Localized pachydermodactyly in tuberous sclerosis

Pachydermodactyly refers to a rare form of digital fibromatosis involving the proximal portions of the fingers. There are only nine cases reported in the literature, ill idiopathic and occurring in young men. We report a 5-year-old Chinese boy with tuberous sclerosis who presented with localized pachydermodactyly since birth. This congenital form of pachydermodactyly may represent an additional cutaneous sign of tuberous sclerosis.     

A new subtype of pachydermodactyly: unilateral pachydermodactyly transgrediens

Pachydermodactyly describes a rare condition of localized fibromatosis, usually symmetrically affecting the interphalangeal joints of both hands. We describe a case of a new subtype of pachydermodactyly in a 14‐year‐old boy, which we term ‘unilateral pachydermodactyly transgrediens’. This atypical pattern is caused by specific localized mechanical manipulation of the hands. This condition contributes to the completely indolent spectrum of pachydermodactyly, and usually does not need therapy. Therefore it is essential not to misinterpret it as an inflammatory state such as juvenile idiopathic arthritis. The correct diagnosis of pachydermodactyly and its rare subtypes, as we describe in this case, often spares the affected patients unnecessary invasive diagnostic procedures and immunosuppressive therapy.     

Pachydermodactyly: a rare form of digital fibromatosis—report of four cases

We report four patients with pachydermodactyly, a rare and poorly recognized form of digital fibromatosis characterized by asymptomatic soft-tissue swelling around one or more proximal interphalangeal joint in young men.     

Two Cases of Pachydermodactyly

We report two cases of pachydermodactyly. Case 1 was a 16-year-old girl who complained of asymptomatic, bulbous, firm swellings which developed insidiously on both sides of the proximal interphalangeal (PIP) joint of her right middle finger. Case 2 was a 14-year-old boy with similar lesions on the sides of the PIP joints of the index and middle fingers of both hands. They both had histories of mild, repetitive mechanical trauma of the fingers. Radiologic findings showed soft tissue swellings without any bony or articular abnormalities. Histopathologic findings from the bulbous swellings revealed marked hyperkeratosis, slight epidermal hyperplasia, and a markedly thickened dermis with a deposition of mucinous material among the collagen fibers. Ultrastructural examinations of both cases showed decreased diameters of collagen fibrils. The lesions temporarily improved with intralesional injection of triamcinolone acetonide. Pachydermodactyly is more commonly found in boys and the affected fingers are more numerous in boys. Both of the present cases had the habit of rubbing and gripping their fingers unconsciously. Mechanical trauma of the fingers around puberty may play an important role in pachydermodactyly.     

Dermatologist‐diagnosed skin diseases among immigrant Latino poultry processors and other manual workers in North Carolina,USA

Background Immigrant Latino workers represent an expanding workforce in rural areas of the USA, where their employment is concentrated in occupations such as poultry processing that entail chemical, infectious, and mechanical skin exposures. Occupation‐related skin illnesses in this vulnerable population are not well characterized. Objectives This study was designed to describe the prevalences of skin diseases among immigrant Latino poultry processors and other manual workers in North Carolina. Methods Community‐based sampling was used to recruit 742 immigrant Latino workers, 518 of whom underwent a physical examination supervised by a board‐certified dermatologist. The presence or absence of skin disease on the face, neck, arms, hands, and feet was recorded. Results Workers ranged in age from 18 years to 68 years. Slightly over half of the sample were male (52.6%). Poultry workers represented 55.8% of the study sample. Infectious skin diseases were the most common diagnosis, present in 52.3% of workers. Inflammatory skin diseases were present in 28.2% and pigmentary disorders in 21.8% of workers. The most common skin conditions were tinea pedis (37.6%), onychomycosis (31.9%), scars (13.7%), acne (11.8%), and melasma (9.3%). Age, sex, first language, and work as a poultry processor accounted in part for the prevalence of these diseases. Conclusions Several skin diseases are highly prevalent in immigrant Latino workers and may relate to work environment. These may impair the quality of life of these workers and predispose them to further illness.     

Dermatoses among poultry workers: “Chicken poison disease”

To investigate the causes of skin eruptions affecting poultry workers, we reviewed our medical records and surveyed a southcentral Pennsylvania poultry processing plant. Examination of the records of routine patch test clinic patients seen from January, 1981, to July, 1982, revealed three poultry workers with allergic contact dermatitis and one with irritant contact dermatitis. We then examined one hundred fifty workers at a poultry processing plant, noting the presence of occupational dermatoses, with special interest in the occurrence of contact dermatitis. We found thirteen dermatoses in nine workers. These included Candida infections, irritant contact dermatitis, allergic contact dermatitis, abrasions and cuts, warts, and dyshidrotic eczema.     

Knuckle pads in live-chicken hangers

A study of live-chicken hangers in a poultry processing plant demonstrated a high prevalence of callosities over the knuckles (knuckle pads) of both hands. Knuckle pads were observed in 56% (23/41) of live-chicken hangers, but in no (0/41) workers from other departments (p less than 0.001). The probable cause was the repeated striking and sliding of the knuckles against metal shackles in which live birds were being placed. Additional medical and ergonomic evaluation would be worthwhile to confirm the probable cause, to determine whether associated tissue disorders are present in the digits of chicken hangers who develop knuckle pads, and to suggest preventive measures.     

Fibromatosis of breast mimicking sarcoidosis

Primary mammary fibromatosis is a rare skin condition which can arise after trauma or previous surgery. The exact etiology is unknown. Very few cases have been reported in literature and the main emphasis is to differentiate this condition from breast carcinoma. We report here an unusual case of a 60 year old female who presented with skin lesion which clinically looked sarcoid with history suggestive of sarcoidosis, but on histopathology fibromatosis of breast was revealed. Complete work up ruled out any carcinomatous changes. Surgical excision of the lesion was done with no recurrence seen in one year follow up period.     

Pachydermodactyly: A Case Report

Pachydermodactyly is an uncommon variant of digital fibromatosis that has previously been related to knuckle pads. It is a benign condition that needs no investigation for an underlying systemic disease. We present a new case of pachydermodactyly and comment on the available literature.     

Localized Mastocytosis of the Vulva

Mastocytosis is a relatively common disorder characterized by mast cell collections in the skin and other organ systems. Affected children are more likely than adults to have limited cutaneous disease. We report two patients with localized vulvar mastocytosis in the absence of other cutaneous findings and review previous reports of vulvar involvement in cutaneous mastocytosis.     

An update on pachydermodactyly and a report of three additional cases

Pachydermodactyly is a benign condition characterized by symmetrical, painless, diffuse swelling of the skin on the lateral aspects of the proximal Interphalangeal joints of the lingers. Histopathological examination shows epidermal hyperplasia, an increase of dermal collagen bundles and a slightly increased number of fibroblasts. We report three adolescent males with pachydermodactyly. We also review 20 published cases, and discuss the differential diagnosis, clearly distinguishing pachydermodactyly from ‘true’ and ‘false’ knuckle pads.     

Pachydermodactyly in Two Young Girls

Abstract: Pachydermodactyly is a benign, superficial fibromatosis frequently observed in young males. We describe the condition in two young girls affected by Ehlers-Danlos syndrome (EDS) and tuberous sclerosis, respectively. We hypothesized that in the patient with tuberous sclerosis, pachydermodactyly is a clinical manifestation of the associated systemic disease, whereas obsessive-compulsive behavior is probably the main cause in the girl with EDS.     

Exposure, skin protection and occupational skin diseases in the glass-fibre-reinforced plastics industry

A total of 100 workers, 86 from the glass-fibre-reinforced plastics (GRP) industry, 11 from polystyrene production and 3 from polyester resin coating manufacture, were examined for occupational skin hazards and for evaluation of skin protection. The workers had been exposed to many chemicals. Those working in the GRP industry had also been exposed to glass fibre and to dust produced by finishing work. 94% used protective gloves. 22 workers, all employed in the GRP industry, had contracted occupational skin disorders. 6 had allergic and 12 irritant contact dermatitis. 4 workers had an accidental injury caused by a peroxide catalyst, fire, hot air and Constant mechanical friction. Allergic dermatoses were due to natural rubber (latex) (4 cases) in protective gloves, phenol-formaldehyde resin (1 case) and cobalt naphthenate (1 case). Irritant hand dermatoses (5 cases) were caused by the combined hazardous effect of unsaturated polyester or vinyl ester resins, organic solvents, glass fibre and dust from finishing work on the skin. Other cases of irritant dermatoses (7 cases) were due to the dust, promoted by mechanical Friction of clothes. Skin disorders in the GRP industry were common (26%) but the symptoms were mild and only 3 patients had been on sick leave because of occupational skin disease.     

Sea urchin spines in the stratum corneum: an early finding related to trauma

Penetrating trauma from sea urchin (Echinoidea) spines has been shown to cause numerous cutaneous reactions, ranging from initial pain that rapidly dissipates and resolves to chronic inflammation and formation of characteristic sea urchin granulomas. Many of these skin‐colored or violaceous papules and nodules form weeks to months after injury, and may be surgically excised. Histopathologic examination commonly shows well‐defined granulomas, the majority of which represent sarcoidal‐type granulomas. Other microscopic patterns, such as foreign body reactions and chronic inflammation, have also been shown. Retained spine fragments are birefringent on polarized microscopic examination and are most likely found in the dermal layer. Herein, we describe a case of traumatic sea urchin cutaneous injury with a unique early cutaneous trauma reaction in a young male who lived in Hawaii. Histopathologic exam was significant for retained spines in the layer of the stratum corneum, but no signs of granulomatous inflammation were observed. This case report emphasizes the unique features of our case and reviews the common clinical and histopathologic features of sea urchin cutaneous reactions.     

汶川地震灾区伤员伤口和创面处理

目的研究地震灾区软组织损伤伤口和烧伤创面特点和处理方法。方法对汶川地震中受伤的124例软组织损伤和烧伤进行回顾性分析和总结。根据受伤情况,采用清创、缝合、换药、皮片移植和皮瓣移植等方法处理伤口和创面。结果地震伤员软组织损伤特点有：伤口污染严重,损伤严重,常伴有感染,严重损伤或感染可导致软组织缺损并需要行皮片或皮瓣修复。120例伤员伤口和创面正常愈合,4例皮肤缺损大者根据统一安排转运至外省医院治疗。结论经过专科医生治疗,地震灾区伤员的软组织损伤伤口和烧伤创面可得到满意的结果。     

Disseminated skin infection due to Mycobacterium fortuitum in an immunocompetent patient

Skin infections caused by atypical rapid-growing mycobacteria, which used considered to be unusual, have become more frequent, especially in immunodepressed patients. Clinical cutaneous disease with these pathogens seems to follow two patterns: in the immunocompetent host, a traumatic injury is followed by the development of localized abscess formation; but in the immunocompromised individual there is no history of trauma and the patient presents with multiple subcutaneous nodular lesions. We describe a rare case of an immunocompetent young woman with disseminated skin infection due to Mycobacterium fortuitum. We emphasize the diagnostic and therapeutic problems associated with such infections.     

Reevaluation of the non-lesional dry skin in atopic dermatitis by acute barrier disruption: an abnormal permeability barrier homeostasis with defective processing to generate ceramide

Atopic dermatitis is characterized by disruption of the cutaneous barrier due to reduced ceramide levels even in non-lesional dry skin. Following further acute barrier disruption by repeated tape strippings, we re-characterized the non-lesional dry skin of subjects with atopic dermatitis, which shows significantly reduced levels of barrier function and ceramide but not of beta-glucocerebrosidase activity. For the first time, we report an abnormal trans-epidermal water loss homeostasis in which delayed recovery kinetics of trans-epidermal water loss occurred on the first day during the 4 days after acute barrier disruption compared with healthy control skin. Interestingly, whereas the higher ceramide level in the stratum corneum of healthy control skin was further significantly up-regulated at 4 days post-tape stripping, the lower ceramide level in the stratum corneum of subjects with atopic dermatitis was not significantly changed. In a parallel study, whereas beta-glucocerebrosidase activity at 4 days post-tape stripping was significantly up-regulated in healthy control skin compared with before tape stripping, the level of that activity remained substantially unchanged in atopic dermatitis. These findings indicate that subjects with atopic dermatitis have a defect in sphingolipid-metabolic processing that generates ceramide in the interface between the stratum corneum and the epidermis. The results also support the notion that the continued disruption of barrier function in atopic dermatitis non-lesional skin is associated with the impaired homeostasis of a ceramide-generating process, which underscores an atopy-specific inflammation-triggered ceramide deficiency that is distinct from other types of dermatitis.     

面部皮肤型孢子丝菌病72例临床分析

目的 报道面部皮肤型孢子丝菌病72例。方法 详细观察72例患者的皮损表现,逐一做真菌学检查,部分做病理检查。采用碘化钾、伊曲康唑、特比萘芬等联合疗法治疗。结果 颜面孢子丝菌病的皮损形态复杂,其中淋巴管型占9.72%,局限型占90.28%,有明确外伤史的26.4%。以碘化钾为基础的联合治疗,6~8周全部治愈。结论 皮损表现和真菌学检查是诊断孢子丝菌病的重要依据。颜面孢子丝菌病形态分布以局限型为主。基层误诊率高达16%。碘化钾为首选基础用药。     

Extensive skin necrosis of the arm in a patient with complex regional pain syndrome

We report a 36-year-old woman with complex regional pain syndrome (CRPS) type 1 presenting with extensive skin necrosis of the left arm. The patient cooled her arm with ice packs to ease severe pain due to CRPS, in spite of repeated cautions against frostbite injury. The regions of skin necrosis corresponded with the sites where she had applied ice packs. We considered that the severe skin necrosis in our case was due to a self-induced frostbite injury.     

Occupational irritant contact dermatitis and fungal infection in construction workers

We have examined 6 construction workers who developed chronic skin diseases on their hands over a period of 15 years (1970–1985). 4 developed a Trichophyton rubrum infection, and the other 2 an irritant contact dermatitis. All of them carried out jobs which caused traumatization of the skin, due to the presence of ethylene glycol and mineral oils during operation of pneumatic hammers in winter. They also suffered other types of skin trauma during their work. Construction workers may be at risk of developing an occupational skin disease involving fungal infection.