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Angioblastoma (Nakagawa)--is it the same as tufted angioma?

Four children affected by intradermal vascular proliferations are presented. The clinicopathological findings closely resemble those of cases described as angioblastoma, a familiar entity in the Japanese literature. Some people suggest that angioblastoma is the same as the tufted angioma described by Wilson-Jones. We found localized hyperhidrosis in two of our patients. Proliferation of eccrine glands near the tumour lobule was a consistent finding in the biopsy specimens from these four patients. In one patient, some of the vascular tufts were replaced by mucinous material. These findings have not been mentioned in previous reports of tufted angioma.

Child↗

Pachyonychia congenita with late onset of nail dystrophy--a new clinical entity?

Pachyonychia congenita syndrome (PCS) is a genetic disease with an autosomal dominant mode of transmission in which the main sign, pachyonychia, usually arises at birth or in childhood together with other disorders of keratinization. A 28-year-old woman developed subungual hyperkeratosis of all toe-nails and thumb-nails associated with pain on pressure and walking. She had a scrotal tongue with leucokeratotic areas, blister formation, plantar hyperkeratosis, palmoplantar hyperhidrosis and dental cavities since childhood. The present case, interpreted as PCS of late onset, could be a clinical variant of the Jadassohn-Lewandowsky syndrome with the late onset of pachyonychia or else an additional form of PCS due to the expression of a new and different allele.

Adult↗

Clinical and biochemical effects of incremental doses of the long-acting somatostatin analogue SMS 201-995 in ten acromegalic patients.

Ten patients (seven women, three men) with active acromegaly, five previously treated and five newly diagnosed, were included in an open-label prospective trial of 3 daily subcutaneous injections of the long-acting somatostatin analogue SMS 201-995 (Sandostatin) at increasing doses in order to obtain maximum growth hormone (GH) suppression. Four patients had received surgery, radiotherapy or bromocriptine. SMS 201-995 doses were increased in a stepwise fashion from 100 micrograms every 8 h (three times daily) to 200, 300 and finally 500 micrograms three times daily at monthly intervals if mean serum GH values failed to decrease to undetectable levels in over 75% of the samples. The optimal dose was maintained for up to 28 months. Significant clinical improvement of headache, soft tissue swelling, facial features, hyperhidrosis and paraesthesia occurred in all patients. Mean 12-h GH levels were significantly suppressed in four patients and fell to normal values in four. Suppression of GH levels was not achieved in two patients. Comparison of the mean interindividual GH values shows that the optimal efficacious dose is 100 micrograms t.i.d. in 7/10 patients. Somatomedin-C (SM-C) was also significantly reduced to below 50% of pretreatment levels in nine patients in whom it was measured. The subsequent increments of SMS 201-995 up to 500 micrograms three times daily did not produce further clinically relevant GH or SM-C suppression. Pituitary tumour shrinkage occurred in five patients. Thyroid function remained normal. Impaired glucose tolerance occurred in four patients. Side-effects (diarrhoea, abdominal discomfort) were mild and transient. Asymptomatic gallstones occurred in three patients on 1500 micrograms/day and one patient on 600 micrograms/day after 6-12 months treatment. This dose-finding study shows that 100 micrograms three times daily SMS 201-995 is an effective therapy for most of the acromegalic patients we treated.

Acromegaly↗

Frey's syndrome.

Frey's syndrome is a form of gustatory hyperhidrosis that develops following surgery, trauma, or disease of the parotid gland area. Several theories have been proposed to explain the resulting symptomatology. Treatment often is difficult or ineffective. An unusual case is presented involving a patient with delayed onset of symptoms who responded well to topical application of 20% aluminum chloride in anhydrous ethyl alcohol.

Aluminum Hydroxide↗

Foot dermatitis from the shoes.

BACKGROUND: Foot dermatitis from the shoes is a common disorder. METHODS: Patients with foot complaints seen at the Contact Dermatitis Unit, Department of Dermatology, Gazi University Faculty of Medicine between May 2001 and May 2002 were evaluated. RESULTS: Four patients with foot dermatitis from shoe allergens were found. Their clinical and patch test results of shoe contact dermatitis were reviewed. We also summarized the standard test allergens for shoe dermatitis. All four patients were treated with topical corticosteroid creams and were informed about patch test-positive materials. CONCLUSIONS: Patients with foot dermatitis from shoes should undergo patch testing to exclude other causes of foot dermatosis. Contact dermatitis from shoes can be managed by the recognition and avoidance of the allergens responsible. The control of hyperhidrosis and the use of topical and systemic corticosteroid therapies may also be beneficial.

Administration, Topical↗

Acrokeratoelastoidosis.

A 45-year-old white woman presented with several years' history of firm, shiny papules on the lateral hands with slight extension to the dorsal fingers. The lesions first appeared between the index fingers and thumbs on both hands. They gradually increased in number, coalescing into plaques and affecting the junction between the palmar and dorsal skin. The patient did not have involvement of her feet. She had been diagnosed previously with chronic eczema that had failed to respond to multiple topical medications. In addition, the patient's sister had similar lesions on both hands. The patient denied any symptoms of hyperhidrosis, excessive sun exposure, or trauma. The plaques were asymptomatic, but were cosmetically unappealing to the patient. On physical examination, small, firm, skin-colored, hyperkeratotic papules, coalescing into plaques, were located on the junction between the palmar and dorsal skin on both lateral margins of the thumb and on the radial side of the index finger (Fig. 1). There were no lesions on the feet. A biopsy taken from a papule on the patient's left hand was consistent histologically with acrokeratoelastoidosis. The biopsy showed marked degeneration of collagen in the dermis with solar elastosis and some smudging of the papillary dermal collagen (Fig. 2). She was treated with clobetasone cream to the affected areas on the hands. After 6 weeks of treatment, she reported no significant improvement.

Clobetasol↗

A case of prolonged confusion after temporal lobe psychomotor status.

A 36-year-old man with prolonged confusion developed after psychomotor status was reported. He had no past history of epileptic seizures or psychotic disorders. The status continued for 20 hours, and twilight state and a slight fever lasted for about 10 days. Thereafter gross impairment of memory and disorientation became remarkable, and, in addition, strong psychic and autonomic disturbances developed, such as visual and auditory hallucinations, excessive excitement, disturbance of sleep, polyphagia, polydypsia, polyuria and hyperhidrosis. The CT scan, carotide angiography, CSF examination, and complement fixation tests for viruses were all within normal limits. The EEGs showed a slowing of the background activity, 0.6--0.8 Hz periodic high voltage wave discharges and random spikes in each temporal area. The clinical symptoms and EEG findings gradually improved without remarkable damage.

Adult↗

A case of citrullinemia with abnormal messenger RNA for argininosuccinate synthetase.

A male neonate, thus far healthy and fed with breast milk, developed rapidly increasing apathy on the third day of life. Sucking became poor and hyperhidrosis was present. Hyperammonemia (3,305 micrograms/dl) was noted. He became comatose and died on the fourth day. There was a profound derangement of amino acid concentration in the body fluids, with highly elevated citrulline levels (4.70 mumols/ml in serum and 8.47 mumols/ml in urine). Autopsy showed diffuse pulmonary bleeding, as the only noteworthy pathological finding. The liver contained no detectable argininosuccinate synthetase (ASS) activity. The defect of ASS in the present case was not due to a reduced amount of ASSmRNA, but its structure was found to be abnormal; it was approximately 1.57 kb in length due to a defect of about 0.1 kb near the 3' end of the coding region.

Amino Acid Metabolism, Inborn Errors↗

Other indications of botulinum toxin therapy.

The therapeutic possibilities of botulinum toxin are manifold and certainly not yet fully exhausted. Apart from the classical indications--focal dystonia and focal spasticity--its use in the management of wrinkles has become well known. Moreover, the toxin is now being administered in many medical fields, including many other kinetic disorders like rare dystonias and tremor. The toxin is also used in a great number of autonomic disorders such as focal hyperhidrosis and even rhinitis. Substantial advances have been made in the field of urology after injections into the sphincter and detrusor muscle. Studies for approval are meanwhile under way concerning the treatment of detrusor hyperreflexia. Gastroenterology is another important field for application of botulinum toxin--be it to the esophagus (e.g. achalasia), stomach, gallbladder, or anorectum. Anal fissure is considered a particularly common indication. Controlled studies for many indications are frequently lacking because of the limited incidence of some of these disorders and symptoms.

Anti-Dyskinesia Agents↗

The borderline syndrome in psychosomatic dermatology. Overview and case report.

The borderline syndrome is one of the most severe disturbances of psychosomatic dermatology. Patients with borderline syndrome are situated 'on the border' of psychosis, neurosis and personality disorders. The skin as a borderline organ carries a symbolic role. The clinical picture includes artefactual skin diseases due to self-mutilation by conscious or unconscious cutting, and rubbing, scratching or para-artefactual manipulations of pre-existing dermatoses. Leading symptoms of the borderline syndrome are poor impulse control, emotional instability and poor ego strength with low frustration tolerance and unstable personal relationships. We present the case of a 38-year-old female patient with borderline syndrome suffering from para-artefactual skin diseases of the face and a massive hyperhidrosis of the hands and feet. Within 9 months she was treated in four acute psychiatric hospitals and by 12 psychiatrists and psychotherapists. Early and accurate diagnosis and high-quality, sophisticated long-term therapy are necessary.

Adult↗

Ross syndrome, an entity included within the spectrum of partial disautonomic syndromes.

Ross syndrome is a degenerative peripheral nervous system disorder defined by the following triad: unilateral or bilateral segmental anhidrosis, hyporeflexia of deep tendon reflexes and Adie's tonic pupils. The most disturbing symptom is segmental compensatory hyperhidrosis. It has only occasionally been reported in the dermatological literature. We present a 35-year-old woman with chronic hepatitis C who developed the characteristic triad of Ross syndrome within 1 month. The patient was otherwise healthy except for an aneurysm of the left medium brain artery not responsible for the syndrome.

Adult↗

Botulinum toxin in dermatology - beyond wrinkles and sweat.

Botulinum toxin (BTX) types A and B have been used with success in cosmetic dermatology and hyperhidrosis treatment. The present review focuses on other uses of BTX in dermatology. Discussed in particular are the available data on BTX in inflammatory diseases, proctology, and some other indications. From studies in various types of eczema, it seems that BTX-A not only acts as a potent inhibitor of acetylcholine but also as an inhibitor of substance P and of glutamate as well. By those mechanisms, BTX-A may be antipruritic, which may help explain the benefits of BTX-A in lichen simplex and dyshidrotic hand eczema. In Hailey-Hailey disease, facial eccrine hidrocystomas, salivary fistulas, and intrinsic rhinitis, BTX-A blocks the secretion of sweat/saliva/mucus. BTX-A has important applications in proctology where it has become the most powerful nonsurgical therapy for anal fissures. In proctalgia fugax and after hemorrhoidectomy, BTX-A is analgesic. Current treatment applications of BTX-A and its limitations are reviewed in this paper.

Journal Article↗

Novel cutaneous uses for botulinum toxin type A.

Botulinum toxin type A is a neurotoxin produced by the bacterium Clostridium botulinum which causes a flaccid muscle paralysis. It has been used extensively in the field of dermatology for the treatment of dynamic rhytides and in the treatment of hyperhidrosis. Botulinum toxin has an excellent safety profile and few side effects when used for these purposes. Recently, botulinum toxin has also been used experimentally in a number of other dermatologic conditions with good results. These conditions include: persistent facial flushing, gustatory sweating and epiphora, anal fissures, familial benign pemphigus (Hailey-Hailey disease), dyshidrotic eczema, and following surgical wound closures. While randomized, controlled prospective trials are still needed to further understand the efficacy and safety of botulinum toxin in these conditions, anecdotal and case report data suggest that botulinum toxin is both safe and efficacious in these and many other procedures.

Administration, Cutaneous↗

A comparison between 133Xenon washout technique and Laser Doppler flowmetry in the measurement of local vasoconstrictor effects on the microcirculation in subcutaneous tissue and skin.

Changes in skin blood flow measured by Laser Doppler flowmetry and changes in subcutaneous blood flow measured by 133Xenon washout technique were compared during activation of the local sympathetic mediated veno-arteriolar vasoconstrictor reflex by lowering the area of investigation below heart level. The measurements were performed in tissue with and without sympathetic innervation. In five subjects, who all had been cervically sympathectomized for manual hyperhidrosis, the Laser Doppler and 133Xenon blood flow measurements were performed simultaneously on the sympathetically denervated forearm, and on the calf with preserved sympathetic nerve supply. The Laser Doppler method registered a 23% reduction in skin blood flow during lowering of the extremities independently of the sympathetic nerve supply to the skin. The 133Xenon method recorded a 44% decrease in blood flow in innervated and unchanged blood flow in denervated subcutaneous tissue during lowering of the extremities. Our results indicate that the Laser Doppler method and 133Xenon method are not comparable, and that the Laser Doppler method is not useful in measuring local sympathetic mediated blood flow changes.

Adult↗

EMLA. A new and effective topical anesthetic.

A eutectic mixture of local anesthetics (EMLA) contains 2.5% lidocaine and 2.5% prilocaine in an oil and water emulsion and has been found to give effective, safe analgesia on normal and diseased skin, making it useful for numerous medical and surgical procedures, such as anesthesia for superficial surgery, split-thickness skin grafts, venipuncture, argon laser treatment, epilation, and debridement of infected ulcers. Other indications have included use in postherpetic neuralgia, hyperhidrosis, painful ulcers, and inhibition of itching and burning. To be effective, EMLA should ideally be applied to the desired area for at least 1 hour under an occlusive dressing. The medication has been approved since May 1991 in Canada for use on intact skin and has been available in Europe for many years. This study discusses the background, efficacy, and current and potential uses of EMLA.

Administration, Cutaneous↗

Eccrine angiomatous hamartoma.

BACKGROUND: Eccrine angiomatous hamartoma (EAH) is a rare benign lesion that mimics a capillary ("strawberry") hemangioma (CH) in appearance. EAH slowly grows and becomes symptomatic whereas CH typically regresses over time. OBJECTIVE: To increase awareness of this rare hamartoma and emphasize the need for histopathologic evaluation of similar appearing lesions and thus prompt excision versus observation. METHODS: Literature review and report of an illustrative case. RESULTS: Histopathologic confirmation of EAH in a lesion clinically indistinguishable from CH and successful surgical extirpation. CONCLUSION: EAH is a rare hamartomatous neoplasma clinically similar to CH in appearance. Histopathologic examination of suspected lesions is recommended for delineation as CH generally involutes spontaneously whereas EAH may require surgical excision due to pain and hyperhidrosis.

Capillaries↗

Green foot.

Pseudomonas aeruginosa may infect the skin surface, nails, hair follicles, or deeper tissues. We report a 13-year-old male with an asymptomatic green discoloration of the toenails and sole of the right foot. Pseudomonas aeruginosa was cultured from the shoe, but not from the discolored skin. We suspect that constant wearing of occlusive, rubber-soled, basketball shoes associated with hyperhidrosis allowed colonization of his shoe with pseudomonas. This case is unique in that colonization resulted in a green color of the foot not associated with infection of the skin.

Adolescent↗

Pachyonychia congenita: a clinical study of 12 cases and review of the literature.

Twelve cases of pachyonychia congenita were reviewed. The mode of inheritance was autosomal dominant. The clinical features of these patients included thickened nails, hyperkeratosis of the palms and soles, thinning of hair or alopecia, painful bullae or ulcerations of the palms and soles, leukokeratosis oris, verrucous lesions of the extremities, hyperhidrosis, premature eruption of teeth, paronychial infections, epidermal cysts with milia, and corneal dyskeratosis at times associated with cataracts. Biopsy from the plantar lesions usually revealed marked hyperkeratosis, acanthosis, moderate hypergranulosis, and minimal dermal inflammatory infiltration. Treatment with keratolytic agents and lubricants is indicated to areas of palmar and plantar hyperkeratosis but usually produces only transient benefit. Squamous cell carcinoma developed in one of the patients over the site of chronic plantar ulcerations. Areas of chronic bullous formation or ulceration should be observed for possible skin malignancy.

Adolescent↗