bluuebunny/crossref_metadata_2025 huggingface dataset:
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).
Subungual hyperkeratosis due to sarcoidosis
Authors: Fujii Kimio , Kanno Yuko , Ohgo Noriko
Published: August 2010

A 30‐year‐old Japanese woman presented to our clinic for evaluation and treatment of subungual hyperkeratotic lesions of the toenails. Five years previously the bilateral parotid glands and hilar lymph nodes became enlarged, and tender subcutaneous nodules developed in the lower extremities. Transbronchial lung biopsies showed epithelioid granulomas and systemic sarcoidosis was diagnosed. A skin biopsy showed nonspecific septal panniculitis, a finding compatible with erythema nodosum. Since then the patient had experienced intermittent episodes of low grade fever, general malaise, dyspnea, diarrhea, and superficial lymphadenopathy, and systemic steroid was administered when the disease was aggravated. The initial erythema nodosum subsided in a couple of months and was replaced by a large number of slightly scaly, erythematous papules and nodules in the lower extremities and in the face. The biopsy specimens of these lesions showed noncaseating epithelioid granulomas in the dermls. These cutaneous lesions waxed and waned along with the systemic symptoms. The patient had never had uveitis or lupus pernio. A few months previously new erythematous papules and nodules had developed in the face and in the lower extremities. Hyperkeratotic verrucous lesions also developed at the hyponychium and beneath the distal portion of the nail plates in all the toenails (Fig. 1a). The proximal and lateral nail folds and the nail plates appeared normal. Repeated potassium hydroxide stain and culture of the subungual hyperkeratotic materials were negative for fungus. Roentgenogram of the toe phalanges was normal. Interestingly, none of the fingernails were affected. A biopsy specimen of the nail bed showed orthokeratotic hyperkeratosis and acanthosis of the epidermis, but no papillomatosis or vacuolated keratinocytes (not shown). There were discrete epithelioid granulomas in the superficial dermis (Fig. 2). Systemic steroid, 30 mg/day, was administered because the patient's lung parenchymal lesions were aggravated. The subungual hyperkeratosis rapidly improved after administrating steroid for 2 weeks (Fig. 1b).
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.
Idiopathic unilateral circumscribed hyperhidrosis
Authors: Koöse Osman , Baloglu Huüseyin
Published: August 2010

A 21‐year‐old man presented with a hyperhidrotic area on the extensor aspect of the right forearm and wrist. The lesion was 8 × 15 cm in size and was sharply demarcated. The patient complained of profuse sweating that lasted 10–80 min and that was sometimes precipitated by heat.

We obtained two different punch biopsies from the left and right wrists. The biopsies showed apparently normal histologic findings. Neurologic examination was normal. We then performed a conventional iodinated‐starch test for sweating. Before the test, the right arm was of an orange colour with iodine‐starch powder; dark blue droplets were seen on the hyperhidrotic area after provocation by heat (Figs 1 and 2).

Laboratory findings revealed a normal complete blood cell count and normal results of renal and liver function tests. Aluminium chloride cream (20%) was used as treatment, but the hyperhidrosis was not controlled.
Chronic leg ulcers associated with hereditary protein S deficiency
Authors: Kulthanan Kanokvalai , Krudum Tawat , Pintadit Pakaimas , Khokkaseam Rajapat , Kullavanijaya Preya
Published: August 2010

A 17‐year‐old Thai man developed erythematous papules on the dorsal surfaces of both feet 7 years previously. The papules progressed to superficial ulcers which slowly increased in size and, although treated by several modalities, never completely healed.

Physical examination revealed two irregular‐shaped superficial ulcers, 4 × 1 and 5 × 1 cm in size, on the dorsal surface of his right and his left foot, respectively (Fig. 1). The ulcers had granulation tissue at the bases with well‐defined borders surrounded by brownish patches. During admission, the superficial vein of the patient's right arm showed painful, warm, cord‐like induration.

A biopsy specimen from the edge of the ulcer of the left leg showed ulcerated epidermis with pseudoepitheliomatous hyperplasia. Diffuse mixed cellular infiltration composed of lymphocytes, histiocytes, and neutrophils was seen in the dermis with fibrosis and some capillary proliferation. The results of skin biopsy cultures for fungus, bacteria, and mycobacteria were negative. The biopsy specimen from the superficial vein of the right arm showed obliteration of the vessel lumen and mixed cellular infiltration composed of lymphocytes, histiocytes, and neutrophils within the wall compatible with thrombophlebitis.

Laboratory studies revealed a normal complete blood cell count, SMA 18, prothrombin time, and partial thromboplastin time. The erythrocyte sedimentation rate was 29 and lupus anticoagulant was not present. Anticardiolipin IgG, IgM, ANA, cryoglobulin, and anti‐HIV antibody were all negative. Levels of C3, C4, and CH50 were normal. Bilateral venogram showed deep vein thrombosis of the left leg. The free protein S level was 41% (normal 60%‐140%) (Microlatex particle‐mediated immunoassay), the protein C level was 90% (normal 75%‐150%), and the antithrombin III level was normal.

Heparin treatment (Nadroparine 20,000 units subcutaneous Injection twice a day for 10 days) was followed by coumadin 3 mg/day, 2 days per week (days 1 and 4), and 2 mg/day, 5 days per week (days 2, 3, 5, 6 and 7), to keep the prothrombin time between 1.5 and 2 times normal control. The ulcers completely healed 3 months after treatment.

The studies of the patient's family members were consistent with hereditary protein S deficiency as in the pedigree shown in Fig. 2. The patient's brother (III‐2), mother (11–2), and aunts (11–1, 11–5) all had low levels of protein S (35%, 63%, 51%, and 69% respectively). His uncle (11–3) died with pulmonary embolism in a private hospital. Unfortunately an autopsy was not permitted. We could not obtain blood samples from the patient's father and grandfather.
Evaluation of oral methotrexate in the treatment of systemic sclerosis
Authors: Krishna Sumanth M. , Sharma Vinod K. , Khaitan Binod K. , Kapoor Anu , Tejasvi Trilokraj
Published: August 2010

Background  Treatment of scleroderma is difficult and currently no treatment can induce complete remission of the disease.

Objective  To evaluate weekly oral methotrexate in the treatment of Indian patients with systemic sclerosis.

Methods  Thirty‐three patients with systemic sclerosis presenting to the department of dermatology (outpatients) who satisfied the inclusion criteria were enrolled into the study. All cases were admitted into the dermatology ward for detailed evaluation. A detailed history and physical examination, including assessment of disease severity by Rodnan skin scoring, was carried out. Baseline investigations included complete blood counts, blood glucose, serum electrolytes, renal function test, liver function tests, urine examination (albumin, sugar, microscopic examination, 24‐h protein), ANA, chest X‐ray, Barium swallow, pulmonary function test, electrocardiogram (ECG), HRCT of chest, and 4‐mm punch skin biopsy from dorsum of the hand. All the patients were treated with oral methotrexate (15 mg/week) for 6 months, following standard guidelines.

Results  The patients included 29 (87.9%) females and four (12.1%) males with a mean age of 31.45 ± 8.76 years. The mean duration of disease was 5.6 ± 4.5 years (range 2 months to 15 years). All the patients had binding down of skin, 31 (93.9%) had Raynaud's phenomenon, 31 (93.9%) had pigmentary change, 21 (63.6%) had hand contractures, 17 (51.5%) had fingertip ulcers, 15 (45.5%) had dyspnoea, 14 (42.4%) had restricted mouth opening, 13 (39.4%) had telangiectasia, 11 (33.3%) had fingertip resorption, eight (24.2%) had joint complaints, six (18.2%) had dysphagia, and one (3.03%) had gangrene. On laboratory investigation ANA was positive in 29 (87.9%) patients, dsDNA was raised in only four (12.1%), baseline chest X‐ray was abnormal in 18 (54.5%), HRCT was abnormal in 27 (81.8%), abnormal PFT in 32 (96.9%), abnormal ECG in five (15.2%), and barium swallow abnormality in 19 (57.5%) patients.

Twenty‐five patients completed the 6‐month follow up. There was subjective improvement in binding down (80%), Raynaud's phenomenon (96%), fingertip ulceration (88.8%), hyperpigmentation (77.2%) and dyspnoea (45.5%). The objective parameters showed statistically significant improvement in mouth openingm, but improvement of skin score, lung function (chest radiograph, PFT, HRCT), and dysphagia was not significant at the 6‐month follow up. In eight patients, treatment was continued for 1 year of methotrexate, which showed statistically significant improvement in skin score.

Conclusion  It was concluded that methotrexate for 6 months only provides subjective improvement, and further studies after 1 year of treatment with methotrexate are recommended.
Lobomycosis in Venezuela
Authors: Paniz‐Mondolfi Alberto E. , Reyes Jaimes Oscar , Dávila Jones Luisana
Published: August 2010

Lobomycosis is a chronic dermal infection that presents a wide spectrum of clinical‐ dermatological manifestations, mainly characterized by the development of keloid lesions as well as nodular, verrucoid and sometimes ulcerous forms. The etiological agent at an international level, according to the consensus nomenclature, has been called Loboa loboi, even though recently it has been accommodated as Lacazia loboi. The present review extensively covers the clinical‐epidemiological aspects as well as the most outstanding historical aspects, including the Venezuelan experience and the presentation of two new cases, which substantiate the Amazon basin as an endemic area for the disease.
Cervical cancer metastasis to the scalp presenting as alopecia neoplastica
Authors: Chung John J. , Namiki Thomas , Johnson Douglas W.
Published: August 2010

A 45‐year‐old Japanese woman was diagnosed in 1996 with squamous cell cancer of the cervix following an abnormal Papanicolaou smear and subsequent diagnostic conization. She underwent total abdominal hysterectomy with pelvic lymphadenectomy and was found to have poorly differentiated squamous cell carcinoma, International Federation of Gynecology and Obstetrics (FIGO) stage IB1. She remained asymptomatic until 2003 when she presented with obstructive uropathy with acute renal failure and hydronephrosis, suspected to be from the recurrence of cervical cancer. This was confirmed when computerized tomography (CT)‐guided lymph node biopsy showed squamous cell carcinoma of the para‐aortic lymph nodes histologically consistent with the cervical primary. In addition, there was evidence of lumbar spine metastasis by positron emission tomography (PET) and bone scans. She received several courses of chemotherapy with cisplatin and 5‐fluorouracil (5FU), as well as radiation therapy.

In July 2004, she was hospitalized for acute renal failure, nausea, vomiting, and anorexia. CT of the abdomen identified widespread metastases in the liver, pancreatic head, and lumbar spine. During this hospitalization, she complained of severe scalp tenderness and patchy hair loss first noticed 3 days prior to presentation. On examination of her scalp, two patches of alopecia were observed (Fig. 1). In the largest patch, there was a 5 × 2.5‐cm, tender, erythematous plaque with atrophy. In the smaller patch, there was a 2 × 1.5‐cm, erythematous, scaly plaque. A punch biopsy of the larger patch revealed atypical epithelial cells in nests with intravascular involvement and diminished numbers of focally miniaturized hair follicles (Fig. 2a). The scalp specimen was histologically identical with biopsy specimens of the cervical primary (Fig. 2b). There was also seborrheic dermatitis, with thick greasy scale, noted on the scalp, which resolved with fluocinolone solution. The patient decided against further treatment for her advanced cervical cancer but did accept hydromorphone for pain. She died 3 months after admission.

Erythematous patches of alopecia

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(a) Biopsy of scalp metastasis. Note nests of atypical epithelial cells and diminished number of focally miniaturized hair follicles. (b) Biopsy of cervical specimen (hematoxylin and eosin stain, ×100)

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Alopecia areata
Authors: Wasserman Dan , Guzman‐Sanchez Daniela Araucaria , Scott Kimberly , McMichael Amy
Published: August 2010

Alopecia areata (AA) is a nonscarring, autoimmune, inflammatory, hair loss on the scalp, and/or body. Etiology and pathogenesis are still unknown. The most common site affected is the scalp. Histopathology is characterized by an increased number of the catagen and telogen follicles, the presence of inflammatory lymphocytic infiltrate in the peribulbar region (“swarm of bees”).

Corticosteroids are the most popular drugs for the treatment of this disease. Etiologic and pathogenic mechanisms, as well as other current treatments available will be discussed in this article.
Measurement of pruritus in a Chinese woman with pemphigoid gestationis using a wrist movement detector
Authors: Hon Kam‐lun Ellis , Chiu Lai‐shan Mona , Lam Man‐chin Adrian , Choi Cheung‐lung Paul , Chan Shirley , Luk Nai‐ming
Published: August 2010

Pemphigoid gestationis (PG) is a rare itching bullous disease of pregnancy and the postpartum period. We describe the use of a new methodology for measuring the nature and intensity of itching in a 21‐year‐old woman with pemphigoid gestationis. At 19 weeks gestation, she developed an itchy rash over the limbs, which spread to the trunk. Blisters then appeared on the hands which subsequently also involved the feet. Intense nocturnal wrist activities in this patient, measured with a wrist monitor and defined as average acceleration in the early hours of sleep, were even higher than that in patients with severe eczema, with an average value of 181.00 ± 43.49 (mean ± standard error) g/min for the first three hours, versus 84.47 ± 8.53 g/min for the group of 24 eczema subjects. Most wrist activities were slower movements at 0 to 1 Hz. This is in striking contrast to the scratching activities at 0 to 3 Hz in eczema subjects. There have been no therapeutic trials for PG. Topical steroid and oral antihistamines are usually ineffective, but worked in our patient. We also discuss the potential application of the monitor in assessing the nature of various dermatological or systemic itching disorders.
Lack of association between the G‐2548A polymorphism of the leptin gene and psoriasis in a Turkish population
Authors: Kara Nurten , Aydin Fatma , Senturk Nilgün , Gunes Sezgin , Canturk M. Tayyar , Bagci Hasan , Bek Yüksel , Turanli Ahmet Yasar
Published: August 2010

Background   Psoriasis is a multifactorial disease in which genetic and inflammatory factors play important roles. Leptin is classified as a cytokine and plays an important role in the regulation of the T‐helper response. A common polymorphism in the promoter of the human leptin gene (G‐2548A) may have a role in the pathogenesis of psoriasis.

Aim  To investigate the association between psoriasis and leptin gene polymorphism (G‐2548A).

Methods  The study involved 109 patients with psoriasis and 125 healthy controls. Analyses of G‐2548A polymorphism of the leptin gene were made by the polymerase chain reaction‐restriction fragment length polymorphism technique. The genotypes (GG, GA, and AA of leptin gene G‐2548A) and alleles (G and A) were scored and the frequencies were estimated. The frequencies of alleles and genotypes in patients and controls were compared. The relationship between leptin gene polymorphism and the clinical features of the patients was analyzed.

Results  Both genotype [odds ratio (OR), 0.921; 95% confidence interval (CI), 0.501–1.694; P = 0.792] and allele (OR, 0.864; 95% CI, 0.600–1.242; P = 0.429) frequencies were not significantly different between patient and control groups. In addition, there was no significant association between genotype and allele frequencies and the clinical characteristics of psoriasis.

Conclusion  In this case–control study, no evidence of association between the G‐2548A variant of the leptin gene and psoriasis was found.
Systemic exposure to lidocaine and tetracaine is low after an application of a lidocaine 7%–tetracaine 7% peel in adults
Authors: Ogden Lazarre , Love Georgette , Basta Susan
Published: August 2010

Background  The lidocaine 7%–tetracaine 7% (LT) peel has been demonstrated to be useful for a variety of dermatologic applications, including cosmetic, laser, and vascular access procedures.

Aim  To measure and evaluate the detectable plasma levels and safety of lidocaine and tetracaine in adult volunteers after a single application of an LT peel, a novel method of topical anesthesia that does not require occlusion.

Methods  A randomized, factorial design study was used to evaluate the pharmacokinetic profile of lidocaine and tetracaine after a single application of the LT peel in adult volunteers (n = 36; mean age, 26.6 years). The LT peel was applied to the anterior surface of the left or right thigh of volunteers for 30, 60, or 90 min over a 50‐, 100‐, or 200‐cm2 area. Venous blood samples were collected at 0, 30, 60, 90, 120, 150, 180, 210, 300, and 420 min after the initial application of the LT peel.

Results  At all time points, plasma concentrations of lidocaine and tetracaine were below the limits of quantification for the assay: 100 ng/mL and 5 ng/mL, respectively. Because of the lack of concentrations above the limit of quantification, it was not possible to determine the pharmacokinetic parameters, other than the maximum concentrations of < 100 ng/mL for lidocaine and < 5 ng/mL for tetracaine. A single application of the LT peel was well tolerated, and no study subject reported an adverse event.

Conclusions  A single application of LT peel to up to 200 cm2 of anterior thigh in adults with a duration of up to 90 min produces systemic levels of lidocaine and tetracaine that are of no clinical significance at all time points measured up to 420 min after the initial application.
Verrucous tuberculid mimicking Kyrle disease
Authors: Khandpur Sujay , Bansal Arika , Ramam M. , Sharma Vinod K. , Das Anup Kumar , Singh Manoj K. , Prasad H. Krishna
Published: August 2010

Cutaneous tuberculosis shows wide clinical variation depending upon the virulence of the organism and host immunity. Tuberculids are difficult to diagnose since the organism often cannot be identified or isolated by microscopy, culture or polymerase chain reaction. The diagnosis rests primarily on the identification of a tuberculous focus elsewhere in the body and response to antitubercular therapy. We present the clinicopathological features of an unusual tuberculid in a 30‐year‐old man, remarkably mimicking Kyrle's disease. He presented with multiple, symmetrically distributed follicular and perifollicular verrucous papules, nodules and plaques on the face, earlobes and extremities since 4 years. Histopathology revealed parakeratotic follicular plug invaginating into the dermis with multiple caseating epithelioid cell granulomas. PCR for M. tuberculosis was negative. The identification of associated axillary tubercular lymphadenitis, strongly positive Mantoux reaction, tuberculoid granulomas on histopathology and complete resolution of the lesions with antitubercular therapy helped in making the diagnosis. We propose the term “verrucous tuberculid” for this entity.
Direct immunofluorescence study in patients with lichen planus
Authors: Kulthanan Kanokvalai , Jiamton Sukhum , Varothai Supenya , Pinkaew Sumruay , Sutthipinittharm Puan
Published: August 2010

Background  Direct immunofluorescence (DIF) studies in patients with lichen planus (LP) show the deposition of multiple immunoglobulins (Igs) at the cytoid bodies (CBs) and fibrin at the dermoepidermal junction (DEJ). The deposition of Ig at the DEJ, as in patients with lupus erythematosus (LE), is occasionally found. For cases with no specific clinical and histologic characteristics, or with ambiguous features, DIF studies may be helpful in disease differentiation.

Methods  From 1996 to 2004, data from 72 patients with LP, diagnosed on the basis of clinical and histologic criteria at the Department of Dermatology, Siriraj Hospital, Bangkok, Thailand, were collected. The results of DIF studies were analyzed.

Results Deposits at the DEJ and CBs were detected in 53% and 60% of cases, respectively. A combination of DEJ (mostly fibrin) and CB (mostly IgM) deposits was found in 38% of cases. A combination of IgM and other immunoreactant deposits, including fibrin at the CBs, was found in 56% of cases.

Conclusions  This study showed that the positive yield of DIF in LP was 75%. Shaggy fibrin deposition at the DEJ, which is the single best indicator in the diagnosis of LP, was found in 56% of cases. The presence of CBs only, which is a poorer indicator than the shaggy deposition of fibrin along the DEJ, was found in 22% of cases. There were no statistically significant differences in positive DIF yield between specimens derived from glabrous skin and oral lesions (P = 0.67). Forty‐four per cent of cases had immunoreactants other than fibrin deposited along the DEJ, which resembled those of LE.
Tanning bed exposure increases the risk of malignant melanoma
Authors: Ting William , Schultz Kara , Cac Natalie N. , Peterson Michael , Walling Hobart W.
Published: August 2010

Background  Epidemiologic studies have associated tanning bed exposure and cutaneous melanoma. The relationship between the extent of tanning bed exposure and the risk of melanoma has not been elucidated in detail.

Methods  Surveys assessing the extent of tanning bed exposure and the history of skin cancer, including malignant melanoma, were collected from academic dermatology clinic patients (n = 1518). Of these, 551 (36.3%) completed all components of the survey. The available medical records, including pathology reports (n = 501; 33%), were reviewed to confirm cases of skin cancer. Data on potential confounding factors, including indoor vs. outdoor occupation and leisure activities, Fitzpatrick skin type, history of blistering sunburn, use of sunscreen and sun protective clothing, history of phototherapy, and level of education, were assessed and compared.

Results  Of the patients surveyed, 487 (32.1%) reported tanning bed exposure. Women aged 45 years or younger accounted for about 60% of all tanning bed users. Seventy‐nine cases of malignant melanoma were reported, 22 in women aged 45 years or younger. In the entire cohort, the “ever‐use” of tanning beds was found to be a significant risk factor for the development of melanoma [P < 0.05; odds ratio (OR), 1.64; 95% confidence interval (95% CI), 1.01–2.67]. The risk was greater in women aged 45 years or younger (P < 0.05; OR, 3.22; 95% CI, 1.01–11.46). Patients with a history of melanoma were significantly more likely to report tanning bed sessions exceeding 20 min (P < 0.01; OR, 3.18; 95% CI, 1.48–6.82); this association was even stronger for women aged 45 years or younger (OR, 4.12; 95% CI, 1.41–12.02).

Limitations  The study was subject to recall bias, included only patients at a midwestern academic practice, and had a relatively low response rate.

Conclusion  Exposure to tanning beds increases the risk of malignant melanoma, especially in women aged 45 years or younger. These findings reinforce the hazards of tanning bed exposure.
Myxoinflammatory fibroblastic sarcoma – report of a rare case at an unusual site with review of the literature
Authors: Premalata Chennagiri S. , Rama Rao Clementina , Padma Maneya , Vijaykumar Manavalan
Published: August 2010

We report a case of myxoinflammatory fibroblastic sarcoma in a thirteen year old gilrl who presented with a tender swelling in the left upper back. The tumor consisted of varying proportions of inflammatory, myxoid and hyalinized areas. Large bizarre cells with virocyte like inclusions and lipoblast like cells were present. To the best of our knowledge this is the first reported case of myxoinflammatory fibroblastic sarcoma of the back, the extremities being the commonest site of involvement. Due to its varied histologic appearance, the tumor should be differentiated from various benign and malignant soft tissue lesions.
Pigmentatio maculosa eruptiva idiopathica: a case report and review of the literature
Authors: Stinco Giuseppe , Favot Francesca , Scott Cathryn Anne , Patrone Pasquale
Published: August 2010

Background  Pigmentatio maculosa eruptiva idiopathica is a rare pediatric disease characterized by asymptomatic, brownish macules involving the neck and trunk with no preceding inflammatory process or history of drug exposure.

Methods  A 9‐year‐old girl presented with brown–gray, nonconfluent, asymptomatic macules on the trunk, neck, and limbs, ranging from 5 to 30 mm in diameter. The macules appeared suddenly with no lesions preceding their occurrence. Histopathologic examination showed basal cell layer hyperpigmentation, and abundant melanophages with a mild perivascular lymphohistiocytic infiltrate in the papillary dermis.

Results  The lesions disappeared spontaneously 1.5 years later with no therapy. No relapse occurred.

Conclusion  Pigmentatio maculosa eruptiva idiopathica must be differentiated from other skin disorders with hyperpigmentation in pediatric practice in order to avoid unnecessary treatment, as spontaneous resolution is expected. Following a literature review, we underline the importance of spontaneous regression as an additional clinical feature for this disease.
Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature
Authors: Bischoff Lindsay , Derk Chris T.
Published: August 2010

Background  Eosinophilic fasciitis is a rare scleroderma‐like illness. The clinical spectrum of the disease has evolved since its initial description.

Methods  We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded.

Results  Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2–94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment.

Conclusion  High dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.
Anogenital ulcerations in a 83‐year‐old woman
Authors: Aktan Şebnem , Akarsu Sevgi , Koç Pınar , Lebe Banu , Özkan Şebnem
Published: August 2010

What is your diagnosis?
Xanthoma disseminatum in a black African woman
Authors: Yusuf Shehu M. , Mijinyawa Mohammed S. , Musa Baba M. , Mohammed Aminu Z.
Published: August 2010

Purpose  To report a partial steroid response of xanthoma disseminatum in a black African woman.

Design  Case report and literature review.

Methods  Histopathologic study of cutaneous tumour and clinical follow‐up.

Results  A 32‐year‐old black African woman with mucocutaneous xanthomatosis and dysphonia, which partially responded to treatment with steroids.

Conclusions  Xanthoma dissseminatum is a rare condition for which there is no medical treatment. We reported the condition in a black African woman whose skin and CNS symptoms regressed remarkably within 22 weeks of steroid therapy.
Nail changes in pemphigus vulgaris
Authors: Habibi Mohammadali , Mortazavi Hossein , Shadianloo Shervin , Balighi Kamran , Ghodsi S. Zahra , Daneshpazhooh Maryam , Valikhani Mahin , Ghassabian Akhgar , Pooli Aydin H. , Chams‐Davatchi Cheyda
Published: August 2010

Background  The frequency and types of nail changes in pemphigus vulgaris (PV) are unclear.

Aim  To determine the frequency and types of nail changes, and their correlation with the number of skin and periungual bullae.

Methods  Seventy‐nine patients with PV, including 59 new patients and 20 patients in relapse, were entered into the study. Microscopic examination in potassium hydroxide and culture for fungus were performed on all clinically abnormal nails.

Results  Twenty‐five (31.6%) of 79 patients showed nail changes, with paronychia (n = 8) and onychomadesis (n = 6) being the most common. One patient in relapse had onychomycosis. The frequency of nail changes in fingers affected by periungual bullae was significantly higher than in other fingers (P < 0.05). The number of nail changes was higher in patients with a larger number of skin bullae and in those with a longer duration of disease (P < 0.05).

Conclusions  Nail changes in PV are common and related to the number of skin bullae and the presence of periungual bullae.
Effect of γδ T cell supernatant on human skin fibroblast proliferation and collagen production – Possible role of transforming growth factor‐β and basic fibroblast growth factor
Authors: Ohtsuka Tsutomu
Published: August 2010

Background  Perivascular mononuclear cell infiltration, vascular injury and diffuse interstitial fibrosis has been shown in systemic sclerosis (SSc).

Objective  To study a role of cellular immune mechanism inducing fibroblast proliferation and collagen synthesis.

Methods  The interaction of γδ T‐cell receptor bearing lymphocyte supernatant to human fibroblast proliferation and collagen synthesis was studied.

Results  The fibroblasts added with γδ T cell supernatant showed increased proliferation compared with those without it and that added with αβ supernatant. The endothelial cells added with γδ T cell supernatants, however, showed significantly decreased proliferation compared with those without it and that added with αβ T cell supernatant. The fibroblasts added with endothelial cell supernatant showed increased proliferation compared with those without it. A doubling of collagen synthesis was observed when human skin fibroblasts were maintained in all of the supernatant. The collagen synthesis was inhibited by antitransforming growth factor‐β antibody and antibasic fibroblast growth factor antibody.

Conclusion  γδ T–cell‐receptor bearing lymphocyte has proliferative and collagen synthesis activity to human skin fibroblasts, but suppressive to endothelial cells.
Mucocutaneous features of Chikungunya fever: a study from an outbreak in West Bengal, India
Authors: Bandyopadhyay Debabrata , Ghosh Sudip Kumar
Published: August 2010

Background  Chikungunya fever (CF) epidemic has recently re‐emerged in India on a large scale affecting many parts of the country. We have reviewed the mucocutaneous manifestations of the disease in a number of cases.

Methods  Twenty six serologically proven cases of CF with rash from the district of 24‐Paraganas (North) in the eastern Indian state of West Bengal were subjected to detailed clinical study to investigate the chronology and evolution of mucocutaneous lesions, the type and distribution of the rash, and systemic changes.

Result  Most patients were in their 3rd and 4th decade (23% each). Males and females were equally affected. Most patients developed the rash concomitantly with the onset or during the febrile illness, but 5 (19.2%) developed rash after defervescence. Most (57.7%) patients developed rash within the first 2 days of fever. The upper limb was the most common site of first appearance of lesions. Morbilliform eruption was the most common type of lesion noted, followed by scaling, macular erythema, intertrigo, hypermelanosis, xerosis, excoriated papules, urticaria and petechial spots. Cutaneous lesions were generally asymptomatic but 5 (19.2%) patients had intense pruritus. Mucosal lesions were found in 4 (15.4%) patients. Moderate to marked joint involvement was universal.

Conclusion  CF exanthem may have varying morphology. Since the disease is spreading widely in different parts of the world, it should be considered in the differential diagnosis of any acute febrile arthropathy with rash.
An increased body mass index is no risk factor for postoperative nausea and vomiting
Authors: Kranke P. , Apfel C. C. , Papenfuss T. , Rauch S. , Löbmann U. , Rübsam B. , Greim C.‐A. , Roewer N.
Published: August 2010

Background: An increased Body Mass Index (BMI) is almost always mentioned as a fundamental risk factor for postoperative nausea (PN), vomiting (PV) or both (PONV). However, multivariate analyses were unable to detect any correlation. Therefore, we asked whether an increased BMI is really a risk factor for PONV.

Methods: For the systematic review, a search of electronic databases and a detailed manual search of reviews were carried out. For the analysis of the original data, 587 adult patients from a randomised controlled antiemetic trial (RCT) who underwent general anaesthesia were allocated to four weight groups: Underweight (BMI<20), Normal Weight (BMI 20–25), Overweight (BMI 25–30) and Obesity (BMI≥30).

Results: Four publications with original data were found. Two described a positive relationship, although not clearly supported by the data. Despite this, most narrative reviews claimed a positive correlation between obesity and PONV by quoting again narrative reviews or misquoting originals. In the RCT, the calculated underlying risk profile for PONV was comparable between all groups. Incidences (95% confidence intervals) of PONV were 45.8% (34.0; 57.6), 41.7 (36.5; 46.9), 47.8 (38.4; 57.1) and 44.1 (31.0; 57.1), for the groups Underweight, Normal Weight, Overweight and Obesity, respectively (P=0.69). The incidences of PN and PV also did not differ with P=0.76 and P=0.36, respectively.

Conclusion: Systematic search of the literature provides no evidence for a positive relationship. Furthermore, our data confirm that an increased BMI is not a risk factor for PONV. This negative finding is important as focussing on the relevant risk factors is needed to allow for an objective risk assessment of PONV (Note).