Erythema scarlatiniforme desquamativum recidivans localisatum

Two patients are reported who had the clinical picture of localized erythema scarlatiniforme desquamativum recidivans. The lesions were thought to be drug induced in a 56-year-old female patient. In the other 54-year-old male patient, staphylococcal infections may have caused the rash.     

Recurring localized scarlatiniform scaled erythema Féréol-Besnier

Recurring scarlatiniform scaled erythema of Féréol-Besnier is a rare disease characterized by recurrent episodes of a prodromal phase with general malaise, head and muscle aches, gastrointestinal complaints and fever followed by an erythematous rash leading to extensive desquamation of the involved skin. It exists in a generalized and localized variant, the latter mainly involving the hands and feet. Its cause is unknown, although it has been speculated that a hyperergic reaction to infectious agents or medications may be etiopathologically involved. A typical case of the localized variant of this obscure disease is described and the common literature is reviewed.     

Epidermolytic hyperkeratosis

A 13-year-old boy presented to the dermatology clinic for treatment of a congenital ichthyosis with a history of generalized erythroderma and trauma related blistering at the time of birth. At the time of presentation he was noted to have red corrugated hyperkeratotic plaques involving the joint flexures, dorsal hands, and neck. Epidermolytic hyperkeratosis is a rare autosomal dominant genodermatosis that presents at birth with generalized erythema, blisters and erosions. In the subsequent months after birth erythema and blistering improves but patients go on to develop hyperkeratotic scaling that is especially prominent along the joint flexures, neck, hands and feet. The disease is caused by mutations in either keratin 1 or keratin 10. Treatment options include urea or alpha-hydroxy acid containing creams as well as topical and systemic retinoids. Epidermolytic hyperkeratosis is also known as bullous congenital ichthyosiform erythroderma (of Brocq) and disorder of cornification type 3.     

Das Erythema scarlatiniforme desquamativum recidivans

Zusammenfassung Auf Grund von 4 eigenen Beobachtungen wird das seltene Krankheitsbild des Erythema scarlatiniforme desquamativum recidivans, seine Differentialdiagnose und Ätiologie besprochen. Es ist klinisch vom Scharlach und dem medikamentösen skarlatiniformen Exanthem meist einwandfrei zu trennen, jedoch kommen Übergangsfälle vor. Nach dem heutigen Wissen ist die Krankheit pathogenetisch und ätiologisch zu den Allergien zu zählen.Mit 7 Textabbildungen.     

泛发性连续性肢端皮炎1例

患者女,35岁。双手足反复起脓疱、糜烂伴疼痛26年,泛发加重6天。体检发现躯干、四肢片状水肿性红斑,其上可见簇集分布的脓疱。双手足可见片状红斑、糜烂,部分表面覆黄色痂皮及散在分布的粟粒大脓疱。双手指甲可见甲周红肿,部分甲下积脓、覆黄色厚痂,甲板缺失、指甲脱落,远端指骨萎缩。可见沟纹舌。诊断:泛发性连续性肢端皮炎。     

硬皮病样表现伴关节挛缩的慢性移植物抗宿主病一例

患儿,男,5岁。双手足质硬、红斑、关节挛缩2年。患儿自幼反复罹患肺脓肿并伴有发热,4年前行基因检测,诊断为JAK3基因相关重型联合免疫缺陷症,遂行异基因造血干细胞移植。2年前先于足部出现脱皮,后双手足背、腕、肘、膝及髋关节处出现红斑、脱皮、硬化,伴手足关节挛缩。皮肤组织病理示移植物抗宿主反应并病毒感染。     

Papular-purpuric "gloves and socks" syndrome

Papular-purpuric "gloves and socks" syndrome is a recently isolated acute self resolving disease. It is characterised by symmetrical cutaneous lesions on the hands and feet, with a sharp limit at the wrists and ankles, presenting a gloves and socks look. Lesions begin as a pruritic oedema and erythema and turn purpuric. Oral mucosal lesions, high fever and arthralgia are also present. Parvovirus B19 has been implicated in this exanthematic disease in about 50% of the cases. We recently observed two typical cases of this syndrome; only one patient had serologic evidence of recent infection by Parvovirus B19.     

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...     

Infantile acropustulosis

Infantile acropustulosis starts in the first few months of life with the recurrent appearance of pruritic intraepidermal pustules containing neutrophils. Predilection sites are the hands and feet. Episodes of pustular eruptions lasting from 8 to 14 days are followed by longer intervals with no symptoms. However, some infants present with skin lesions more widely distributed over the body and with eosinophils as a major constituent of the pustules. In these cases differentiation from other eosinophilic pustular dermatoses of infancy, e.g., the pustular variant of toxic erythema, eosinophilic pustular folliculitis, and transient neonatal pustular melanosis, may become difficult. The etiology of infantile acropustulosis is unknown. The disease clears up spontaneously within the first few years of life. In some cases DADPS therapy has been of benefit.     

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.     

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.     

Cryopyrin蛋白相关周期性综合征一例

患者女,29岁.反复面部、躯干弥漫性红斑26年.手、足关节变形14年.皮疹每日发作,伴寒战、低热、眼红、关节痛.体检:身材矮小,面容特殊,呈塌鼻,额部凸出样,双眼结膜充血,而部、躯干弥漫性红斑,手、足关节,变形.面部经冷风刺激后,皮疹加重呈典型风团.根据典型症状诊断为Cryopyrin蛋白相关周期性综合征.     

Primary erythromelalgia: case report

Erythromelalgia is a rare clinical syndrome characterized by heat, redness and intermittent pain in the extremities, being most frequent the bilateral development in the lower extremities. Local cooling brings relief to symptoms, while heating, physical exercises and use of stockings/socks intensify the discomfort. This condition can be primary or idiopathic or secondary to haematological disorders and vascular inflammatory and degenerative diseases. It is reported the case of an eighteen-year-old male who presented, at the early age of two, development of the symptoms of erythema ,heat and pain followed by desquamation of hands and feet, in outbreaks, with intervals 4 to 5 years long between the crises.     

Pustular skin disorders: diagnosis and treatment

The differential diagnosis for pustular skin disorders is extensive. The distribution of the lesions and the age of the patient are characteristics that may provide strong clues to the etiology of cutaneous pustular eruptions. In adults, generalized pustular dermatoses include pustular psoriasis, Reiter's disease and subcorneal pustular dermatosis. Medications can cause generalized pustular eruptions, such as in the case of acute generalized exanthematous pustulosis; or more localized reactions, such as acneiform drug eruptions, which usually involve the face, chest and back. Localized pustular eruptions are seen on the hands and feet in adults with pustulosis palmaris et plantaris and acrodermatitis continua (both of which may be variants of psoriasis); on the face in patients with acne vulgaris, rosacea, and perioral dermatitis; and on the trunk and/or extremities in patients with folliculitis. A separate condition known as eosinophilic folliculitis occurs in individuals with advanced human immunodeficiency disease. Severely pruritic, sterile, eosinophilic pustules are found on the chest, proximal extremities, head and neck. Elevated serum immunoglobulin E and eosinophilia are often concurrently found. In neonates, it is especially important to make the correct diagnosis with respect to pustular skin disorders, since pustules can be a manifestation of sepsis or other serious infectious diseases. Generalized pustular eruptions in neonates include erythema toxicum neonatorum and transient neonatal pustular melanosis, both of which are non-infectious. Pustules are seen in infants with congenital cutaneous candidiasis, which may or may not involve disseminated disease. Ofuji's syndrome is an uncommon generalized pustular dermatosis of infancy with associated eosinophilia. As in adults, neonates and infants may develop acne or scabies infestations. In this article, we review the most common pustular dermatoses and offer a systematic approach to making a diagnosis. We also report the most up-to-date information on the treatment of these various cutaneous pustular conditions.     

Gloves and socks syndrome associated with parvovirus B19 infection

The spectrum of disease caused by parvovirus B19 infection ranges from asymptomatic to a serious disease. Parvovirus B19 plays a role in the pathomechanism of gloves and socks syndrome, erythema infectiosum, acute polyarthralgia, aplastic crisis in persons with hemolytic anemia, and birth defects. Gloves and socks syndrome has an acute febrile course. Painful edema of hands and feet, and numerous small confluent petechiae in the same localization are the most characteristic signs. Blisters and erosions may occur in oral mucosa. General discomfort and arthralgia accompany skin lesions. The disease tends to be self-limiting. A typical case of gloves and socks syndrome in a 36-year-old woman is reported. Serological tests disclosed the parvovirus B19 infection, confirming the causal relationship between the syndrome and this infection.     

Novel SLC39A4 mutations in acrodermatitis enteropathica

Acrodermatitis enteropathica is an autosomal recessive disease characterized by skin involvement due to defective intestinal zinc absorption. Usually, the skin lesions include erythema, erosions, and small blisters in perioral, perianal regions, and hands and feet, which develop soon after weaning from the breast. The acrodermatitis enteropathica gene has been localized to chromosomal region 8q24.3 and subsequently the SLC39A4 gene has been disclosed as the acrodermatitis enteropathica gene. SLC39A4 mutations have been demonstrated in several acrodermatitis enteropathica families, and in this study we have examined two Japanese acrodermatitis enteropathica families for SLC39A4 mutations. The mutation detection strategy consisted of polymerase chain reaction amplification of all 12 exons and flanking intronic sequences, followed by direct nucleotide sequencing. It revealed three novel mutations, 1017ins53, which creates a premature termination codon, and two mis-sense mutations, R95C and Q303H.     

Recurrent toxin-mediated perineal erythema: eleven pediatric cases

BACKGROUND: Recurrent toxin-mediated perineal erythema is a cutaneous disease mediated by superantigens made by staphylococci and streptococci, which, to our knowledge, has only been reported in young adults. We describe recurrent toxin-mediated perineal erythema in 11 children and outline the differences between recurrent toxin-mediated perineal erythema and Kawasaki disease in this age range. OBSERVATIONS: Eleven children (7 male and 4 female) presented with the sudden appearance of asymptomatic erythema, which was salmonlike in color and rapidly desquamating, involving the perineum in 10 patients and extending to the perianal area in 1 patient. At the onset of the rash, all patients were in good health, although 9 had mild fever for 1 to 2 days before its appearance. Physical examination also revealed an erythema of the hands and feet in 4 patients and strawberry tongue in 7. Two patients had a facial impetigo, and another showed a perianal streptococcal dermatitis. A group A beta-hemolytic streptococcus was isolated from the throat in 10 cases and from a perianal culture in 1 case. In 8 cases, resolution was spontaneous, but all patients were treated with systemic antimicrobial therapy for 10 days. Three patients had a personal history of cutaneous rashes on the perineal area during the last years before consultation. Rash recurrence was observed in 3 of the 11 patients at the follow-up examination. Conclusion Recurrent toxin-mediated perineal erythema can be observed not only in young adults but also in childhood.     

A Sporadic Case of Mal de Meleda Caused by Gene Mutation in SLURP-1 in Korea

Mal de Meleda (MDM), also known as keratoderma palmoplantaris transgrediens, is a rare inherited form of palmoplantar keratoderma. It is characterized by erythema and hyperkeratosis of the palms and soles, extending to the dorsal aspects of the hands and feet. A 15-year-old Korean female presented with sharply demarcated hyperkeratotic plaques on the palms and soles, which extended to the dorsal surfaces of the hands and feet, in a "glove-and-socks" distribution. The histopathologic study showed marked hyperkeratosis, acanthosis, and normogranulosis, without epidermolysis. Her genetic study detected compound heterozygous mutation in exon 3 of the ARS gene encoding SLURP-1. Family history did not reveal any other affected members and no consanguineous relationship was found. In view of these findings, we diagnosed this case as the first reported sporadic case of MDM in Korea, the farthest location from the endemic island of Meleda.     

Allergic contact dermatitis from Basic Red 46 in flame-retardant work clothing

A 55-year-old man presented with a 6-month history of widespread erythema and itching, involving the entire body except for the head, hands, feet, and the axillary vault, since beginning a job that required him to wear flame-retardant clothing made of red Nomex® fabric. His problem tended to disappear when he was removed from his work environment. There was no personal or family history of atopy.     

Toxic erythema of palms and soles associated with high-dose mercaptopurine chemotherapy

We encountered a case of distinctive palmar-plantar erythema with desquamation of the fingers in a patient receiving high-dose mercaptopurine combined with allopurinol. He was receiving 400 mg/d of mercaptopurine with 200 mg/d of allopurinol when a painful, livid erythema involving his hands and feet developed. Over the ensuing 24 hours, desquamation of the distal fingertips was noted. The mercaptopurine was discontinued and the patient was treated with topical fluocinonide ointment under occlusion. Over the next 96 hours, the erythema and pain resolved entirely. To date, this is the eighth case of a painful desquamating erythema of the palms and soles occurring as a complication of chemotherapy. We suggest that high-dose mercaptopurine combined with allopurinol that blocks xanthine oxidase, a necessary enzyme in the catabolism of mercaptopurine, was responsible for our patient's clinical presentation.