Fatal group A streptococcal necrotizing fasciitis and toxic shock syndrome in a patient with psoriasis and chronic renal impairment

A 78-year-old woman presented with rapid onset of skin pain which evolved into oedema, discoloration and infarction. She was diagnosed with group A beta-haemolytic streptococcus (Streptococcus pyogenes) necrotizing fasciitis and streptococcal toxic shock syndrome. The patient had a past history of psoriasis and end-stage renal impairment. Despite treatment with multiple antibiotics in an intensive care unit, the skin infarction involving the upper trunk continued to expand and the patient died within 24 hours of hospital admission. Group A streptococcus and Staphylococcus aureus were cultured from a tissue biopsy. Renal failure and compromised skin barrier function are known to predispose to invasive streptococcal infections, but necrotizing fasciitis has only rarely been reported in association with psoriasis. This case illustrates the fulminant nature of the infection.     

Effects of diclofenac on experimental streptococcal necrotizing fasciitis (NF) in rabbit

Abstract Aggravation of necrotizing fasciitis (NF) by the administration of non-steroidal antiinflammatory drugs (NSAIDs) has recently been suggested. A rabbit model of streptococcal NF was used to study the effects of parenteral administration of an NSAID on NF evolution and outcome. Of 16 rabbits inoculated with a Streptococcus pyogenes suspension together with staphylococcal alpha toxin, 8 were treated with two doses of 4 mg/kg diclofenac on day 1 after inoculation. Clinical, bacteriological and histological studies were performed until day 10. Under our experimental conditions, NSAID treatment significantly limited NF extension. A specific inverse relationship between the extent of inflammation and bacterial density in NF lesions was observed on day 1 after inoculation in the treated group suggesting that the greater severity of NF in humans treated with an NSAID could be due to the therapeutic delay induced by the misleading clinical effects of the NSAID, and not to inhibition of antibacterial defence. Received: 23 October 1997 / Received after revision: 23 June 1998 / Accepted: 30 July 1998     

儿童坏死性筋膜炎1例

临床资料患儿,男,6岁。主因右股前内侧皮肤肿胀变黑伴发热11天,于2008年12月26日收住我院。患儿11天前右股前内侧出现直径2mm的白色水疱,     

Group a beta-haemolytic streptococcal necrotising fasciitis: early diagnosis and clinical features

Necrotising fasciitis (NF) due to group A beta-haemolytic streptococci (GAS) is a rare but still life-threatening soft-tissue infection characterised by rapidly spreading inflammation and subsequent necrosis of the muscle fascia and of the surrounding tissues. Previous studies have emphasised that the outcome of patients with NF depends essentially on early diagnosis and treatment, consisting of extensive surgical debridement, along with appropriate antibiotic therapy. However, one of the striking features of the published series of GAS NF is that there was a delay in diagnosis in several cases, which underscores the difficulty of the early diagnosis of the condition. The goal of this article was to review the clinical features and diagnostic tools that could facilitate the early recognition of GAS NF.     

Elejalde syndrome--a melanolysosomal neurocutaneous syndrome: clinical and morphological findings in 7 patients

BACKGROUND: Silvery hair and severe dysfunction of the central nervous system (neuroectodermal melanolysosomal disease or Elejalde syndrome) characterize this rare autosomal recessive disease. Main clinical features include silver-leaden hair, bronze skin after sun exposure, and neurologic involvement (seizures, severe hypotonia, and mental retardation). Large granules of melanin unevenly distributed in the hair shaft are observed. Abnormal melanocytes and melanosomes and abnormal inclusion bodies in fibroblasts may be present. Differential diagnosis with Chédiak-Higashi syndrome and Griscelli syndrome must be done. OBSERVATIONS: We studied pediatric patients with silvery hair and profound neurologic dysfunction. Immune impairment was absent. Age of onset of neurologic signs ranged from 1 month to 11 years; the signs included severe muscular hypotonia, ocular alterations, and seizures. Mental retardation since the first months of life was noted in 4 cases. Psychomotor development was normal in 3 cases, but suddenly the patients presented with a regressive neurologic process. Four patients died between 6 months and 3 years after the onset of neurologic dysfunction. One patient showed characteristic ultrastructural findings of Elejalde syndrome. CONCLUSIONS: Elejalde syndrome is different from Chédiak-Higashi and Griscelli syndrome and is characterized by silvery hair and frequent occurrence of fatal neurologic alterations. Psychomotor impairment may have 2 forms of presentation: congenital or infantile. Although Elejalde syndrome and Griscelli syndrome are similar, the possibility that they are 2 different diseases, although probably allelic related, is suggested.     

Phakomatosis pigmentovascularis II A and II B: clinical findings in 24 patients

Nearly 200 cases of phakomatosis pigmentovascularis (PPV) have been reported worldwide, most of them of Japanese origin. There are 5 types and 10 subtypes of PPV. Its etiology might be explained by the twin spotting phenomenon. The relative frequency of PPV at the National Institute of Pediatrics was 5.8 per 100,000 pediatric patients and 0.634 per 100,000 dermatological patients. We report 24 cases of PPV with an average follow up of 5 years and the following findings: PPV type II A in 4 male and 2 female patients with melanosis bulbi in 3 and glaucoma in 1. PPV type II B in 7 male and 11 female patients, with melanosis bulbi in 9, glaucoma in 9, iris mammillations in 2, Sturge Weber syndrome in 6 female patients, and Klippel-Trenaunay syndrome in 2 males, hemifacial, hemicorporal, or limb hypertrophy without venous insufficiency in 6 female and 4 male patients. During the follow-up time of 60 months, progressive fading of melanotic and vascular macules were observed in 7 patients. No other types of PPV were found. Systemic involvement in PPV was related to the body surface area affected by the vascular macules. Ectodermal and mesodermal migration disorders might be involved in the pathogenesis of PPV.     

Risk factors associated with necrotizing fasciitis of the lower limbs: A multicenter case-control study

ObjectiveThe aim of this case-control study was to identify risk factors associated with necrotizing fasciitis (NF) of the lower limbs.Patients and methodsWe conducted a prospective case-control study in hospital dermatology departments in 5 sub-Saharan African countries over a 2-year period (April 2017 to July 2019). The cases were patients with NF of the lower limbs and the controls were patients with leg erysipelas. Each case was matched with two controls for age (± 5 years) and sex. We analyzed local and general factors.ResultsDuring the study period, 159 cases (73 females, 86 males) were matched with 318 controls. The mean age was 48.5 ± 15.8 years for cases and 46.5 ± 16.2 years for controls (P = 0.24). The main local signs of NF were cutaneous necrosis (83.7%), pain (75.5%) and induration (42.1%). Multivariate analysis showed the following to be independent risk factors associated with NF of the lower limbs: obesity (odds ratio [OR] = 2.10; 95% confidence interval [CI]: 1.21–3.42), diabetes (OR = 3.97; 95% CI: 1.95–6.13), nicotine addiction (OR = 5.07; 95% CI: 2.20–11.70), use of non-steroidal anti-inflammatory drugs (NSAIDs) (OR = 7.85; 95% CI 4.60–14.21) and voluntary cosmetic depigmentation (OR = 2.29; 95% CI: 1.19–3.73).ConclusionOur study documents the role of NSAID use at the onset of symptoms as a risk factor for NF of the lower limbs. However, the originality of our study consists in the identification of voluntary cosmetic depigmentation as a risk factor for NF of the lower limbs in sub-Saharan Africa patients. Our results also identified typical overarching factors such as diabetes, obesity and nicotine addiction. Knowing these factors and taking them into account will enable optimization of management strategies for these conditions.     

Vitiligo: clinical findings in 1436 patients.

We retrospectively analyzed the clinical and epidemiological profiles of patients with vitiligo attending the pigmentary dermatoses clinic. One thousand four hundred and thirty-six patients were seen between 1989 and 1993. Males constituted 54.5% of the group and females 45.5%. Mean age of the patients was 25 years, and average disease duration at the time of hospital visit was 3.7 years. Vitiligo vulgaris was the commonest form of the disease in 1002 (69.8%) patients followed by focal vitiligo in 214 (14.9%) and segmental vitiligo in 72 (5.0%). The sites of onset were the face, trunk, and legs in descending order of frequency. Less than 20% body area involvement was seen in 1356 (94.4%) of the patients. Leukotrichia was present in 165 (11.5%), and Koebner's phenomenon was observed in 72 (5.0%). Twenty nine (2.0%) patients had associated halo nevi. Of the various diseases associated with vitiligo, atopic/nummular eczema was seen in 20 (1.4%) patients, bronchial asthma in 10 (0.7%), diabetes mellitus in 8 (0.6%), thyroid disease in 7 (0.5%), and alopecia in 6 (0.4%). A family history of vitiligo was present in 165 (11.5%) patients.     

Five cases of necrotizing fasciitis: Lack of skin inflammatory signs as a clinical clue for the fulminant type

Herein, we describe five patients with necrotizing fasciitis (NF) who had variable outcomes and clinical manifestations. At the onset, all patients exhibited purpura with or without blister and ulceration accompanied by severe pain and tenderness in the affected skin. Out of five patients, three lacked inflammatory signs such as redness and heat, and two of the three patients showed fulminant progression and died despite intensive treatments including surgical debridement, antimicrobial therapy, close monitoring and physiological support. Tissue specimens from the patients without skin inflammatory signs showed mild neutrophil infiltration in addition to necrosis from the epidermis to subcutaneous fat, and variable amounts of thrombi. Furthermore, numerous bacteria were detected by Gram stain. By contrast, the remaining two patients with skin inflammatory signs revealed slower progression, and tissue specimens from both patients showed heavy neutrophil infiltration, but bacteria were hardly detected. Therefore, these cases suggest the possibility that the paucity of skin inflammatory signs, such as redness and heat, in NF may be a clinical clue to predict the fulminant type.     

The value of bacteriology and serology in the diagnosis of necrotizing fasciitis

Fourteen patients with necrotizing fasciitis are described. In thirteen the cause was Streptococcus pyogenes [Group A beta haemolytic streptococcus (BHS)]; in the fourteenth, Staphylococcus aureus was responsible. In the acute fulminating form of the disease, BHS can be cultured from the affected tissues. In the less acute form, particularly when the patient has been previously treated with antibiotics, other bacteria colonize the tissues and the BHS cannot be isolated. Serological evidence of infection with Streptococcus pyogenes can be ascertained in all such patients by finding high levels of anti-desoxyribonuclease B and anti-hyaluronidase. Measurement of the anti-streptolysin O titre is not helpful. Once the diagnosis is made, surgical removal of all necrotic tissue is still the treatment of choice.     

Eyelid sporotrichosis: Unique clinical findings in 72 patients

Sporotrichosis on the eyelids is uncommon and has been rarely reported. As the largest series of 72 adults and children with eyelid sporotrichosis from Jilin ?P??rovince in China, this study provides useful information for the improved diagnosis and treatment of sporotrichosis.     

Epidermal nevus syndromes: clinical findings in 35 patients

Of the patients with epidermal nevi, 10-18% may have disorders of the eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms.     

Generalized granuloma annulare: clinical and laboratory findings in 100 patients

Clinical and laboratory records of 100 biopsy-proved cases of generalized granuloma annulare seen at the Mayo Clinic between 1966 and 1986 were reviewed. The skin eruption involved predominantly annular lesions in 67 patients and predominantly nonannular papules in 33. The ratio of female-to-male patients was 2.9:1 in the annular group and 1.4:1 in the nonannular group. The mean age at onset was 51.7 years. The eruption was symptomatic in 34 patients, and specific precipitating factors could be implicated in 16 patients. No consistently associated systemic disorders were identified. Diabetes mellitus was diagnosed in 21% of our referral group of generalized granuloma annulare cases, compared with 9.7% in 1350 cases of localized granuloma annulare and 10.3% in 1383 cases of all forms of granuloma annulare seen at the Mayo Clinic in the same period. Serum lipid abnormalities were more common in the generalized annular group. Follow-up data indicated a chronic, relapsing course in most patients.