopic dermatitis (AD) in Japan are afflicted with persistent erythema of the face (atopic red face) that is not only resistant to topical corticosteroid, but often becomes worse with its use. During a three-year period (1991-1993), we treated 79 inpatients with severe AD by a combination of careful daily skin care, use of emollients, and exclusion of exacerbating factors. Occular complications before and after treatment were examined in these cases. After withdrawal of topical corticosteroid, almost all of the patients showed a temporary worsening of their skin condition. Immediately thereafter, their occular symptoms did not change. Cataract was found in 20 cases (25.3%), and retinal detachment in 9 (11.4%). After 2 months, 11 cases of cataract and 5 cases of retinal detachment in the peripheral retina were observed. However, these incidences were similar to the numbers reported in Japan during conventional treatment with topical corticosteroid. The development of cataract or retinal detachment had no relationship to serum IgE levels, personal history of respiratory atopy, the duration of topical corticosteroid use on the face, or treatment with systemic corticosteroid. Our observations suggest that patients who habitually tap or rub their faces strongly tend to develop cataract or retinal detachment at a statistically significant higher frequency. Patients with AD should have ophthalmologic examinations every one to two months for at least one year after a facial oozing attack or withdrawal of corticosteroid.     

Erythema nodosum-like eruption in sarcoidosis

Two cases of sarcoidosis with tender, erythematous nodules on the legs are reported. The cutaneous lesions were clinically similar to those of erythema nodosum, but histologically showed non-caseating epithelioid granulomas. A review of 14 cases of this particular sarcoid eruption reported in Japan showed that 13 had ocular involvement as in our cases. In the light of the high frequency of ocular involvement, a skin biopsy should be considered in patients presenting with erythema nodosum-like eruptions situated on the legs.     

Reversal effects of topical retinoic acid on the skin of kidney transplant recipients under systemic corticotherapy

The systemic long-term corticosteroid treatment administered to kidney graft recipients (KGR) within the framework of the required immunosuppressive therapy induces an atrophy of the skin, from the sixth month onwards. We studied the effect of topical all-trans retinoic acid (0.05%; Galderma Labs.) applied to the forearms of 27 KGR (14 men, 13 women) over a 6-month period. Twenty-four subjects completed the trial. The following results were obtained in the treated forearm versus the untreated forearm (excipient alone): clinically, an increase in skin thickness; by noninvasive techniques, an increase in skin thickness, skin elasticity, skin conductance, and TEWL, and a reduction in the size of the corneocytes. No change in stratum corneum lipid content was observed. A sex-related difference was noted in the response to treatment under our experimental conditions, the female patients responding better. A punch biopsy (4 mm) was performed on both forearms of four patients after the 6-month period. Histologic and ultrastructural examination revealed epidermal and dermal changes evoking increased cellular metabolism in the retinoic acid-treated forearms.     

Buruli ulcer and current situation in Japan: A new emerging cutaneous Mycobacterium infection

Buruli ulcer (BU) is a new emerging disease and the third most common chronic mycobacterial infection in humans, caused by Mycobacterium ulcerans. Approximately 5000 cases are reported annually from at least 33 countries around the globe, but more from the tropical nations. A total of 32 cases have been reported from Japan sporadically since 1980. None of the cases were related to international travel. Of the total reported, M. ulcerans ssp. shinshuense, a subspecies speculated to be domestic to Japan or in Asia, has been isolated from 23 cases. The mode of transmission and its incubation period remain unclear, despite several proposed hypotheses, including several vectors and cutaneous wound as port of entry for the pathogen. M. ulcerans invades the skin, subcutaneous tissue, fascia and eventually forms extensive ulceration. Smear, culture, histopathology and polymerase chain reaction are established diagnostic tools to identify M. ulcerans. Multiple antimicrobial therapy is a commonly used therapeutic method, but patients often need extensive debridement and, at times, skin grafting, especially when diagnosis is delayed. Thus, expanding a system for improved awareness and diagnosis in Japan and Asia is important, together with elucidating the candidate vector and the mode of transmission. Here, to establish a base for future progress in better understanding of this infectious disease, we reviewed the characteristics of the disease together with an update of reported cases in Japan.     

Scleroedema of Buschke and diabetes mellitus: a report of two cases

Two cases are described of scleroedema of Buschke and diabetes mellitus. These cases illustrate the distinctive natural history of scleroedema where it occurs in association with diabetes mellitus. A possible mechanism for this association is discussed.     

Two cases of L-tryptophan ingestion induced eosinophilia-myalgia syndrome

Eosinophilia-myalgia syndrome (EMS), a new connective tissue disease was reported and named in 1989 from New Mexico, U.S.A. L-tryptophan has been suspected as the causative agent of EMS. This L-tryptophan was made in Japan, but no definite case of EMS has been reported in Japan. We report 2 patients with EMS. A 72-year-old woman and a 74-year-old woman, who had been treated with oral L-tryptophan 1 g/day for 4 and 5 months by the same doctor simultaneously. The clinical courses of the two cases were similar. A diffuse erythema and swelling appeared on the arms and spread over the whole body. The skin lesions turned into lustrous sclerosis. Eosinophilia was remarkable in the early stage. ANA, DNA anti-body and ENA antibodies were all negative. Neurological examination revealed a mild peripheral neuropathy. No sclerodactylia and no Raynaud's phenomenon in our cases are characteristic findings in EMS. The simultaneous onset of two cases using the same drug at the same time implicates the close relation of L-tryptophan ingestion to EMS.     

Evaluation of 2 self-administered questionnaires to ascertain dermatitis among metal workers and its relation with exposure to metalworking fluids

We performed an exploratory study to evaluate 2 self-administered questionnaires assessing hand dermatitis and investigate a possible exposure-response relationship between dermal exposure to semi-synthetic metalworking fluids (SMWF) and dermatitis. In a cross-sectional survey on dermatitis, a symptom-based questionnaire and a picture-based skin screening list were applied in 80 SMWF-exposed workers and 67 referents. To evaluate the accuracy of the questionnaires, 47 subjects were examined by a dermatologist. Dermal exposure levels to SMWF were assessed on hands, forearms, and face with a observational method that was validated using a fluorescent tracer method. The symptom-based questionnaire had a relatively high sensitivity (0.86) but moderate specificity (0.64), and the skin screening list had a low sensitivity (0.36) and a relatively high specificity (0.84). The skin screening list seemed to represent the more severe cases of dermatitis and showed a significant relation with exposure for dermatitis on hands, forearms, or face. In epidemiological surveys where workers are not seen by a dermatologist, the skin screening list seems to be more appropriate to detect cases of dermatitis, as its higher specificity results in less false positives. Alternatively, it would be preferable applying the symptom-based questionnaire; workers with symptoms should be seen by a dermatologist to identify false positives.     

Systemic sclerosis revealing T-cell lymphoma

We describe a case of systemic sclerosis (SSc) occurring together with malignant lymphoma. A 43-year-old man, who had noticed sclerodactyly 1 month before consultation, was admitted for progressive skin sclerosis on his forearms and chest. SSc was diagnosed. Immediately after admission, skin sclerosis rapidly extended to the neck and trunk, and subcutaneous tumors developed on the neck, chest and back. Skin sclerosis was prominent at the sites where subcutaneous tumors were present. The tumors were diagnosed as non-Hodgkin's lymphoma of T-cell phenotype derived from soft tissue. Following 4 cycles of chemotherapy, he had complete remission and the skin sclerosis remarkably improved. It is possible that cytokines produced by T-cell lymphoma cells were responsible for the development of skin sclerosis in this case.     

Comparison of two pH meters used for skin surface pH measurement: the pH meter 'pH900' from Courage & Khazaka versus the pH meter '1140' from Mettler Toledo

Background/aims: Measurement of skin surface pH is used in clinical research to evaluate hazardous shifts in pH following external exposures and to evaluate the state of diseased skin with acute or chronic changes. It is therefore important to measure skin surface pH as precisely as possible. The aim of this study was to compare two commercially available pH meters used for skin surface pH measurement, to reveal differences between them in measured skin pH on the forearm.
Methods: The first pH meter (pH900) had a pointed electrode and a stabilisation period of 3 s. The second pH meter (pH meter 1140) had a circular electrode and no fixed stabilisation period. Twelve healthy subjects (6 male and 6 female Caucasians) entered the study. The pH measurements were performed once an hour from 8 a.m. to 3 p.m. on both forearms in five areas from the elbow to the "wristwatch" zone. In each area, three measurements were performed next to each other with both pH meters (15 measurements per arm per hour per pH meter).
Results: The pH900 has a higher measuring level and a higher variation than the pH meter 1140.
Conclusion: A skin surface pH meter with a circular electrode and with no fixed stabilisation period is preferable. It is recommended that the pH meter be allowed to stabilise for at least 7 s before the result is read.     

A retrospective review of 20 hypertensive leg ulcers treated with mesh skin grafts

BACKGROUND: Few articles have been published about hypertensive leg ulcers and their surgical treatment. Since mid of the year 2000, it has been our policy to treat all hypertensive leg ulcers very early with mesh split-thickness skin grafts. The present series consists of 15 patients whose hypertensive leg ulcers, including five bilateral cases, were treated with 20 mesh grafts from 2000 to 2002. SUBJECTS AND METHODS: All patients, nine women and six men, had a long history of hypertension. The same surgical procedure was applied to all 15 patients: a complete mechanical debridement of all necrotic tissues, immediately followed by mesh skin grafting. RESULTS: Patients were discharged from the hospital after an average post-operative period of 16 days. Upon leaving the hospital, the patients had lesions completely healed in 14 of 20 cases. The graft take had been complete after an average period of 14 days. In six cases, one or two very small patches of skin graft had necrosed and complete healing required an additional period of 1 to 3 months. In all 20 cases, pain had disappeared within 1 week from surgery. DISCUSSION: All patients were on opioid therapy before surgery. With medical treatment only, hypertensive leg ulcers used to heal after a mean period of 15 months. After surgery, the average healing period was 2 weeks and opioids were stopped within 1 month after surgery. CONCLUSION: The review of the present series shows that early mesh grafting of hypertensive leg ulcers is beneficial, because healing is very quick and the pain will disappear quasi-instantly.     

疣状肢端角化症的临床及病理特点研究

目的：探讨疣状肢端角化症的临床及病理特点。方法：分析本文及近40年来13篇文献报道的17例肢端角化症先证者的临床及病理资料。结果：发病年龄，10例（59％）〈20岁，7例（41％）〉25岁；有家族史者10例（59％）。皮损主要分布于双手背、足背及前臂、小腿伸侧。皮损表现16例（94％）为扁平疣样损害，1例表现为寻常疣样损害，6例（35％）伴有掌跖角化，5例（29％）伴有指趾甲受累，3例（18％）伴有毛囊角化病。皮损组织病理均表现为表皮明显角化过度、颗粒层及棘层增厚、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，无角化不全及空泡化细胞。结论：疣状肢端角化症大部分患者有家族史，常于加岁前发病。皮损主要分布四肢伸侧，基本皮疹为扁平疣样的褐色扁平丘疹。皮损病理显示表皮明显角化过度、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，是诊断疣状肢端角化症的主要依据。     

Measurement and perception of skin colour in a skin cancer survey

A population-based prevalence survey of skin cancer was conducted in Nambour, Queensland, in 1986. The skin colour of 807 participants was assessed in three ways: quantitatively, graded by a dermatologist, and self-reported. Quantitation of skin pigmentation was obtained by measuring the reflectance of light of wavelength 650 nm, at six sites. Females showed higher mean reflectance (paler skin) than males at all sites with the greatest difference on the lateral forearms. Prevalent skin cancer in males, and solar keratoses in both sexes were correlated with inherently pale skin colour on an unexposed site, and the presence of keratoses was correlated with darkly-pigmented backs of the hands (P less than 0.001). Both dermatologists' and participants' grading of skin colour were moderately correlated with measured skin colour. For dermatologists, correlation was highest with reflectance from the medial upper arms (r = 0.35, right arm; 0.30, left) in males, and the lateral forearms (r = 0.34, right; 0.38, left) in females. Correlations between reflectance values and self-reported innate skin colour were highest for the upper arms (r = 0.26, right; 0.24, left) in males, and for forearms (r = 0.42, right and left) in females. Prevalence of actinic lesions was more highly correlated with subjectively assessed skin colour than with quantitative skin pigmentation.     

Refined localization of dyschromatosis symmetrica hereditaria gene to a 9.4-cM region at 1q21-22 and a literature review of 136 cases reported in China

BACKGROUND: Dyschromatosis symmetrica hereditaria (DSH) is an autosomal dominant pigmentary genodermatosis characterized by hyperpigmented and hypopigmented macules on the extremities, which has recently been mapped to an 11.6-cM interval on chromosome 1q11-21. So far, most cases of DSH have been reported in Japan and dermatologists around the world might think this disorder mainly occurs in Japan. In fact, there are 17 DSH families including 136 cases reported in China since 1980, but most of them are described in Chinese. OBJECTIVES: To refine the previously mapped region that facilitates the identification of the DSH gene and to delineate the clinical and genetic features of Chinese DSH cases by a literature review of 136 cases reported in China. METHODS: We performed genotyping and linkage analysis using polymorphic microsatellite markers at 1q11-22 in two Chinese DSH families, and reviewed all of the DSH cases reported in China since 1980. RESULTS: A cumulative maximum two-point lod score of 3.68 was produced with marker D1S506 at a recombination frequency of theta = 0.00 in these two families. Haplotype analysis refined the DSH locus to a 9.4-cM interval flanked by D1S2343 and D1S2635. The genetic and clinical features of Chinese cases with DSH were summarized. In some Chinese cases, hyperpigmented and hypopigmented macules were scattered on the neck and chest, but among Japanese patients there were no similar skin lesions to be reported on these sites. CONCLUSIONS: This study confirms linkage of DSH to a previously mapped region and refines the DSH gene to a 9.4-cM interval at 1q21-22. Likewise, the literature review indicates that DSH is not an uncommon disorder in China and the differences in the distribution of skin lesions could be related to race and environment.     

Occupational skin diseases among dental nurses

Out of 923 female dental nurses in the Helsinki district, 799 were interviewed using a computer-assisted telephone interview. A structured questionnaire was used to inquire about skin, respiratory symptoms, atopy, work history and methods, and exposure at work. The 328 nurses, who reported work-related dermatitis on their hands, forearms or face, were invited to an interview by an occupational physician; 245 nurses participated. 31 nurses had previously been diagnosed with an occupational skin disease (OSD). 133 nurses with a suspected OSD were selected for further clinical examinations with prick and patch testing. Among the 107 nurses examined, 22 new cases of OSD were diagnosed. There were altogether 29 cases of allergic contact dermatitis, 15 of contact urticaria, 12 of irritant contact dermatitis, and 1 case of onychomycosis. Rubber chemicals and natural rubber latex (NRL) in protective gloves, as well as dental-restorative plastic materials [(meth)acrylates], were the most common causes of allergy. 42% of the OSD cases in the studied population had been missing from the statistics (Finnish Register of Occupational Diseases). Plastic gloves or NRL gloves with a low-protein content are recommended for dental work. Skin exposure to (meth)acrylates should be avoided.     

TRANSIENT NEONATAL PUSTULAR MELANOSIS

A Mexican-American boy presented at birth with an extensive eruption consisting of 0.5 to 1.0 cm hyperpigmented macules with a distinct peripheral scale involving primarily the forearms, abdomen and lower back (Fig. 1). Rare intact vesicopustules were also identified. There was an unremarkable prenatal history, and the infant was a product of a normal vaginal delivery. With the exception of the skin lesions and moderate hepatosplenomegaly, the physical examination was normal. Gram stains of the pustules showed numerous neutrophils but no bacteria. Bacterial cultures, of the skin and blood, TORCH screen (toxoplasmosis, rubella, cytomegalic virus, and herpes virus) and a VDRL were negative. On the second day of life, the patient developed several pustules with surrounding erythema consistent with erythema toxicum neonatorum. Wright-stained smears of these lesions showed abundant eosinophils. Hepatosplenomegaly resolved by the third day of life and at the time of discharge only hyperpigmented macules persisted. Follow-up visit six weeks later showed no evidence of skin lesions.     

Photodistributed blue-gray pigmentation of the skin associated with long-term imipramine use

A 72-year-old white woman presented with progressively increasing slate-gray pigmentation of the face and extensor aspect of the forearms, which she had been suffering from for 8 years. She had been taking imipramine for approximately 30 years. Her other medications included ranitidine and anacin. Physical examination revealed slate-gray hyperpigmentation of the skin photodistributed on the face (Figs 1, 2) and extensor aspects of the forearms. Relative sparing of the skin folds, mucous membranes, sclerae, teeth, and nails was noted. The remainder of the physical examination revealed no abnormalities. Skin biopsy specimens from the right cheek and right forearm were obtained. Histologic examination revealed collections of variably sized, round to ovoid, yellow-brown globular deposits in the upper and mid dermis (Fig. 2). The deposits were identified within macrophages and free within the dermis. The epidermis was unremarkable and free of deposits. The deposits stained for melanin with a Fontana-Masson stain, but did not stain for iron.     

Cutaneous manifestations of Costello syndrome

Cutaneous findings are common in Costello syndrome, but have not been extensively reviewed in the dermatology literature. We present the cutaneous and histopathologic findings from two cases of Costello syndrome and review previously described cutaneous features of this syndrome. Both patients had manifestations of Costello syndrome with thick, lax skin on the dorsal aspects of hands and feet, deep palmar and plantar creases, curly hair, hyperkeratoses, acanthosis nigricans, papillomas, and multiple pigmented lesions. One patient had multiple syringomas on the forearms, a finding not previously reported. Pigmented lesions have previously been reported as nevi in the literature though no biopsies have been reported. We conclude that thick, loose skin on the dorsal aspects of hands and feet and deep palmar and plantar creases are cardinal manifestations of Costello syndrome and benign tumors of ectodermal origin such as papillomas, calcified epitheliomas, dermoid cysts, mammary fibroadenosis, and syringomas are important features of this syndrome.     

Pachydermoperiostosis--report of a case and review of 121 Japanese cases

A case of 23 years old man with idiopathic pachydermoperiostosis is reported. He showed cutis verticis gyrata, clubbed fingers and periosteal new bone formation without any causative basic disorder. His serum level of FSH, LH, estradiol and estriol were elevated, but their significance was not clear. Histological examination of the skin from the forehead revealed sebaceous hyperplasia and dermal thickening, where deposit of alcian blue and colloidal iron positive substance were detected. The deformed forehead and eyelids were corrected by plastic surgery. One hundred and twenty one cases of pachydermoperiostosis reported so far in Japan are briefly reviewed. Most of them were male (94.1%), about one fourth had a family history. The principal features are: clubbing of the digits (88.4%), periosteal new bone formation (94.1%), coarsening of the facial features with furrowing of the skin of the face (72.7%) and cutis verticalis gyrata (59.5%). Arthralgia (40.5%), hyperhidrosis of the feet and hands (44.6%), gastric hypertrophy (8 cases), gastric ulcers (5 cases) and endocrine abnormalities (17 cases) were also reported.     

Three-year retrospective review of superficial radiotherapy for skin conditions in a Perth radiotherapy unit

Western Australia has only two superficial radiotherapy units, one of which is located at Fremantle Hospital, and run by the radiation oncologists of Perth Radiation Oncology Centre. A 3-year retrospective review was undertaken of all patients who underwent treatment at this unit from 1999 to 2001. Patients were identified from the unit's log book, and data was collected from their files. For malignant skin conditions, 369 lesions were treated in 259 patients over the study period. The patients' median age was 76 years. A wide variety of conditions were treated, but the most common diagnoses were basal cell carcinoma (237 lesions) and squamous cell carcinoma (92 lesions), most commonly located in the head region. The most frequently used treatment schedule was 36 Gy in six fractions over a 3-week period. Where radiotherapy was administered as primary treatment, the diagnoses had been biopsy-proven in only 53% of cases. Fifty-four patients underwent treatment of benign skin disease over the study period; most commonly keloid scars (41 patients) followed by warts (six patients). We conclude that superficial radiotherapy has a distinct role in dermatology, particularly for skin carcinomas around the nose and eyes, which cannot presently be superseded by electron beam therapy.