Accelerated nodulosis in a patient with psoriasis and arthritis during treatment with methotrexate

A 66-year-old woman with longstanding psoriasis involving the skin presented with asymmetrical polyarthritis. Methotrexate (MTX) was given initially intramuscularly and orally. Intramuscular MTX was discontinued, and a few months after she had been taking only oral MTX she developed nodules, first in surgical incisions, and subsequently in her buttocks, thighs, legs, and arms. Reduction of the dose of oral MTX was followed by gradual diminution in size of the nodules and then total disappearance.     

Intradermal tophi in gout: a case-control study

OBJECTIVE: To describe the characteristics of intradermal tophi in patients with gout and search for factors associated with their development. METHODS: This is a case-control study of patients with gout: cases (Group A, n = 21) had intradermal (not subcutaneous) plaques of monosodium urate (MSU) crystals located in sites distant to articular or paraarticular structures, and controls (Group B, n = 42) had gout but no intradermal tophi. Both Group A and Group B were paired by sex, age (+/-5 years), and duration of the disease (+/-3 years). Analysis included serum and urinary uric acid levels at first visit, radiographic stage of gout, the presence of associated diseases, and previous therapy, specifically, chronic glucocorticoid and diuretic usage. RESULTS: Intradermal tophi were located in the legs, forearms, buttocks, thighs, arms, and abdominal wall. Patients in Group A had a greater number of nonintradermal tophi in common sites (11.9+/-12.5 vs. 4.2+/-7.9, mean +/- SD; p = 0.018), decreased glomerular filtration rate (46.74+/-25.11 vs. 70.87+/-30.18 ml/min; p = 0.042), advanced radiographic changes (57.2 vs. 7.1%; p = 0.0001), and longterm glucocorticoid self-medication (76 vs. 36%; p = 0.006). We found no differences in other associated diseases between groups. CONCLUSION: Intradermal tophi were commonly found in the legs and forearms, and less frequently in the buttocks, thighs, and abdominal wall of gouty patients, and were associated with longterm self-prescribed glucocorticoids and chronic renal failure. The occurrence of intradermal tophi in these patients appeared to correlate with advanced disease.     

Fibrous myopathy. A rheumatic complication of drug abuse

A progressive fibrous myopathy may result from chronic intramuscular drug abuse. This complication may mimic other rheumatic disorders and early recognition may prevent disability. The patient described here presented with fixed flexion and extension contracture of hips and knees, respectively, after abusing meperidine and other agents for 3 years. Soft tissues of thighs and buttocks were "wood hard," EMG showed absence of action potentials in affected muscles, and biopsy revealed extensive replacement of muscle with dense, acellular fibrous tissue. Possible mechanisms are discussed.     

Squamous cell carcinoma complicating perianal hidradenitis suppurativa

Hidradenitis suppurativa or Verneuil's disease is usually a chronic, recurrent, suppurative and cicatricial disorder. It most often affects the skin of the axillae, groins and perineum, and less commonly the buttocks and upper thighs. A squamous cell carcinoma located in the hidradenitis-affected area is a rare complication. We present a review of the twenty-six published cases adding one of our own.     

Calciphylaxis in man. A syndrome of tissue necrosis and vascular calcification in 11 patients with chronic renal failure

Eleven patients with chronic renal failure and presumed secondary hyperparathyroidism developed a syndrome of medial calcinosis of the arteries and painful ischemic ulcers of the fingers, legs, or thighs, or any combination of the three. Five patients required maintenance hemodialysis; six had functioning renal homografts. Severe hyperphosphatemia had existed in each; seven showed roentgenographic evidence of subperiosteal resorption. Similarities are evident between the lesions and experimentally produced calciphylaxix. The lesions demonstrated a relentless, progressive course, with serious morbidity and mortality. Hyperplastic or adenomatours parathyroid tissue was removed from ten of 11 patients unergoing surgical procedures; healing followed in seven patients. Treatment with phosphate-binding antacids to lower serum phosphorus levels may prevent this syndrome. Total or subtotal parathyroidectomy should be considered when ischemic skin lesions appear in uremic patients or in renal transplant recipients.     

Total gluteal pouching with pseudoanus caused by burn injury: report of a case

PURPOSE: We report the case of a 69-year-old man who had suffered a third-degree burn injury of the buttocks close to the perianal region at the age of 2 years. Although initial attempts for conservative treatments failed, wound healing was achieved after numerous skin grafts. However, after prolonged healing, the patient developed scar contracture, resulting in total effacement of the gluteal folds and natal crease, and formation of a gluteal pseudoampulla and a pseudoanus. As a result of misinterpretation of the local anatomy, for the next 50 years the patient had to defecate into the gluteal pouch and empty this pseudoampulla by manually compressing the buttocks and irrigating with chamomile tea. METHOD: After a thorough examination revealed the presence of a normal, uninjured anus hidden by the scarred pouch, we excised this burn scar to expose the unburned perianal tissue and covered the resulting defect with a meshed, split-thickness skin graft. RESULT: Anorectal function was preserved, the anatomic contour was restored, and primary wound healing was achieved after one single-stage operation. CONCLUSION: To prevent such deformities, we recommend early excision and skin grafts as initial treatment, as opposed to conservative treatment, and thorough follow-up examinations to avoid misinterpretation of such a complex, altered local anatomy.     

Infantile pyramidal protrusion as a manifestation of lichen sclerosus et atrophicus

BACKGROUND: A perineal infantile lesion previously described as "skin tag/fold" had recently been named infantile perianal pyramidal protrusion. It appears on the perineal median raphe of girls as a pyramidal soft tissue swelling, covered by smooth, red or rose-colored skin. Its pathogenesis is unknown. As in the case of other perianal lesions, knowledge about it is important, as concern about signs of child abuse grows. OBSERVATIONS: Four girls, 2 of them sisters, with infantile perianal pyramidal protrusion were studied. Three of these girls showed subtle clinical evidence of classic lichen sclerosus et atrophicus on first examination. The other girl developed vulvar lesions of lichen sclerosus et atrophicus months after the diagnosis of infantile perianal pyramidal protrusion. All 4 protrusions disclosed histopathological findings diagnostic of lichen sclerosus et atrophicus. CONCLUSIONS: Infantile perianal pyramidal protrusion is, at least in some patients, a peculiar form of lichen sclerosus et atrophicus that can precede other, more characteristic manifestations. We suggest changing the name to the more precise infantile perineal protrusion. Knowledge of this hitherto unrecognized clinical form of lichen sclerosus et atrophicus can help to explain anogenital symptoms and avoid its misinterpretation as a sign of sexual abuse.     

Cholestyramine ointment to treat buttocks rash and anal excoriation in an infant

OBJECTIVE: To describe a novel treatment for perianal excoriation in an infant receiving a promotility agent. CASE SUMMARY: A 2-month-old boy with reflux, and regurgitation was treated with cisapride. Shortly after cisapride therapy he developed a rash on his buttocks and anal irritation that progressed in severity despite the use of numerous topical products and extended diaper-free periods. A topical cholestyramine ointment compound was prepared and administered, resulting in complete resolution within 3 days. DISCUSSION: Cisapride can decrease the gastrointestinal transit time, which can lead to less time for bile acid reabsorption in the distal ileum. If high concentrations of bile acids are contained in the stool, they can irritate the anus and buttocks in a manner similar to the skin irritation experienced by patients with ostomies. Cholestyramine, a bile acid sequestrant, can irreversibly bind the bile when applied topically and bring relief to the patient. CONCLUSIONS: Topical cholestyramine ointment may be a safe and efficacious treatment option for perianal irritation due to bile acids.     

Thyrotropin-secreting adenomas of the hypophysis

A 28-year-old married female developed histoid papules and nodules de novo over her face, extremities, back, buttocks and thighs. She had developed erythema nodosum leprosum lesions without any antileprosy treatment. Histopathology from a histoid nodule showed well defined nodular collections of plump, spindle-shaped histiocytes. A few globi were also seen with Ziehl Neelson staining. Leucocytoclastic vasculitis was present in the ENL lesion. The CD4:CD8 ratio was 1.5:1.     

Ethical guidelines for studies using experimental animals

BACKGROUND: Adult T-cell leukemia-lymphoma (ATL) can occur in siblings infected with HTLV-1. CASE REPORTS: Two Caribbean siblings developed ATL a few years apart. One case has been reported previously. Both individuals had peripheral lymph node T-cell lymphoma and a few atypical lymphocytes on blood smear. Lymphocytosis, bone marrow biopsy, abdominal computed tomographic scanning, and chest radiography were normal. Clonal rearrangement of T-cell receptor was present in skin lesions for both patients and in the blood for one. HTLV-1 serology was positive. Clonal integration of HTLV-1 provirus was demonstrated in skin lesions in one patient and in blood lymphocytes in the other. Chemotherapy, then interferon alpha, were unsuccessful in the first patient. Topical metchloretamine was partially effective for the second patient. DISCUSSION: ATL in siblings is explained by mother-to-child transmission of HTLV-1 infection during breastfeeding.     

Facultative skin pigmentation in caucasians: an objective biological indicator of lifetime exposure to ultraviolet radiation?

To investigate age and gender trends in facultative and constitutive skin pigmentation we measured skin pigmentation non-invasively and objectively by skin reflectance spectroscopy in 653 caucasians (336 females and 317 males; mean age 38 years, range 0-85) who were not using artificial tanning devices. In all subjects, measurements were performed in the late winter and pre-spring period at five sites exposed to ultraviolet (UV) radiation: the forehead, the upper chest, the upper back and the lateral and medial aspects of the upper arm, and in UV-unexposed buttock skin. Constitutive pigmentation at the buttocks was highest in the first years of life and then decreased substantially during the first two decades of life (P < 0.01). After the age of 25 years, buttock pigmentation remained at a constant level (P = 0.20). There was no gender difference in constitutive pigmentation. Facultative skin pigmentation increased with age for all the measured sites with the highest levels found at the lateral aspect of the upper arm. Based on observations in this study we propose the idea of a 'sun exposure index' (SEI) for individuals, based on objective measurements of skin pigmentation. The SEI is calculated as the increase in facultative pigmentation above the constitutive level and is expressed as a percentage of the constitutive level. The SEI appeared to be related to cumulative lifetime UV exposure and may be used in epidemiological research as an objective estimate of UV exposure at different body sites in caucasians.     

Hereditary neurocutaneous angiomatosis. Report of four cases

The authors report the coexistence of vascular nevi (hemangiomas and arteriovenous malformations (AVMs) of the skin) with AVMs and venous malformations of the brain in male siblings from two related but nonconsanguineous families of three generations. The proband, his siblings, parents, aunts, uncles, and cousins were examined, underwent magnetic resonance (MR) imaging and MR angiography, and when appropriate, cerebral angiography. A father had vascular nevi and a mother, his sister, had an azygos anterior cerebral artery. No other cutaneous or cerebrovascular malformations were present in the parents. Each of the two families had two boys and one girl, 9 to 18 years of age. All the children had vascular nevi and all of the boys had coexisting cerebrovascular malformations: AVMs in three, and a venous malformation in another. One boy had three cerebral AVMs. Two boys had a cerebral hemorrhage, and one also had focal motor seizures. The skin lesions were not those of the Sturge-Weber-Dimitri, Rendu-Osler-Weber, or Wybum-Mason syndromes. The association of cutaneous and cerebrovascular malformations was seen only in males in these families. but females have also been reported in the literature. The results obtained in these families and three other families reported from Western and Central Europe indicate that the association of cerebral and cutaneous vascular hamartomas constitutes a distinct, hereditary clinicopathological entity with autosomal dominant inheritance and variable penetrance. The clinical manifestations of this syndrome are visible, painful vascular nevi, epilepsy, cerebral hemorrhage, and focal neurological deficits. The preponderance of male patients with the full expression of the syndrome suggests a possible hormonal influence on the expression of the gene.     

Infants' use of object parts in early categorization.

Two experiments involving object-manipulation tasks were performed to examine whether 1- to 2 year-olds form superordinate-like categories by attending to object parts. In Study 1, 14-, 18-, and 22-month-olds were tested with contrasts of animals, furniture, insects, and vehicles. Fourteen- and 18-month-olds behaved systematically toward categories with different parts (legs or wheels) but not toward categories with matching parts (legs or legs). In Study 2, infants were tested with novel animals and vehicles generated by removing or attaching legs or wheels. In the absence of part differences, all three age groups failed to form superordinate categories. The two younger groups chose to categorize by parts (i.e., legs or wheels) rather than by category membership (animal or vehicle). The results suggest a perceptual basis for categorization whereby infants form dynamic categories, on-line, that are based on the characteristics of the input. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

A pediatric case of atypical Mycobacterium avium infection of the skin

We report a case of cutaneous atypical mycobacteriosis in a 12-year-old healthy girl due to Mycobacterium avium. The cutaneous symptoms were three well-defined subcutaneous nodules on both buttocks and on the posterior surface of the left thigh. One had a fistulous opening on the skin surface. Histopathological examination revealed epithelioid cell granulomas surrounded by dense lymphocytic infiltration and acid-fast bacteria were seen with modified periodic acid-carbol fuchsin staining. Using Ogawa's medium at 37 degrees C, acid-fast bacteria were isolated from the biopsied specimen and identified by the DNA-DNA hybridization method as Mycobacterium avium. In drug susceptibility test, these were resistant to all antituberculous drugs. Oral administration of minocycline 100 mg/day for two months had little effect on the two remaining lesions, which were therefore excised. Based upon reported cases of Mycobacterium avium complex, we considered that our pediatric patient with multiple intradermal or subcutaneous nodules on the buttocks and the thigh exhibited the characteristic symptoms of M. avium infection.     

Ultrastructure of B-cells of Langerhans islets in rats after the administration of L-leucine

Six women aged 31 to 70 years had folate deficiency and neuropsychiatric disorders. The three with acquired folate deficiency were depressed and had permanent muscular and intellectual fatigue, mild symptoms of restless legs, depressed ankle jerks, diminution of vibration sensation in the legs, stocking-type hypoesthesia and long-lasting constipation; D-xylos absorption was abnormal. The bone marrow was megaloblastic in only one patient, and she and one other had atrophy of the jejunal mucosa. The third was a vegan. All three recovered after folic acid therapy. The other three were members of a family with the restless legs syndrome, fatigability and diffuse muscular pain. One also had subacute combined degeneration of the spinal cord and kidney disease but no megaloblastosis; she improved spectacularly after receiving large daily doses of folic acid. The other two also had minor neurologic signs, controlled with 5 to 10 mg of folic acid daily. Unrecognized and treatable folate deficiency (with low serum folic acid values but normal erythrocyte folate values) may be the basis of a well defined syndrome of neurologic, psychiatric and gastroenterologic disorders, and the restless legs syndrome may represent the main clinical expression of acquired and familial (or inborn) folate deficiency in adults.     

Interaction of transport resistances with biochemical reaction in packed-bed solid-state fermentors: effect of temperature gradients

Subdermal superficial liposuction, first presented by the authors at the ISAPS Congress at Zurich in 1989, is performed with thin three-hole Mercedes cannulas (diameter ranges from 1.8 to 2 mm) to treat small and secondary adiposities and to allow better skin retraction. Suction of the subdermal layer of fat reduces the thickness and consistency of the superficial fat and enhances the possibility of skin retraction. In cases where there is a large adiposity of the abdomen, arms, or inner thighs, there is a conspicuous volume of fat whose weight tends to overstretch and to carry the overlying skin downward. In these cases we need to reduce the large fat volume to permit effective skin retraction. Therefore, we apply the principles of traditional liposuction with those of subdermal superficial liposuction to aspirate large amounts of fat from all the adipose layers. We call this technique Massive All Layer Liposuction (MALL). The amount of skin shrinkage after this "defatting" procedure is remarkable and the clinical results are very good. The MALL technique can be applied to other areas as well. In our experience this new liposuction technique has dramatically reduced the indications of abdominoplasties and dermolipectomies of inner thighs and arms.     

Femoral hernia

BACKGROUND: Atopy is reported to play an insignificant role in wheezing during infancy in contrast to later childhood. For this reason skin testing may not be included in a workup of wheezing infants. OBJECTIVE/METHOD: In order to evaluate the degree and evolution of skin sensitization to allergens in infants with asthma, we have retrospectively analyzed the skin test results from 40 referred asthmatic children less than 36 months of age, who had had more than three wheezing episodes and whose symptoms improved on treatment with beta-agonist and anti-inflammatory agents. RESULTS: Skin sensitization (epicutaneous) to common indoor and outdoor aeroallergens and foods were demonstrated in 23 (58%) of these patients. Asthmatic children with the onset before 12 months of age and duration of less than 12 months were sensitive predominantly to foods. Those with later onset asthmas and longer duration developed sensitivity first to indoor and then to outdoor allergens. The most common food, indoor, and outdoor allergens were egg, dust mite, and pollens, respectively. Six of these patients had repeat skin tests later. Two demonstrated a decrease in skin sensitivities to foods, one gained a new sensitivity to grass, while three remained unsensitized. CONCLUSION: Skin sensitization to allergens was common in selected asthmatic infants and evolves in the order of exposures: foods, indoor allergens, and outdoor allergens. Repeat skin tests showed changing skin test patterns in some patients.     

Olmsted syndrome: report of a new case

We report the case of a 20-year-old man, who was born with an intense erythema of the genital area, unresponsive to any treatment employed. When he was 9 months old, he presented with well-defined hyperkeratotic erythematous plaques around the mouth, eyes, nose, and perianal area, with similar plaques on the lateral aspect of the neck and axillae. At the same time the erythema of the genital area became hyperkeratotic. When he was 2 years old, he presented with a disabling palmoplantar keratoderma, initially focal, and later diffuse, also unresponsive to local or systemic treatments employed. The lesions have varied during the course of the disease without ever clearing completely. The axillary and inguinal plaques have shown spontaneous resolution on occasion. Six skin biopsies have been performed with no conclusive histological diagnosis of any of the typical disorders of keratinization. All treatments, topical and systemic, including etretinate and acitretin, have failed to improve the condition. We believe that this patient has Olmsted syndrome, a rare form of palmoplantar keratoderma with periorificial keratotic plaques.