scholarly journals Linear porokeratosis: a case report

2012 ◽  
Vol 4 (3) ◽  
pp. 105-112
Author(s):  
Slobodan Stojanović ◽  
Marina Jovanović ◽  
Nada Vučković ◽  
Siniša Tasić ◽  

Abstract Porokeratosis is a rare genodermatosis based on chronic keratinization disorder histologically characterized by the presence of a cornoid lamella and various clinical manifestations. Five most commonly described types of poroketarosis are porokeratosis of Mibelli or ”classic” porokeratosis, disseminated superfi cial actinic porokeratosis, disseminated palmoplantar porokeratosis, linear porokeratosis, and punctate porokeratosis. In all of the fi ve clinical types of porokeratosis described today, cases of planocellular skin carcinoma are described, except in punctate type cases. Use of topical CO2 laser ablation, cryotherapy and topical use of 5% Imiquimod cream, have shown favorable effects in local treatment of porokeratosis. The authors present a clinical case of a girl suffering from linear porokeratosis over the course of the last four years, spreading on the inside of her right arm along the lines of Blaschko. Linear porokeratosis was histologically confi rmed by biopsy of skin lesions and dermoscopy. Dermoscopic fi ndings, used as an auxiliary method, also indicated linear porokeratosis. Successful liquid nitrogen cryotherapy prompted the authors to present a case in which the applied treatment proved to be successful, but also to emphasize the need for timely treatment in order to prevent malignant alterations of these changes.

2011 ◽  
Vol 3 (4) ◽  
pp. 153-158
Author(s):  
Zorica Perić-Hajzler ◽  
Lidija Zolotarevski ◽  
Dušan Šofranac ◽  
Lidija Kandolf Sekulović

Abstract Lichen planus is an acquired inflammatory disease of the skin, mucous membranes and nails. It is characterized by pruritic polygonal livid papules. The disease was first described by Erasmus Wilson in 1869. It is primarily a disease of adults, and it usually occurs between the ages of 30 and 60, without gender predominance. The exact incidence and prevalence of this disease are unknown, but it is thought to affect less than 1% of the general population (0.14 to 0.80%) (1). A 63-year old male patient was admitted to our Department with itchy erythematous papules and plaques which appeared a month before admission. On admission, numerous erythematous and livid papules and plaques of polygonal shape up to 5 mm in diameter were present in the lines of Blaschko, along the left lower extremity, left side of the trunk and the left upper arm (Figures 1-3), while mucous membranes, nails and scalp were spared. Blaschko-linear distribution of skin lesions was first described by a German dermatologist Alfred Blaschko in 1901 in his work ”The distribution of nerves in the skin and their relationship to diseases of the skin”. In 1978, Happle first published that genetic mosaicism was the cause of these peculiar skin changes (1,4,6). Although knowledge of mosaicism in the skin was further elucidated in articles of several authors (Taieb in 1994, Bolognia in 1994, Heide 1996), the exact mechanism and molecular basis for the development of Blashcko linear distribution has not been fully clarified yet (5). Blaschko lines may be related to X-linked, congenital and inflammatory dermatoses, and they may be found in several skin conditions like segmental forms of atopic dermatitis, erythema multiforme, pemphigus vulgaris, vitiligo, and granuloma annulare. This is a case report of a patient with a rare form of lichen planus, with typical clinical manifestations and with Blaschko-linear distribution. Lichen planus in the lines of Blaschko was also described in several other dermatoses: lichen striatus, lichen sclerosus, morphea, porokeratosis of Mibelli, mucinosis follicularis and psoriasis vulgaris. The treatment included topical corticosteroids under occlusion, due to comorbidities, with satisfactory response. Other options include, topical calcineurin inhibitors, intralesional and systemic corticosteroids, retinoids, phototherapy and in resistant cases that severely affect the quality of life methotrexate, cyclosporine and thalidomide.


2020 ◽  
Vol 23 (2) ◽  
pp. 109-114
Author(s):  
Ekaterina V. Orlova ◽  
L. M. Smirnova ◽  
N. I. Melikova ◽  
K. Yu. Popova

In mid 2020, the number of new coronavirus (COVID-19) cases has exceeded globally six million. The clinical manifestations of COVID-19 infection are numerous mostly causing damage to the bronchopulmonary system in the form of a dry cough, rhinitis, dyspnea, fever, myalgia, and general weakness. Skin manifestations of COVID-19 are rare and currently include five types of skin lesions occurring during the infection process, which are reliably recorded in only a limited number of case reports and certain analytical publications. This article describes the clinical case of a 28-year-old patient with urticaria-like disseminated rash and confirmed COVID-19-associated pneumonia.


2020 ◽  
Vol 99 (6) ◽  
pp. 283-286
Author(s):  
S.N. Shchava ◽  
◽  
T.E. Zayachnikova ◽  
E.A. Serdjukova ◽  
E.E. Shipovskova ◽  
...  

The article presents a clinical case of a rare genetic disease in a newborn child – focal dermal hypoplasia (FDH), or Goltz syndrome. This disease primarily affects females. The girl was born into a family with a 4-year-old healthy daughter. There were no genetic disorders or kinship in family history. The article reviews a variety of clinical manifestations of FDH including skin lesions, skeletal (syndactyly) and eye abnormalities (retinal and chorioids coloboma, dystrophy of the optic nerve). It also presents genetic and molecular testing findings.


2019 ◽  
Vol 1 (9) ◽  
pp. 53-57
Author(s):  
T. N. Gavva ◽  
L. V. Kuzmenkova ◽  
Yu. N. Fedulaev ◽  
T. V. Pinchuk ◽  
D. D. Kaminer ◽  
...  

A case of lung damage in systemic lupus erythematosus (SLE) in a 33-year-old woman is described. This case is of clinical interest due to the complexity of diagnosis due to the fact that SLE is a disease with diverse clinical manifestations involving many organs and systems, which often makes it difficult to timely recognize the onset of the disease. SLE still remains a challenge and requires special attention to the patient s history, clinical and laboratory parameters of the patient, as well as specific immunological examinations.


Author(s):  
O.A. Uspenskaya ◽  
◽  
N.V. Tiunova ◽  
M.L. Zhdanova ◽  
I.Z. Zardiashvili ◽  
...  

2020 ◽  
Vol 16 (1) ◽  
pp. 33-42 ◽  
Author(s):  
Alexander K.C. Leung ◽  
Joseph M. Lam ◽  
Kin F. Leong

Background: Scabies is a skin disease caused by an obligate human parasite mite Sarcoptes scabiei var. hominis. Children under the age of two and elderly individuals are at the greatest risk. Knowledge of this condition is important for an early diagnosis to be made and treatment to be initiated. Objective: The review aimed to familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of scabies. Methods: A search was conducted using Pubmed with the built-in "Clinical Queries" tool. The search term "Scabies" was used. The categories of "epidemiology", "diagnosis", "therapy", "prevention" and "prognosis" had a limited scope for primary clinical studies. Meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews were included. Only papers published in the English language were included. A descriptive, narrative synthesis was provided of the retrieved articles. Results: Worldwide, scabies affects 200 to 300 million individuals annually. The average prevalence is estimated to be 5 to 10% in children of developing countries. Transmission usually occurs after close prolonged skin-to-skin contact. Classic scabies is characterized by an erythematous papular eruption, serpiginous burrows, and intense pruritus. Sites of predilection include the webs of the fingers, volar wrists, lateral aspects of fingers, extensor surfaces of elbows and knees, waist, navel, abdomen, buttocks, groins, and, genitals. A clinical diagnosis of classic scabies can be made on the basis of the history and clinical findings. Other clinical variants include crusted scabies, nodular scabies, and bullous scabies. Finding the mite, ova, or fecal pellets on microscopic examination of scrapings taken from skin lesions confirms the diagnosis of scabies infestation. For eradication of scabies mites, the drugs of choice are topical permethrin and oral ivermectin. Conclusion: Scabies is a highly contagious parasitic cutaneous disease that is stigmatising and debilitating. Increased awareness, accurate diagnosis, and prompt treatment are essential for the effective control of scabies and for the prevention of the spread of the disease.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Xiaowen Hu ◽  
Guofeng Zhang ◽  
Xianmeng Chen ◽  
Kai-Feng Xu

Abstract Objective To clarify the epidemiological and clinical features of Birt–Hogg–Dubé syndrome (BHDS) in Chinese patients. Methods We identified reports on Chinese patients with BHDS by searching the China Academic Journals Database, Wanfang Chinese Database, and PubMed databases, either in Chinese or English languages published from January 1, 2008 to December 31, 2020. Studies without sufficient clinical data were excluded and cases under 18 years old were excluded. Results Twenty papers were included and comprised 120 families with 221 cases. Most families with BHDS were reported from institutions in Beijing (66.7%) and Jiangsu Province (15.8%); 80.8% of cases were reported within the past five years. The average duration from clinical presentation to diagnosis was 9.6 years. The average age was 47.0 ± 13.9 years (range, 18–84 years) and the ratio of male to female was 1:1.6. The most common manifestations of BHDS were multiple pulmonary cysts (92.4%), spontaneous pneumothorax (71.0%), skin lesions (18.1%) and renal tumors (3.6%). Pulmonary cysts were predominantly distributed in the lower lobe on chest CT imaging. Family history of spontaneous pneumothorax was identified in 84.7% of the families and average number of pneumothoraxes was 1.8 (range, 1–6). The FLCN gene mutation c.1285dupC/delC in exon 11 was the most frequent mutation observed (17.4% of patients). The recurrence rate of pneumothorax after conservative treatment (including tube thoracostomy) was 29/41 (71%) while the pneumothorax recurred after surgical treatment (pulmonary bullectomy or pleurodesis) in only 4/37 (11%). Conclusions Although BHDS has been increasingly reported in the recent years, only minority of families were reported from institutions outside of Beijing and Jiangsu Province. The dominant clinical manifestations were pulmonary cysts associated with recurrent pneumothorax, while skin lesions and renal tumors were less commonly reported. Delayed diagnosis along with suboptimal management appear to represent critical challenges for Chinese patients with BHDS.


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S428-S428
Author(s):  
Jennifer Jubulis ◽  
Amanda Goddard ◽  
Elizabeth Seiverling ◽  
Marc Kimball ◽  
Carol A McCarthy

Abstract Background Leishmaniasis has many clinical manifestations and treatment regimens, dependent on species and host. Old world leishmaniasis is found primarily in Africa and Asia, and is associated with visceral disease, while new world disease, seen primarily in Latin America, is more commonly mucocutaneous. We present a case series of pediatric African patients with New World cutaneous leishmaniasis (NWCL). Methods Data extraction was performed via chart review, analyzing travel history, clinical presentation, diagnosis, and management in children with cutaneous leishmaniasis presenting to the pediatric infectious diseases clinic in Portland, ME. Biopsy specimens were sent to the federal CDC for identification by PCR and culture. Results Five cases of NWCL were diagnosed in pediatric patients in Maine from November 2018 through February 2020. Median age of patients was 10 years (range 1.5-15 years). Four cases (80%) occurred in children from Angola or Democratic Republic of Congo, arriving in Maine via Central/South America, with one case in a child from Rwanda who arrived in Maine via Texas. Three patients had multiple skin lesions and two had isolated facial lesions. Leishmaniasis was not initially suspected resulting in median time to diagnosis of 5 months (range 1-7 months). Four patients were initially treated with antibacterials for cellulitis and one was treated with griseofulvin. After no improvement, patients underwent biopsy with 2 patients diagnosed with L panamensis, 1 with L braziliensis, 1 with mixed infection (L panamensis and L mexicana), and 1 with Leishmania species only. One patient was managed with surgical excision, 3 with ketoconazole, and 1 was observed off therapy. Four patients were referred to otolaryngology. All continue to be followed in infectious disease clinic. Conclusion We present five cases of new world cutaneous leishmaniasis in African pediatric patients arriving to Maine through Latin America or Texas. Patients were diagnosed with cellulitis, tinea corporis or atopic dermatitis initially, underscoring importance of high index of suspicion in migrant patients. Detailed travel history and epidemiologic knowledge is essential to diagnosis, as patients may present with illness not congruent with country of origin. Optimal therapy remains unclear. Disclosures All Authors: No reported disclosures


2021 ◽  
Vol 13 (2) ◽  
pp. 181-188
Author(s):  
Giuseppe Ingravallo ◽  
Francesco Mazzotta ◽  
Leonardo Resta ◽  
Sara Sablone ◽  
Gerardo Cazzato ◽  
...  

Coronavirus disease 19 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is associated with various clinical manifestations, including skin lesions. In particular, during the COVID-19 pandemic lock-down period numerous chilblain-like lesions, mainly located on the feet, were observed in adolescents. The latter were often asymptomatic or associated with very mild respiratory symptoms. Here, we report three cases of acral nodular lesions in SARS-CoV-2 swab-negative adolescents with histological findings of chronic immune-mediated inflammation and immunohistochemical evidence of SARS-CoV-2 spike glycoproteins in endothelial cells and eccrine sweat glands. In one of these cases, the virus presence was confirmed by electron microscopy.


2021 ◽  
Vol 11 (2) ◽  
pp. 21-28
Author(s):  
V.P. Novikova ◽  
◽  
A.A. Pokhlebkina ◽  
D.V. Zaslavsky ◽  
A.I. Khavkin ◽  
...  

Enteropathic acrodermatitis is a rare hereditary form of zinc deficiency, characterized by periorial and acral dermatitis, alopecia and diarrhea. Refers to congenital disorders of zinc metabolism, inherited as an autosomal recessive disease resulting from mutations in the gene for the zinc transporter SLC39A4. The prevalence ranges from 1 to 9:1,000,000, with an overall incidence of 1:500,000 newborns. The disease usually manifests itself in infancy, within a few weeks of stopping breastfeeding and switching the baby to a cow's milk-based formula, or in the first days of life if artificially fed from birth. The classical clinical manifestations of acrodermatitis enteropathic are characterized by the triad: acral and periofital dermatitis, alopecia and diarrhea, but all three signs together occur only in 20% of cases. Diarrhea may develop concurrently with skin symptoms, may precede or occur later. Characteristic signs of skin lesions include sharply demarcated, dry, scaly erythematous plaques or edematous foci with vesicles and pustules on the skin of the elbow and knee joints, distal extremities, genitals, in the inguinal folds, which are usually symmetrically distributed, have sharp boundaries and irregular outlines. The course of the skin syndrome is long, as it progresses, non-healing erosive and ulcerative areas appear. Plasma zinc deficiency is the gold standard for diagnosis. Most infants with AE have low plasma zinc concentrations (<500 mcg/L or <50 mcg/dl), but a level of less than 70 mcg/L on an empty stomach or less than 65 mcg/dl in older non-dieting children is considered diagnostically significant. Treatment for this disease usually includes enteral or parenteral zinc administration, at a dose of 1-3 mg/kg/day. for elemental zinc. A clinical response is observed within 5–10 days. Supportive zinc therapy is necessary throughout the patient's life, although periods of remission have been reported. Topical therapy is also used: Dexpanthenol in the form of a cream, applied 3 times a day in the area of dermatitis, can enhance re-epithelialization. There is no significant evidence of improvement with topical zinc application. No activity restrictions are required for patients with acrodermatitis enteropathic. Key words: zinc deficiency, enteropathic acrodermatitis, children


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