Sharp Demarcation Research Articles

Spectrum of seborrheic keratoses in south Indians: A clinical and dermoscopic study

Even though seborrheic keratoses (SK) have been well characterized clinically and histopathologically, data regarding clinical and dermoscopic correlation of different types of SK are inadequate. We carried out a study to establish any correlation between the clinical and dermoscopic appearance of SK and its variants. This was a descriptive study conducted in the Department of Dermatology, a tertiary care institute, from August 2008 to June 2010. Patients with SK were evaluated with respect to age, sex, age of onset, duration, site of lesions, number of lesions, and morphology. Dermoscopy was performed in all cases. A total of 250 cases of SK were recruited. A male-to-female ratio was 1:1.04. The most common age group affected by SK was 60 years and above (40%). The most common clinical variant was common seborrheic keratosis (CSK) (60%). Comedo-like openings (CL) (80%), fissures and ridges (FR) (52%), and sharp demarcation (SD) (83%) were consistent finding on dermoscopy in CSK. Dermatosis papulosa nigra (DPN) and pedunculated seborrheic keratoses (PSK) had characteristic CL and FR in both of them. Fingerprint (FP) (55%) and network-like (NL) (88%) structures were commonly seen in flat SK. Stucco keratoses demonstrated SD (100%) and NL structures (100%). The most common clinical variant of SK was CSK, followed by DPN, PSK, Flat SK, and stucco keratoses. Dermoscopic findings were consistent with those described in the literature.

Index of Suspicion * Case 1: Fever, Barking Cough, Stridor, and Rash in an Infant * Case 2: Erythematous Papulovesicular Rash in a 3-month-old Girl * Case 3: Vomiting and Unrelenting Headaches in a 5-year-old Girl

A 6-month-old boy presents with a temperature of 38.9°C and barking cough of 3 days’ duration as well as a rash over his body for 1 day. On physical examination, his temperature is 37.5°C, respiratory rate is 28 breaths/min, and blood pressure is 87/52 mm Hg. He appears nontoxic and has a barking cough, injected conjunctiva with whitish discharge, dry lips, mild inspiratory stridor, a blanching erythematous maculopapular rash on his trunk and extremities, and small cervical lymph nodes. The rest of his physical findings are normal. His Hgb is 12.8 g/dL (128 g/L), WBC count is 11.7×103/mcL (11.7×109/L) with 75% neutrophils and 23% lymphocytes, and platelet count is 515×103/mcL (515×109/L). His stridor is considered to be due to viral croup, and he is treated with intramuscular (IM) dexamethasone and nebulized racemic epinephrine. A lateral neck radiograph reveals widening of the retropharyngeal space (Fig. 1), and he is started on clindamycin. CT scan of the neck reveals a right retropharyngeal abscess (Fig. 2). He continues to have high spiking fevers and experiences stridor at rest. He is started on IM dexamethasone every 6 hours, and his fever subsides the following day. Figure 1. Lateral neck radiograph showing widening of the retropharyngeal space. Figure 2. CT scan of the neck showing a right retropharyngeal abscess (arrow). On hospital day 6, the corticosteroids are stopped, but the fever returns the following day. Two days later, an erythematous maculopapular rash reappears, and the child’s lips are dry and cracked. On day 10, repeat CT scan shows that the retropharyngeal abscess has resolved. An additional imaging study reveals the cause of his symptoms. A 3-month-old previously healthy girl presents to the ED with a 1-day history of a blistering rash. Her parents noted an erythematous area on her occiput after …

Soft matter electrolytes based on polymethylmetacrylate dispersions in lithium bis(trifluoromethanesulfonyl)imide/1-butyl-3-methylimidazolium bis(trifluoromethanesulfonyl)imide ionic liquids

Ion transport in a polymer–ionic liquid (IL) soft matter composite electrolyte is discussed here in detail in the context of polymer–ionic liquid interaction and glass transition temperature. The dispersion of polymethylmetacrylate (PMMA) in 1-butyl-3-methylimidazolium hexafluorophosphate (BMIPF 6) and 1-butyl-3-methylimidazolium bis(trifluoromethanesulfonyl)imide (BMITFSI) resulted in transparent composite electrolytes with a “jelly-like” consistency. The composite ionic conductivity measured over the range −30 °C to 60 °C was always lower than that of the neat BMITFSI/BMIPF 6 and LiTFSI-BMITFSI/LiTFSI-BMIPF 6 electrolytes but still very high (>1 mS/cm at 25 °C up to 50 wt% PMMA). While addition of LiTFSI to IL does not influence the glass T g and T m melting temperature significantly, dispersion of PMMA (especially at higher contents) resulted in increase in T g and disappearance of T m. In general, the profile of temperature-dependent ionic conductivity could be fitted to Vogel–Tamman–Fulcher (VTF) suggesting a solvent assisted ion transport. However, for higher PMMA concentration sharp demarcation of temperature regimes between thermally activated and solvent assisted ion transport were observed with the glass transition temperature acting as the reference point for transformation from one form of transport mechanism to the other. Because of the beneficial physico-chemical properties and interesting ion transport mechanism, we envisage the present soft matter electrolytes to be promising for application in electrochromic devices.

Modernity, Enhanced Working Memory, and the Middle to Upper Paleolithic Record in the Levant

The Eurocentric (or rather Western European) outlook on cultural evolution envisioned a sharp demarcation between the phenomena grouped under the title Middle Paleolithic (i.e., archaic) and the phenomena recognized as Upper Paleolithic (i.e., modern). This view became the framework for testing cognitive, social, and economic hypotheses explaining the emergence of modern behavior. After nearly a century of research, it seems that this categorization is not applicable in the Levant despite some broad similarities between the two regions. The Levantine archaeological record provides us with the option to think outside the “European box” with regard to this important phase in human cultural evolution.

Oligodendroglial neoplasms with ganglioglioma-like maturation: a diagnostic pitfall

Although oligodendroglial neoplasms are traditionally considered purely glial, increasing evidence suggests that they are capable of neuronal or neurocytic differentiation. Nevertheless, ganglioglioma-like foci (GGLF) have not been previously described. Herein, we report seven examples where the primary differential diagnosis was a ganglioglioma with an oligodendroglial component. These five male and two female patients ranged in age from 29 to 63 (median 44) years at initial presentation and neuroimaging features were those of diffuse gliomas in general. At presentation, the glial component was oligodendroglioma in six and oligoastrocytoma in one; one was low-grade and six were anaplastic. A sharp demarcation from adjacent GGLF was common, although some intermingling was always present. The GGLF included enlarged dysmorphic and occasionally binucleate ganglion cells, Nissl substance, expression of neuronal antigens, GFAP-positive astrocytic elements, and low Ki-67 labeling indices. In contrast to classic ganglioglioma, however, cases lacked eosinophilic granular bodies and CD34-positive tumor cells. Scattered bizarre astrocytes were also common and one case had focal neurocytic differentiation. By FISH analysis, five cases showed 1p/19q codeletion. In the four cases with deletions and ample dysmorphic ganglion cells for analysis, the deletions were found in both components. At last follow-up, two patients suffered recurrences, one developed radiation necrosis mimicking recurrence, and one died of disease 7.5 years after initial surgery. We conclude that GGLF represents yet another form of neuronal differentiation in oligodendroglial neoplasms. Recognition of this pattern will prevent a misdiagnosis of ganglioglioma with its potential for under-treatment.

Trichoblastoma of the Breast Detected by Screening Mammography: A Diagnostic Pitfall

Trichoblastoma of the Breast Detected by Screening Mammography: A Diagnostic Pitfall

Donor Dominance Cures CHILD Nevus

Background: Congenital hemidysplasia with ichthyosiform nevus and limb defects (MIM 308050, CHILD) syndrome is an X-linked dominant, male-lethal, multisystem birth defect. Patients suffer from an inflammatory nevus that covers large areas, predominantly of one side of the body, with a sharp midline demarcation. Treatment of CHILD nevus is notoriously difficult. Objective: The aim of this study was to develop a novel surgical approach for this disorder. Methods: In 2 patients, the CHILD nevus was dermabraded, and the area was covered with split skin grafts obtained from a contralateral unaffected donor region. In a third patient, papillomatous, strawberry-like lesions on fingers and toes were excised, and the defects were covered with full-thickness grafts obtained from the unaffected left, gluteal area. Results: Highly satisfying functional and cosmetic results were documented during a follow-up period ranging from 3 to 8 years. Conclusion: The favorable outcome, superior to that obtained by simple dermabrasion or extensive plastic surgery, can best be explained by the donor dominance of the grafted skin samples that carried, in all or most cells, the mutant X chromosome in an inactivated form.

Simulation of 3D needle-tissue interaction with application to image guided prostate brachytherapy

To improve global control of disease and reduce global toxicity, a complex seed distribution pattern should be achieved with great accuracy during brachytherapy. However, the interaction between the needle and prostate will cause large deformation of soft tissue. As a result, seeds will be misplaced, sharp demarcation between irradiated volume and healthy structures is unavailable and this will cause side effects such as impotence and urinary incontinence. In this paper, a 3D nonlinear dynamic finite element simulation method with application to robot-assisted image guided brachytherapy is proposed. A magnetic resonance imaging (MRI) based simulation model is built which considers real patient data, nonlinear material property and interaction between the needle and soft tissue. The sensitivities of seed placement error to the needle insertion point, needle orientation and insertion distance are analysed. The result shows that shorter distance between insertion point and target position with compensation in distance can reduce placement error effectively. This method can be used as a complementary instruction for robot-assisted image guided brachytherapy.

Asymmetric endoscopic inflammation of the ileal pouch: A sign of ischemic pouchitis?

Pouchitis is associated with dysbiosis and dysregulated mucosal immunity, although secondary pouchitis with special etiologic factors, such as ischemia, can occur. The aim was to describe a disease phenotype of the ileal pouch with an endoscopic appearance suggestive of ischemia. We identified consecutive patients with endoscopic asymmetric inflammation of the pouch (inflammation of side of the pouch with a completely normal other limb of the pouch one limb and a sharp demarcation along the staple suture line). Patients with Crohn's disease (CD) of the pouch or antibiotic-responsive pouchitis, matched for duration of the pouch, served as controls. Histology slides of mucosal biopsies were re-reviewed independently by 2 blinded gastrointestinal pathologists. Demographic, clinical, endoscopic, histologic, and imaging characteristics were compared between the groups. Ten patients with "ischemic" pouchitis, 15 with CD of the pouch, and 15 with antibiotic-responsive pouchitis were studied. Pyloric gland metaplasia was observed only in the groups with CD of the pouch (23.1%) or antibiotic-responsive pouchitis (13.3%). Of patients with "ischemic" pouchitis, 80% had extracellular hemosiderin or hematoidin deposits (versus 30.8% those with CD of the pouch and 13.3% of those with pouchitis, P = 0.003). The majority of patients (80%) with "ischemic" pouchitis did not respond to conventional antibiotic therapy. It appeared that subsequent abdominal surgeries after pouch construction and a history of postoperative portal vein thrombi were associated with "ischemic" pouchitis. Endoscopic asymmetric inflammation of the pouch may represent an ischemia-associated pouchitis with characteristic clinical, radiographic, and histologic features. Its hemodynamic, cellular, and molecular basis of mechanism warrants further study.

Mutation of the Variant α-Tubulin TUBA8 Results in Polymicrogyria with Optic Nerve Hypoplasia

The critical importance of cytoskeletal function for correct neuronal migration during development of the cerebral cortex has been underscored by the identities of germline mutations underlying a number of human neurodevelopmental disorders. The proteins affected include TUBA1A, a major alpha-tubulin isoform, and microtubule-associated components such as doublecortin, and LIS1. Mutations in these genes are associated with the anatomical abnormality lissencephaly, which is believed to reflect failure of neuronal migration. An important recent observation has been the dependence of cortical neuronal migration upon acetylation of alpha-tubulin at lysine 40 by the histone acetyltransferase Elongator complex. Here, we describe a recognizable autosomal recessive syndrome, characterized by generalized polymicrogyria in association with optic nerve hypoplasia (PMGOH). By autozygosity mapping, we show that the molecular basis for this condition is mutation of the TUBA8 gene, encoding a variant alpha-tubulin of unknown function that is not susceptible to the lysine 40 acetylation that regulates microtubule function during cortical neuron migration. Together with the unique expression pattern of TUBA8 within the developing cerebral cortex, these observations suggest a role for this atypical microtubule component in regulating mammalian brain development.

Polioencefalomalacia experimental induzida por amprólio em ovinos

Para estabelecer um modelo experimental para o estudo da etiologia, patologia e patogênese da polioencefalomalacia em ruminantes, a condição foi induzida em cinco ovinos pela administração oral de amprólio nas doses diárias de 500 e 1.000mg/kg de peso animal, respectivamente por 28-59 dias e 13-39 dias. Todos os ovinos morreram ou foram eutanasiados in extremis após um curso clínico de 3-7 dias. Os sinais clínicos incluíam depressão, incoordenação, midríase, bruxismo, cegueira e decúbito com opistótono e movimentos de pedalagem. Salivação excessiva e posição de cavalete foi observada em um ovino e mioclonias em um outro. Os principais achados de necropsia restringiam-se ao sistema nervoso central e incluíam tumefação do encéfalo com achatamento dos giros telencefálicos e hemorragias nos lobos parietal e occipital do telencéfalo; as hemorragias ocorriam também nas áreas submeníngeas da medula espinhal e do mesencéfalo. Histologicamente, havia necrose segmentar laminar de neurônios (neurônios vermelhos) associada a edema, tumefação de células endoteliais, hemorragias e infiltração por macrófagos espumosos (células gitter). Essas alterações eram mais marcadas nos lobos frontal, parietal e occipital do telencéfalo e havia uma demarcação abrupta entre as lesões e o neurópilo normal adjacente. Adicionalmente, lesões semelhantes, mas menos acentuadas, eram observadas no mesencéfalo, tálamo e hipocampo. Levando em consideração a reproducibilidade regular dos aspectos da polioencefalomalacia em ovinos pela administração de amprólio, esse modelo pode ser útil para o estudo da doença.

Poster — Wed Eve—16: Optoacoustic Detection of Tissue Thermal Damage

Minimally invasive thermal therapy has been investigated as an alternative treatment modality for solid tumours including breast, liver and prostate. Thermal therapy is typically delivered as a single‐fraction, stand‐alone therapy. It involves heating tissues to greater than 55 C over a period of a few minutes, which results in coagulative necrosis. It can potentially achieve highly conformal 3D coagulation volumes, exhibits sharp demarcation between treated and spared tissues, and tissue effects can be observed and thus potentially controlled in real time. This paper describes a new approach to guiding the progress of thermal therapy using optoacoustics, a technique which combines the high optical contrast and high resolution associated with near‐infrared optical imaging and ultrasound imaging, respectively. In this study, thermal lesions were induced in bovine liver ex vivo via non‐contact single fiber laser heating at 810 nm. Optoacoustic signals were obtained using an optoacoustic imaging system comprised of an Nd:YAG pumped Titanium‐Sapphire laser delivering 6 ns pulses and an annular array of 8, 4 MHz transducers. Optoacoustic signal increased up to 2.5 fold with heating times from 1 to 6 minutes. Furthermore, tissue coagulation was clearly visible in the optoacoustic images compared to the surrounding native tissue. The results demonstrate that optoacoustic signals are sensitive to changes in tissue optical and mechanical properties that occur when tissues are thermally damaged, and, hence, optoacoustic imaging may be a suitable candidate for guiding thermal therapy delivery.

Maize host requirements for Ustilago maydis tumor induction.

The biotrophic pathogen Ustilago maydis causes tumors by redirecting vegetative and floral development in maize (Zea mays L.). After fungal injection into immature tassels, tumors were found in all floral organs, with a progression of organ susceptibility that mirrors the sequential location of foci of cell division in developing spikelets. There is sharp demarcation between tumor-forming zones and areas with normal spikelet maturation and pollen shed; within and immediately adjacent to the tumor zone, developing anthers often emerge precociously and exhibit a range of developmental defects suggesting that U. maydis signals and host responses are restricted spatially. Male-sterile maize mutants with defects in anther cell division patterns and cell fate acquisition prior to meiosis formed normal adult leaf tumors, but failed to form anther tumors. Methyl jasmonate and brassinosteroid phenocopied these early-acting anther developmental mutants by generating sterile zones within tassels that never formed tumors. Although auxin, cytokinin, abscisic acid and gibberellin did not impede tassel development, the Dwarf8 mutant defective in gibberellin signaling lacked tassel tumors; the anther ear1 mutant reduced in gibberellin content formed normal tumors; and Knotted1, in which there is excessive growth of leaf tissue, formed much larger vegetative and tassel tumors. We propose the hypothesis that host growth potential and tissue identity modulate the ability of U. maydis to redirect differentiation and induce tumors.

Experimental investigation of transition from Fowler–Nordheim field emission to space-charge-limited flows in a nanogap

An experimental investigation of the transition from Fowler–Nordheim (FN) field emission to space-charge-limited (SCL) flows in a nanogap is presented. Electrodes with gap size D∼30–70 nm corresponding to D/λo up to a maximum of ∼2×103, where λo is the de Broglie wavelength of the space-charge-electrons, are experimented. The transition from the FN field emission to the classical SCL flow is a function of the applied bias and lies in the range 5–15 V. The equilibrium transmitted current density for the 50 nm sample indicates a transition from the FN to the quantum SCL flow at ∼0.4 V with D/λo of ∼35 and then gradually to the classical SCL behavior as the voltage is increased beyond ∼9 V. The experiments indicate no sharp demarcation between the different regimes.

The Germinal centre‐derived lymphomas seen through their cellular microenvironment

The human lymph node is a complex tissue resulting from the microenvironmental organisation of different cell populations linked by topographical and/or functional relationships. Germinal centres (GCs) of lymphoid follicles contain a meshwork of follicular dendritic cells in addition to B-cells and some CD4(+) T cells. Moreover, there is a sharp demarcation around the whole follicle centre, which is highlighted by fibroblastic reticulum cells. On the whole, GC exerts a role in B cell physiology and malignancy. In GC-derived lymphomas, gene expression profiling studies have raised the possibility that survival of the affected patients may be associated with signatures preferentially expressed in non-malignant T cells and macrophages and/or dendritic cells. Immunohistological analyses in lymphoma biopsy samples have confirmed that the biological behaviour and tumour progression may be influenced by the tumour microenvironment. This review will examine GC-derived lymphomas, including follicular lymphomas, Hodgkin lymphomas and angioimmunoblastic T-cell lymphoma, through their integrated cellular microenvironment, highlighting those findings which may serve as a useful surrogate marker for tumour diagnosis or tumour progression, together with key molecules involved in tumour development.

Inner Border—Specific and Significant Colposcopic Sign for Moderate or Severe Dysplasia (Cervical Intraepithelial Neoplasia 2 or 3)

Some investigators have reported that presence of the inner border may be a colposcopic sign for the presence of high-grade cervical neoplasia. The inner border can be defined as a sharp acetowhite demarcation or transformation zone within a less opaque acetowhite area. The association of the colposcopic sign inner border with moderate/severe cervical intraepithelial lesions has not been thoroughly investigated. This retrospective study examined the correlation between the inner border and cervical intraepithelial neoplasia (CIN) 2 or 3, and also evaluated the correlation of the presence or absence of the inner border with specific types of human papillomavirus (HPV). Colpophotographs and cervicograms of 947 women (mean age, 32 years: range 15–79 years) who had been referred to a colposcopy clinic because of an abnormal cervical finding were reviewed by two independent colposcopists in order to evaluate retrospectively the occurrence of the colposcopic sign inner border. Histologic evaluation was based on colposcopy-directed punch or cone biopsies. Human papillomavirus testing and typing were performed using Hybrid capture I or a polymerase chain reaction-enzyme immunoassay. Among women with an atypical transformation zone, the prevalence of the colposcopic sign inner border was 7.6% (53/695). Histological examination detected CIN 2 or 3 in 70% of women with inner border. The sensitivity and specificity of the colposcopic sign inner border for detection of CIN 2 or 3 were 20% and 97%, respectively. The odds ratio for CIN 2 or 3 among patients with inner border was 7.7 (95% confidence interval, 4.2–14.3). No significant association was found between inner border and any high-risk HPV type. Inner border associated with CIN 2 or 3 was significantly more frequent in women younger than 35 years (P < 0.05). The investigators conclude from these findings that colposcopic sign inner border is a rare but valuable and highly specific marker for CIN 2 or 3 in young women.

Laparoscopic Gastropexy for the Treatment of Gastric Volvulus Associated with Wandering Spleen

A 2.5-year-old boy was referred to the emergency room for a sudden onset of diffuse and increasing abdominal pain with lethargy, abdominal distension, and vomiting, all in the past 24 hours. A plain abdominal X-ray showed gastric distension. Two liters of gastric contents were evacuated by suction. The abdominal sonogram showed an unusual position of the spleen in the left-lower quadrant, with no splenic ischemia. The diagnosis of gastric volvulus associated with a wandering spleen was then evoked. Laparoscopic exploration revealed a nonischemic spleen, absence of normal supporting ligaments for the spleen, and gastric distension with flaccid gastric walls. The spleen was then easily moved in the left-under quadrant. A parietal peritoneal posterolateral incision was made, opposite the large gastric curve, up to the diaphragm (7 cm). This delimitated a sharp demarcation zone between the two edges of the incised peritoneum. The stomach was fixed to the peritoneal incision, covering and anchoring the spleen in a good position. Recovery was uneventful, and an abdominal sonogram performed 4 years after the surgery shows a viable spleen in its correct location. The rarity of gastric volvulus associated with a wandering spleen and its fast clinical improvement with medical treatment often delays the diagnosis and the surgical treatment. Laparoscopy in this case has a dual relevance: diagnosis and therapeutic management (splenectomy or gastropexy). Laparoscopic gastropexy for the treatment of gastric volvulus associated with a wandering spleen is an easy procedure and combines the advantages of all the surgical techniques previously described.

Adrenal hemangioma‐adenoma: An exceedingly rare adrenal collision tumor

Adrenal collision tumors are rare clinical entities referring to separate coexisting adjacent tumors involving an adrenal gland with sharp demarcation between the two and without a substantial histologic admixture at the interface. We report a case of a 60-year-old female patient with an exceedingly rare adrenal hemangioma-adenoma collision tumor. To our knowledge, this is the first report of a collision tumor comprising an adrenal hemangioma and an adenoma.

Pneumocystis jiroveci pneumonia in patients with rheumatoid arthritis treated with infliximab: A retrospective review and case–control study of 21 patients

To establish proper management of Pneumocystis jiroveci pneumonia (PCP) in rheumatoid arthritis (RA) patients treated with infliximab. PCP has been observed in 0.4% of patients with RA treated with infliximab in Japan. Data from patients with RA (n = 21) who were diagnosed with PCP during infliximab treatment and from 102 patients with RA who did not develop PCP during infliximab therapy were collected from 14 rheumatology referral centers in Japan. A retrospective review of these patients and a case-control study to compare patients with and without PCP were performed. The median length of time from the first infliximab infusion to the development of PCP was 8.5 weeks. At the onset of PCP, the median dosages of prednisolone and methotrexate were 7.5 mg/day and 8 mg/week, respectively. Pneumocystis jiroveci was microscopically identified in only 2 patients, although the polymerase chain reaction test for the organism was positive in 20 patients. The patients with PCP had significantly lower serum albumin levels (P < 0.001) and lower serum IgG levels (P < 0.001) than the patients without PCP. Computed tomography of the chest in all patients with PCP revealed ground-glass opacity either with sharp demarcation by interlobular septa or without interlobular septal boundaries. Sixteen of the 21 patients with PCP developed acute respiratory failure, but all survived. PCP is a serious complication that may occur early in the course of infliximab therapy in patients with RA. For the proper clinical management of this infectious disease, physicians need to be aware of the possibility of PCP developing during infliximab therapy.

Papular-purpuric gloves and socks syndrome associated with B19V infection in a 6-year-old child

A 6 year-old girl was admitted for evaluation of a fever associated with a petechial rash of 2 days’ duration. She was in good general condition with no acute distress. Inspection of the skin revealed an amazing papular and purpuric rash of predominantly acral and symmetrical distribution and sharply demarcated on the ankles. All laboratory tests were found normal. Rash and fever completely resolved in less than 3 days. Serological testing for parvovirus B19 (B19V) antibodies was positive for IgM but negative for IgG. Moreover, B19V DNA was detected in serum with a viral load of 2.24 × 10 8 copies per mL. So we concluded of a paediatric case of popular-purpuric gloves and socks syndrome (PPGSS) associated with B19V infection. PPGSS is an idiosyncratic reaction to viral infection. The syndrome has been associated with several viruses such as HHV6, measles, coxsackie B6, and above all B19V. PPGSS occurs mostly in young adults. It is characterised by a typical papular and purpuric rash with an acral distribution and a sharp demarcation on the wrists and ankles. The rash is often pruritic and can be accompanied by mucosal lesions and/or systemic symptoms such as fever, asthenia and lymphadenopathy. Most of the time, the disease is self-limited with a short course and a benign prognosis. A very similar disease has been described in some children. The distinctive clinical characteristics of PPGSS in children should be recognized by paediatrician in particular at the emergency room in order to avoid superfluous explorations.