A new variant of punctate acrokeratoderma associated with a pigmentary disorder

We report a Japanese patient with skin-coloured keratotic papules on the hands and feet, and pronounced hyperkeratosis of the palms and soles. There was no evidence of fragmentation of dermal elastic tissue on histology. Freckle-like pigmentation was also present on the dorsa of the hands and feet, and this appeared to be closely related to the keratotic disorder. The mode of inheritance was autosomal dominant. We consider our case to be a new variant of familial punctate acrokeratoderma. associated with a pigmentary disorder.     

两足一手型手足癣致病菌种红色毛癣菌的基因型分析

目的对两足一手型手足癣患者致病菌种红色毛癣菌进行菌株水平的基因型分析,通过手足间菌株基因型异同推测手足间菌株传染途径。方法扩增红色毛癣菌NTS区的串联重复系列TRS-1区,对临床分离菌株进行基因分型。结果共分析了229株红色毛癣菌,包括从63例两足一手型手足癣患者手足部位分离到173株红色毛癣菌和15例两足两手型手足癣患者手足部获得56株红色毛癣菌。两组中,80%以上的患者手足部位菌株基因型相同,且两组患者基因型无统计学差异。结论两足一手型手足癣患者手部菌株主要来自其感染的足部。     

Acral lichen sclerosus et atrophicus

BACKGROUND: Lichen sclerosus et atrophicus rarely affects the feet or hands and in this case, it is generally part of widespread cutaneous involvement. We report a case of lichen sclerosus et atrophicus involving only the extremities and the vulvar and perigenital area. PATIENTS AND METHODS: A 56-year-old woman presented with lesions of the hands and feet, with ivory white papules on the dorsal aspect of the feet and the distal phalanx of the fingers, a few small keratotic papules with central depressions in the hollow of the palms, erythema on soles and thenar and hypothenar eminences. Further examination revealed lichen sclerosus et atrophicus of the vulva and genitocrural skinfolds. Histological study of these various cutaneous lesions yielded similar results and revealed the typical features of lichen sclerosus et atrophicus. DISCUSSION: A few cases of lichen sclerosus et atrophicus confined to the hands and/or feet have been reported, involving the palms and soles or nail folds, but none has so far affected the genitalia. To our knowledge, no cases of lichen sclerosus et atrophicus involving both faces of the hands and feet and the genital region have ever been reported.     

骨膜增生厚皮症1例

患者男,21岁。头面部回状头皮伴脂溢、多汗,双手指、足趾增大3年。患者临床表现为皮肤增厚、杵状指/趾。实验室检查正常。X线片示:双手及足骨皮质增厚。诊断:骨膜增生厚皮症。     

Dorsal sensory impairment in hands and feet of people affected by Hansen's disease in Israel

INTRODUCTION: Sensory testing in people affected by Hansen's disease is usually performed on palms and soles only. In Israel, both palmar/plantar and dorsal aspects of limbs are routinely tested. OBJECTIVES: The aim of this study was to describe the magnitude of dorsal sensory impairment (SI) in limbs and compare the frequency of SI on palms and soles with that on the dorsum of hands and feet. DESIGN: In a cross-sectional study, limbs of 140 patients registered at The Israel Hansen's Disease Centre during the years 1999-2003 were tested for their sensory status. Both palmar/plantar and dorsal aspects were tested using Semmes-Weinstein monofilaments. SI was defined as not feeling stimuli applied with the 2 g monofilament. RESULTS: SI was detected on the dorsum in 43% of sites on hands and only in 27% on palms. 64% of sites on dorsum of feet had SI compared to 53% on the soles. SI was detected in up to 18% in hands with no palmar SI, and in 6% of feet with no plantar SI. Furthermore, SI on palms and soles was found to be accompanied by dorsal SI in all hands and in 97% of feet. CONCLUSION: SI on dorsum of limbs occurs more frequently than SI on palms and soles. Therefore sensory testing should also consider inclusion of the dorsal aspect of hands and feet.     

Multiarticular Tophaceous Gout with Severe Joint Destruction: A Pictorial Overview with a Twist

Tophi are the visible dermatological signs of gout. A case of tophaceous gout in a middle-aged man with no other metabolic derangement is being presented with multiple tophi on the hands and feet overlying joints as well as on the fingers and toes. We thought it to be of educational value to demonstrate needle-like crystals of urate by polarizing microscopy. X-rays of hands and feet showed dramatic destructive changes. The patient presented with mottled hypopigmentation on anterior and posterior knees and dorsa of hands and feet where he applied hot “aankda” leaves and covered them with bandage resulting in irritant dermatitis with postinflammatory hypopigmentation. This proved to be a red herring in this case.     

Extensive hemangiomatosis of the extremities with the same histopathological pattern as the early lesion of Kaposi's sarcoma

For 13 years a 28-year-old man had on his hands and feet bluish red patches, which had slowly progressed in size. Biopsy specimens exhibited an abnormal vascular pattern similar to that seen in early lesions of Kaposi's sarcoma. Skeletal radiographs of the hands and feet showed lesions indicating hemangiomatosis.     

Erythema scarlatiniforme desquamativum generalisatum

Erythema scarlatiniforme desquamativum generalisatum (Féréol-Besnier disease) is a rare skin disease characterized by generalized erythematous rash with subsequent desquamation. An 86-year-old woman presented with generalized erythema followed by an extensive, scarlatiniform peeling especially of the hands and feet. This generalized episode may be followed by erythema scarlatiniforme desquamativum localisatum recidivans, which is a recurring variant of the disease, localized to the hands and feet.     

Palmoplantar keratoderma of Sybert

A 13-year-old boy and a 7-year-old boy, who are brothers, presented with a life-long history of erythema, hyperkeratosis, and desquamation of the hands and feet. Symptoms improved with the use of topical glucocorticoids and keratolytics. PPK of Sybert is characterized by palmoplantar hyperkeratosis with transgrediens, autosomal dominant inheritance, and the absence of associated systemic features.     

Leprosy: recognizing red flags

A 28-year-old man from the Philippines presented with multiple papules and plaques symmetrically distributed on the arms and legs. This was associated with worsening paraesthesia of the hands and feet. A right common peroneal nerve decompression had been performed 2 years earlier. He was diagnosed with multibacillary leprosy on skin biopsy and subsequently treated with oral rifampicin, clofazamine and dapsone.     

Bullous Pemphigoid in Infancy: Case Report and Literature Review

Abstract: We report a 2-month-old girl who developed bullous pemphigoid on her hands and feet shortly after receiving her routine immunizations. Infantile bullous pemphigoid has a clinical presentation distinct from bullous pemphigoid seen in older children and should be included in the differential diagnosis of blisters involving the hands and feet. Our patient responded well to topical corticosteroid therapy.     

进行性对称性红斑角化症1家系报道

患者女,26岁。出生后6个月发病,双手、足对称性红斑角化25年。体检:一般情况良好。双侧掌跖及腕关节屈侧对称性分布边界清楚的角化性红斑,边缘覆以少许脱屑,压之不褪色,指/趾甲均未受累。实验室检查未见异常。手背侧缘皮损组织病理示:表皮明显角化过度,伴轻度角化不全,颗粒层及棘层肥厚,表皮增生至同一水平线,真皮小血管轻度扩张充血,管周少量淋巴细胞浸润。诊断:进行性对称性红斑角化症。     

Grotton's acrogeria with bone involvement

A 13-year-old girl had a bird-like face, deficiency of the subcutaneous fatty tissue, dry, thin, transparent and wrinkled skin, especially on the hands and feet, prominent veins and telangiectasia and mottled hyper-pigmentation. X-ray studies revealed acro-osteolysis of the hands and feet. The clinical features corresponded well with Gottron-type acrogeria. The clinical symptoms of premature ageing syndromes, such as progeria, lipodystrophia totalis, Cockayne syndrome, metageria and acrogeria are summarized briefly and compared with the clinical picture observed in our patient.     

Acrokeratoelastoidosis

Two cases of acrokeratoelastoidosis comprised of smooth shiny papules on the hands, feet and legs, are reported. Focal acral hyperkeratosis, degenerative collagenous plaques of the hands and keratoelastoidosis marginalis of the hands are three closely related conditions.     

进行性肢端黑变病

报告1例进行性肢端黑变病。患者男，15岁。双手（足）、指（趾）甲、面颊、耳郭出现褐色斑疹9年。皮肤科检查示双手（足）背侧及掌（跖）侧缘、面颊、耳郭、指（趾）甲弥漫分布暗褐色斑疹及斑片，双手（足）背关节处及耳郭皮肤轻度萎缩、变硬，手指间关节屈侧可见黑褐色色素性条纹，指（趾）甲有纵行的黑褐色色素线。4个月后手（足）背、掌（跖）侧缘、面颊及耳郭弥漫性暗褐色斑疹稍减退，耳郭呈现蛛网状线状褐色斑。组织病理检查示基底层黑素细胞增多，透明细胞呈簇状，黑素增加，真皮浅层见大量噬黑素细胞及黑素颗粒。     

中国蒙古族进行性对称性红斑角化症伴真菌感染1例

患者女,28岁,蒙古族,双手足背、双掌跖、双腕部及双踝部对称性红斑角化28年,手足脱屑伴甲改变20年.皮肤科情况:双手足背、双掌跖、双腕部及双踝部边界清楚、对称分布红斑,掌跖角化明显,伴有脱屑,左手拇指甲和右足拇趾甲部分颜色变黄及浑浊肥厚.实验室检查:手足、左手拇指甲和右足拇趾甲真菌镜检及培养阳性,真菌培养示红色毛癣菌...     

A Case of Dermatopathia Pigmentosa Reticularis

Dermatopathia pigmentosa reticularis (DPR) is a very rare disorder with the diagnostic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. Many other dermatologic findings have been associated with this triad, including adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and nonscarring blisters on the dorsa of the hands and feet. The mode of inheritance is unclear but may be autosomally dominant. To our knowledge, only 11 cases have been reported in the world, and none has previously been described in the Orient. We present a Korean patient with the typical features of the DPR triad, along with adermatoglyphia, hypohidrosis, and nonscarring blisters on the dorsa of the feet.     

Photo-onycholysis due to sparfloxacin

This paper describes a case of sparfloxacin-induced photodermatitis associated with photo-onycholysis in a 36-year-old man. He was being treated with sparfloxacin, streptomycin, ethambutol and pyrazinamide for pulmonary tuberculosis. He developed an exaggerated sunburn-like rash over the face and the dorsa of hands and feet, and painful onycholysis of finger- and toe-nails. Withdrawal of sparfloxacin resulted in resolution of the skin rash and nail tenderness.     

Acral localized acquired cutis laxa associated with rheumatoid arthritis.

We report the first case of the acral localization of the acquired form of cutis laxa associated with severe rheumatoid arthritis. The skin laxity was preceded by episodes of itching and swelling of the hands and feet. Histopathology showed that the elastic fibers were lost in the areas of cutis laxa and decreased in adjacent skin. The pathogenetic relationship with rheumatoid arthritis or the intake of related drugs is discussed.     

Treatment of hyper-IgE dermatitis with interferon-γ

Background Interferon-γ has been suggested as causal therapy in both hyper-IgE syndrome and severe atopic dermatitis. Aim To investigate the therapeutic effect of short-term, low-dose interferon-γ both as monotherapy and in combination with topical treatment, in a patient with a variant of the hyper-IgE syndrome. Method Interferon-γ was administered subcutaneously (50 μ/m² X 3 d/w) for 4 weeks. Three regions were studied: hands, flank and feet. The hands were treated with betamethasone-17–valerate cream and the feet with mupirocin ointment, while the flank was treated only with a moisturizer. Results All regions showed significant reductions of the clinical scores (0.0001 < P < 0.0280), but hands and feet showed significantly greater reduction than Hank (P < 0.004). Conclusions The results suggest that short-term, low-dose interferon-γ treatment is a potentially useful therapy in hyper-IgE syndrome dermatitis when used in combination with topical mupirocin or a steroid.