bluuebunny/crossref_metadata_2025_split huggingface dataset:
ORGAN‐SPECIFIC AUTOANTIBODIES IN VITILIGO PATIENTS AND THEIR RELATIVES
Authors: MANDRY ROCÍO C. , ORTÍZ LUIS J. , LUGO‐SOMOUNOS AÍDA , SÁNCHEZ JORGE L. 
Published: August 2010

Abstract Background. Recent data have supported an autoimmune etiology of vitiligo. Genetic factors also seem to play an important role in this disorder. The purpose of this study was to determine the presence and frequency of organ‐specific autoantibodies in vitiligo patients and their relatives. Methods. Twenty patients with vitiligo and two first or second degree relatives of each patient were interviewed and their sera analyzed for the presence of antithyroglobulin (ATg), antithyroid microsomal (ATM), antiparietal cell (APC), and antiadrenal (AAd) autoantibodies. The sera of 20 normal patients were used as control. Results. A significantly increased frequency of ATg, ATM, and APC antibodies was found in vitiligo patients when compared to normal controls. First and second degree relatives had significantly increased frequencies of ATg and ATM. A high incidence of autoimmune/endocrine diseases was found in the patients' relatives, including a 25% prevalence of vitiligo. Conclusions. These findings not only support an autoimmune etiology for vitiligo, but also highlight the strong genetic contribution to this pigmentary disorder.
ALOPECIA AREATA: IMMUNOHISTOCHEMISTRY AND ULTRASTRUCTURE OF INFILTRATE AND IDENTIFICATION OF ADHESION MOLECULE RECEPTORS
Authors: GHERSETICH ILARIA , CAMPANILE GRAZIA , LOTTI TORELLO 
Published: August 2010

Abstract Background. Alopecia areata (AA) is a noncicatricial alopecia with still unknown pathogenesis, but increasing evidence suggests that an immunologic process might be responsible for the disease. Materials and Methods. Nineteen patients with AA were studied with ten of them in the progressive phase of the disease and nine in the stabilized phase. Biopsies of both affected and unaffected skin were taken. For immunohistochemistry, monoclonal antibodies directed against CD3, CD4, CD8, CD10a, CD36, and HLA‐DR antigens, were used, as well as antibodies directed against adhesion molecules icam‐1, ELAM‐1 and LFA‐1. For electron microscopy (EM), specimens were fixed in glutaraldehyde‐sodium cacodylate buffer, post‐fixed in osmium tetroxide, and stained with uranyl acetate. For statistical analysis, sections from involved and uninvolved skin of each patient for each antibody, the sign test, Fisher's F‐test, and the Tukey‐Kramer test were used. Results. There was a rich infiltrate of CD4+ cells and CD1a+ cells, particularly in the perivascular zone of both unaffected and affected skin (here in the perivascular and in the peribulbar zone) in the progressive phase of AA. In the stabilized phase the infiltrate was scant, both in unaffected and affected skin and limited to the peribulbar area. Receptors of adhesion molecules (ICAM‐2, ELAM‐I, LFA‐1) were strongly expressed, mainly at the microvascular level in both unaffected and affected skin in the progressive phase, but were only weakly or not at all expressed in the stabilized phase, again in unaffected and affected skin. Ultrastructural data confirmed the immunohistochemical findings and showed close contacts between infiltrating lymphocytes and Langerhans'‐lineage cells mainly in the progressive phase. Conclusions. Our results suggest that: 1) an immunologic process, apparently carried out by CD4+ lymphocytes and by dendritic CD1a+ and CD36+ cells, may play a key role at least in the early phase of the disease involving primarily microvessels and later on the bulbar area; 2) the expression of adhesion molecule receptors is involved at the beginning of the disease by mediating the adherence of leukocytes to endothelial cells and subsequent trafficking into the dermis.
SILICONE GRANULOMA IN ACRAL SKIN IN A PATIENT WITH SILICONE‐GEL BREAST IMPLANTS AND SYSTEMIC SCLEROSIS
Authors: ANDERSON DAREN R. , SCHWARTZ JOSEPH , COTTRILL CAROLYN M. , McCLAIN STEVE A. , ROSS JEFFREY S. , MAGIDSON JORY G. , KLAINER ALBERT , BISACCIA EMIL 
Published: August 2010

A 68‐year‐old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu‐like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone‐gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured.Physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal.According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X‐ray energy dispersive analysis by scanning electron‐microscopy confirmed the presence of elemental silicon in the small refractile particles.The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow‐up.
Wells' syndrome related to tetanus vaccine
Authors: Moreno Marta , Luelmo Jesus , Monteagudo Manuel , Bella Rosa , Casanovas Arnau 
Published: August 2010

A 69‐year‐old woman was admitted to the Surgery Department of our hospital in November 1993 because of persistent erythematous skin lesions suspected clinically to be infectious cellulitis‐ Two months before admission she had noticed a red itchy pruriginous plaque, neasuring 10 × 5 cm, on her left buttock that had become larger in spite of treatment with oral antimicrobials. One month before entry, two more lesions flared up on her right buttock and left groin that soon became infiltrated. She did not notice any systemic symptoms. Past history of urticaria, atopy, tinea infection, or insect bites was not recalled. Two months before the first skin lesion erupted, the second dose of a tetanus vaccine had been given to the patient.The patient's temperature was 36.5°C on admission. On physical examination the patient appeared well and there were no systemic abnormalities. Examination of the skin revealed a red indurated plaque covering her left buttock and another one measuring 4 × 5 cm on the right buttock. A red nodular lesion, 3 × 2 cm in size, on her left groin was also noticed (Fig. 1).Laboratory investigation revealed a total white count of 9.7 × l03mm3, with 68% neutrophils and 22% lymphocytes. Erythrocyte sedimentation rate was 110/h. Her erythrocyte count was 3.33 × 106mm3. Blood glucose, kidney and liver function tests, and complement were all normal, and a polyclonal hypergammaglobulinemia of 23 g/L was seen on immunoelectrophoresis. Antinuclear antibodies (ANA) and alpha‐1‐antitrypsin antibodies were negative. Parenteral antibiotic therapy with penicillin and clindamycin was started with no improvement. After surgical debridement, no purulent exudate was found.A skin biopsy specimen of an infiltrated plaque was taken, showing an inflammatory infiltrate of lymphocytes and eosinophils, mostly perivascular, in the superficial and deep dermis, but especially in the deep subcutaneous tissue, with histiocytes and clusters of eosinophilic granules on the collagen fibers. Eosinophilic cellulitis (Wells' syndrome) was diagnosed and therapy with 20 mg/day of prednisone was started immediately. The skin lesions improved in a few days and the dose was tapered 3 weeks after starting therapy with oral steroids. She has not experienced any recurrence.
Vitiligo following the resolution of psoriatic plaques during PUVA therapy
Authors: Halcin Cynthis , Hann Seung‐Kyung , Kauh Young C. 
Published: August 2010

Vitiligo Is an acquired idiopathic disorder, involving 1–4% of tiie world population and characterized by depigmented white patches of the skin that lack the dopa‐positive melanocytes.It has been associated with physicai trauma and, systemic and cutaneous diseases. Among the many dermatoses, psoriasis has been reported to be associated with vitiiigo in the same individuals independently, or vitiligo may precede the formation of psoriasis at the same location. Currently, psoralen plus ultraviolet A iight (PUVA) is one of the efficacious treatments of psoriasis and vitiligo with side‐effects of hypopigmentation and vitiligo‐like lesions. We describe a patient with psoriasis vuigaris in whom vitiligo appeared in the same areas and configurations as his psoriatic plaques as they resolved while being treated with topicai PUVA.A 19‐year‐old Caucasian man was referred for treatment of his psoriatic flare. His medical history revealed a vitiiigo patch on his right calf at age five. At age 14, he developed initially psoriatic plaques on his knees and elbows which then gradually spread to the legs, arms, hands, trunk, scalp, and the genitai area. The only therapy used was a mid‐potency topical steroid ointment with some reiief. Recently the condition had deteriorated enough to seek medical attention.Physical examination revealed sharply demarcated erythematous, silvery‐white scaly papules, patches and plaques of various sizes on trunk, extremities, palms, buttocks, and penis. The patient received topical PUVA with 0.1% trimethoxalen cream for 3 months. Uitravioiet A light (UVA) was applied at 0.1 J/cm with an increment of 0.1 J/cm at each session as tolerated. At approximately the tenth session, depigmented lesions were noted around the margins of the regressing psoriatic plaques. This progressed continually until the vitiligous‐like lesions completely replaced the resolved psoriatic piaques in exactiy the same configurations (Fig. 1).Skin biopsies of the depigmented areas revealed parakeratosis, hypogranulosis, acanthosis, and a sparse iymphocytic infiitrate around dilated tortuous capillaries in the upper corium (Fig. 2). Also a Fontana‐Masson stain showed an absence of melanocytes as may be seen in vitiligo (Fig. 3).
A case of systemic nodular panniculitis associated with M1 (Val213) Z phenotype of α1‐protease inhibitor
Authors: Gaillard Marie‐Christine , Bothwell Janet , Dreyer Leonora 
Published: August 2010

A 31‐year‐old woman presented with a 13‐year history of tender, subcutaneous nodules on her arms, abdomen, buttocks, back, and thighs. In the most acute phase the inflammation of the subcutaneous tissue was more diffuse. Subsequently the nodules ulcerated and discharged an oily fluid. The course fluctuated, with periods of intense inflammation at several fatty sites and times of low‐grade inflammation. Systemic symptoms included Raynaud's phenomenon, joint pains involving her hands, elbows, and shoulder, and morning stiffness lasting about 1–2 h. The patient was never completely free of disease in spite of almost continuous treatment, including chloroquine for 3 months which was stopped following visual problems, potassium iodide which caused asphyxic reaction and was stopped, pulse therapy with cyclophosphamide which was associated with some improvement, dapsone for 3 months which had no effect, and prednisone (up to 90 mg/day) for a few weeks which resulted in minimal improvement.The patient had previously been treated for urinary tract infections and had been investigated for primary infertility. At the time of the present examination, there were multiple depressed scars on her arms and buttocks. Some discharging sinuses with adjacent areas of inflammation were present on the buttocks.Re‐examination of biopsies taken 10 years previously and diagnosed as indicative of Weber‐Christian disease showed subcutaneous fat necrosis with dystrophic calcification. An inflammatory infiltrate consisting of polymorphonuclear leukocytes, lymphocytes, macrophages, and foam cells surrounded the necrotic tissue. Some of the lesions formed sinuses lined by granulation tissue extending from the subcutaneous necrotic tissue to the surface of the skin.The full blood count was normal, as were the sedimentation rate, electrolytes, and amylase. The liver function tests demonstrated a raised Gamma‐glutamyl transferase (GGT), but were otherwise normal. The anti‐nuclear factor (ANF) was positive, with a titre for anticentromere antibodies of > 1/640. Anti‐ds‐DNA and rheumatoid factor were negative.The phenotype (Pi) and genotype of α1‐protease inhibitor (α1‐PI) was determined in the patient and her family. This was performed as previously described.1 The phenotype of the patient's mother and all her siblings was found to be (Pi) Ml (Val213)Z, while her father was (Pi) M1(Val213)M1(Val213) (Table 1). Concentrations of α1‐PI were somewhat below the normal range (2–4 g/L) in the patient and other family members with a similar phenotype, but so too was the concentration in the unaffected father. In addition, there was a modest but significant reduction in plasma elastase inhibitory capacity (EIC) in subjects with the Pi M1(Val213)Z phenotype.
Localized cutaneous infection by Scedosporium prolificans (inflatum)
Authors: Gillum Paul S. , Gurswami Arthur , Taira James W. 
Published: August 2010

A 15‐year‐old African‐American man was first seen in July 1992 complaining of a painful growth on the toe of 6 weeks duration. Initial examination revealed a 2‐cm tender verrucous plaque on the dorsal aspect of the right great toe, just distal to the DIP joint. There were focal erosion and underlying granulation tissue. There was no fever or regional lymphadenopathy, and the patient was otherwise healthy and asymptomatic. Initial diagnostic considerations included viral verruca, blastomycosis, Mycobacterium marinum, and (remotely) squamous cell carcinoma. Tissue was submitted for laboratory evaluation, and the lesion was treated empiricaily with liquid nitrogen, Hematoxylin and eosin stained sections showed epidermal acanthosis with underlying granulomatous inflammation. Special stains and cultures for acid‐fast bacteria (AFB) and fungi (on Mycosel agar) were negative. On follow‐up 1 month later, the lesion measured 8 mm; the patient failed to keep further appointments. He returned in July 1993, and examination revealed a well‐circumscribed 3.5 cm ulcerated piaque, extending from the proximal nail I fold to the DIP joint, with minimal surrounding erythema and serosanguinous discharge. Again, the patient was otherwise asymptomatic. Soft tissue radiographs showed no bone or joint involvement. A repeat biopsy showed siminar changes, in addition to extensive dermal necrosis. Cultures for fungus and AFB were again negative. The patient missed and follow‐up appointments until July 1994, At that time, history and examination were essentially unchanged. A third biopsy was submitted for analysis and failed to identify an etiologic agent. Our differential diagnosis included Trichophyton verrucosum, M. marinum, and deep fungal infections.Over the next 2 months, there was no response to empiric trials of minocycline 100 mg po BID, fluconazole 100 mg po qD, or griseofulvin 250 mg po BID. Finally the lesion was excised, and the base curetted and fulgurated. The surgical specimen was submitted for histology and microbiology. Sections stained with hematoxylin and eosin displayed a hyperkeratotic and encrusted proliferation of squamous epithelium without atypia, but with scattered intraepidermal abscesses. The underlying dermis showed extensive fibrovascular proliferation with plasmacytic and lymphocytic Infiltration, similar to the previous biopsies. Special stains did not reveal any visible micro‐organisms. Part of the surgical specimen was plated on inhibitory mold agar (BBL) and brain heart infusion agar (BBL). Four days later colonies appeared in pure culture and were subsequently identified as Scedosporium prolifions (Fig. 1). The lesion has not recurred after 10 months.
Membranous lipodystrophy associated with insulin lipoatrophy
Authors: Kim Kyun Tae , Ahn Sung Ku , Choi Eung Ho , Lee Seung Hun 
Published: August 2010

A 60‐year‐old Korean woman had been diabetic since she was 40 years old. Initially, she was injected with beef insulin; after a few years of good response, her blood sugar became erratic and uncontrollable. Purified pork insulin was added. She had been receiving daily 55 units NPH purified pork insulin in the AM and 45 units of lente purified insulin in the PM. She was advised and trained to inject at different sites, but she did not necessarily do so, and she had injected repeatedly at the same site on the right buttock. Gradually, atrophic patches developed on the right buttock. On physical examination, two coin‐sized, annular, depressed, and atrophic lesions with a hypopigmented surface were seen on the buttock. These were painless and spongy on palpation. She had no history of any vascular or connective tissue disease or evidence of infection or trauma.Laboratory work‐up revealed normal complete blood count and differential count and normal urinalysis, and an SMA 18 screen showed a blood sugar of 200 mg/dL. An incisional biopsy was carried out at the site of the right buttock; histologic sections showed numerous lobules of small lipocytes with proliferation of capillaries, degenerative loss of adipose tissue, and multiple cysts of irregular shape and variable size. The wall of the cysts characteristically showed anuclear, homogeneous, eosinophilic material. There was slight infiltration of mononuclear cells around the blood vessels in the fat lobule (Figs 1–3).
Microcystic adnexal carcinoma
Authors: Sabhikhi Abha K. , Rao Clementina Rama , Kumar Rekha Vijay , Hazarika Diganta 
Published: August 2010

A 55‐year‐old man presented with an ulcer on the right sole present for 8 years. The ulcer measured 6.5 × 3 cm and affected the entire distal sole of the right foot; the margins were everted and an intermittent serosanguineous discharge was present. The general condition of the patient was good, with findings limited to the ulcerated lesion. There was no history of hypertension, diabetes mellitus, or venous stasis. A biopsy taken from the ulcer edge was interpreted as squamous cell carcinoma, Grade I. A transmetatarsal amputation was carried out and the specimen sent for histopathologic examination. Histologically, the epidermis showed ulcerated areas; adjacent areas showed hyperkeratosis and irregular acanthosis. Keratin cysts containing well‐developed lamellar keratin were present in the upper dermis (Fig. 1). Nests and strands of squamous and basaloid cells, having scanty eosinophilic cytoplasm, alternated with the cysts. Areas of ductular differentiation were also noted. The epithelial strands were separated by concentric bands of moderately cellular fibrous tissue in the upper and mid‐dermis. In the deeper areas of the tumor the epithelial nests became progressively smaller in size, diminishing to small clusters of two or three cells, and were surrounded by a sclerotic stroma. Cytologic atypia was minimal and no significant mitotic figures were identified. The neoplasm showed extensive infiltration of subcutaneous fat and striated muscle with frequent perineural involvement in the deeper parts. There was no extension to bone or perichondrium. Immunoperoxidase staining carried out for carcinoembryonic antigen (CEA) showed positivity in the lumina and lining cells of the ducts (Fig. 2).Based upon the classical microscopic appearance, a diagnosis of microcystic adnexal carcinoma was made. The patient has been followed for a period of 3 years with no evidence of tumor recurrence.
Waardenburg syndrome type II in a Taiwanese woman with a family history of pseudoxanthoma elasticum
Authors: Mancini Anthony J. 
Published: August 2010

A 37‐year‐old Taiwanese woman presented to the Dermatology Clinic for a skin exam to rule out pseudoxanthoma elasticum (PXE). The patient stated she had a known family history of PXE, with a 31 ‐year‐old brother with biopsy‐proven cutaneous lesions of the lateral neck and another brother (33 years of age) with recently documented angioid streaks on ophthalmologic examination. The patient stated that two other living brothers were without known cutaneous lesions and had refused ophthalmologic examinations. A fifth brother had died in infancy of unknown causes. There was no other known family history of PXE, and neither of the affected brothers had a known history of cardiovascular disease.The patient's past medical history was significant only for gestational diabetes during pregnancy. She had no history of hypertension, angina, myocardial infarction, cerebrovascular accident, visual difficulty, bleeding abnormality, or intermittent claudication.Physical examination was notable for multiple hypo‐ and depigmented macules and patches of the anterior trunk, face, and dorsal hands. There were no cutaneous findings of PXE. Scalp examination revealed diffuse depigmentation of the hair roots (Fig. 1), and the patient admitted to the regular use of hair dyes since experiencing premature graying at 22 years of age. Ophthalmologic exam revealed heterochromia iridis (Fig. 2). The Windex (see below) was calculated to be 1.65 (within normal limits). Fundoscopic examination was normal. There was no history of hearing impairment.An expanded family history was performed after noting the patient's clinical phenotype suggestive of Waardenburg syndrome, and revealed several relatives with early graying, leukoderma, heterochromia iridis, and congenital deafness (see pedigree. Fig. 3). All of the patient's relatives lived in Taiwan, and therefore examinations were not possible.
Acantholytic dermatosis in chronic renal failure
Authors: Chua Sze Hon , Giam Yoke Chin 
Published: August 2010

A 53‐year‐old Chinese man with chronic renal failure complained of persistent small raised papules behind the ears and on the nape of the neck for the past 6 months. These lesions were slightly pruritic but nontender. He was diagnosed as having chronic renal failure secondary to chronic glomerulonephritis and had been on maintenance hemodialysis for the past 9 years. He had pulmonary tuberculosis 18 months earlier and had completed a full course of antituberculous therapy consisting of 2 months of 800 mg/day ethambutol, and 9 months of 450 mg/day rifampicin and 300 mg/day isoniazid. His present medications included 100 mg/day asprin, 0.25 μg/day calcitriol, daily calcium, and multivitamin supplements which he had been taking for the past 3 years. He worked mainly on a night shift as a cab driver for the past 20 years and gave no history of excessive sun exposure. There was no family history of any acantholytic disorder.Physical examination revealed an anemic patient with pallor and the sallow discoloration of chronic renal failure. Multiple discrete, brownish scaly papules (approximately 20 in number), measuring between 2 and 6 mm in diameter, were clustered behind both ears and on the nape of the neck just below the hairline (Fig. 1). Some of the firm papules had a central depression. The rest of the body was unaffected. There were no signs of significant sun damage present. Laboratory tests showed the following: hemoglobin 7.6 g/dL, white blood cell count 8000/mm3, platelets 234,000/mm3, urea 21.0 mmol/L, Na 135 mmol/L, K4.8 mmol/L, Cl 101 mmol/L, HCO320.0 mmol/L, blood glucose 5.9 mmol/L, creatinine 1176 nmol/L, total calcium 2.39 mmol/L, and inorganic phosphate 2.29 mmol/L.On biopsy, the papular lesions showed suprabasal clefts, focal acantholysis with corps ronds, and grains (Fig. 2). The adjacent dermis showed patchy infiltrates of lymphocytes and mononuclear cells. The histologic findings resembled Darier's disease.Most of the skin lesions were excised and followed by primary skin closure. No recurrence was noted after 6 months of follow‐up.