Methotrexate treatment in a case of juvenile pityriasis rubra pilaris

An 8‐year‐old boy who was initially diagnosed with plaque psoriasis failed management with topical therapies and skin biopsy confirmed the suspected diagnosis of juvenile pityriasis rubra pilaris (PRP). Pityriasis rubra pilaris is a rare inflammatory disorder of the skin characterized by follicular keratotic papules coalescing into plaques, along with palmoplantar keratoderma. Treatment modalities include topical and systemic therapies, although previous studies have not shown much benefit with methotrexate in children. We present a case in which methotrexate led to significant improvement of the skin findings in a child with type IV juvenile pityriasis rubra pilaris.     

Combination of 595-nm pulsed dye laser,long-pulsed 755-nm alexandrite laser,and microdermabrasion treatment for keratosis pilaris: retrospective analysis of 26 Korean patients

Abstract

Keratosis pilaris (KP) has beenpresented as small keratotic follicular papules with or without surrounding erythema. Various treatments with laser or light therapy have been used for the management of KP with various clinical outcomes. In the present study, we investigated the efficacy and safety of a combination therapy for KP. A total of 29 anatomical sites with KP in 26 patients were treated using a 595-nm pulsed dye laser (PDL) with nonpurpuragenic fluences, a long-pulsed 755-nm alexandrite laser, and microdermabrasion. Clinical improvement was assessed by comparing preand posttreatment clinical photographs and patient satisfaction rates. Evaluation of the clinical results three months after the treatments showed that 12 of the 29 anatomical sites (41.4%) demonstrated Grade 3 clinical improvement, ten (34.5%) had Grade 2 clinical improvement, four (13.8%) showed Grade 1 improvement, and three (10.3%) showed Grade 4 improvement. We observed that KP lesions improved not only in erythema and skin texture, but also in brownish dyschromias. Potential adverse events were not observed, except prolonged posttherapy scaling. Our observations demonstrate that combination therapy using a 595-nm PDL, a long-pulsed 755-nm alexandrite laser, and microdermabrasion can have a positive therapeutic effect on KP.     

Case for diagnosis. Atypical Grover’s disease

A 55-year-old male presented with an eight-month history of erythematous papules and plaques with demarcated areas of spared skin on his trunk, upper extremities, neck, and face. Grover’s disease is a rare, acquired disorder of unknown origin, which is classically characterized by the appearance of erythematous papules on the upper trunk that are usually transient. As in the present case, there are reports of atypical disease, with facial involvement, pityriasis rubra pilaris-like lesions, and a more chronic course.     

Benign Cephalic Histiocytosis: A Case Report

Histiocytic skin disorders are usually classified as either Langerhans'' cell histiocytosis (LCH) or non LCH, based on the pathology. Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytitic disorder and is characterized by self-healing multiple small eruptions of yellow to red-brown papules on the face and upper trunk. Histologic features of this disorder show dermal proliferation of histiocytes that have intracytoplasmic comma-shaped bodies, coated vesicles and desmosome-like structures. In this study, we report on a 7-month-old boy who contained small yellow-red papules on his face that spread to his upper trunk. The clinical and histologic features in this patient were consistent with BCH.     

A pilot trial of dermoscopy as a rapid assessment tool in pediatric dermatoses

Dermoscopy is a noninvasive technique to assess skin architecture. A pilot study was conducted using polarized dermoscopy as a tool to monitor the pediatric skin barrier. Ten pediatric patients (age range, 1-14 years) with mild to moderate atopic dermatitis (AD), ichthyosis vulgaris (IV), and/or keratosis pilaris (KP) participated in a 4-week clinical trial. After a week of emollient usage alone, a mid-potency topical corticosteroid cream was added twice daily if necessary to treat erythema, dermatitis, or pruritus. The participants were assessed at weeks 0, 1, and 4 using the eczema area and severity index (EASI) for atopic dermatitis, investigator global assessment for atopic dermatitis, children dermatology life quality index (CDLQI), and clinical and dermoscopic photography. Dermoscopic appearance demonstrated dermal vascular ectasia in AD and KP, hyperkeratosis and prominence of the interkeratinocyte space in AD and IV and widening of the follicular orifice in KP. Improvements in these dermoscopic abnormalities were noted after emollient usage, mirroring improvements in clinical appearance, EASI, and CDLQI. Dermoscopy is a promising tool to assess localized improvement in skin architecture in pediatric dermatoses. Further studies and development of scoring systems will be needed to apply this technology to clinical practice.     

Papular, profuse, and precocious keratosis pilaris

Keratosis pilaris (KP) is a frequent and benign condition in children characterized by the presence of rough, follicular papules and varying degrees of erythema. Different variants have been described, including simple KP and red KP. Between September 2007 and October 2010, 11 children with profuse and precocious KP seen at the department of pediatric dermatology were included. They defined an underemphasized clinical variant of childhood KP: the papular, profuse, and precocious KP characterized by early age of onset (<18 mos), extensive involvement of the limbs and cheeks, and papular nature of lesions. No clinical association has been found. The main complication was episodes of folliculitis. Diagnosis was delayed for all patients. Treatment is difficult, but association between emollient and keratolytic agents can provide some help.     

Tinea versicolor mimicking pityriasis rubra pilaris

Tinea versicolor is a common noninvasive cutaneous fungal disease. We recount a case of tinea versicolor that mimicked type I (classic adult) pityriasis rubra pilaris. A 54-year-old white man reported a 20-year history of a recurrent pruritic eruption that had marginally improved with use of selenium sulfide shampoo and treatment with oral antihistamines. Results of a skin examination revealed erythematous plaques; islands of spared skin; and follicular erythematous keratotic papules on the trunk, shoulders, and upper arms. A lesion was scraped to obtain skin scales for potassium hydroxide staining. Examination of the stained samples revealed the characteristic "spaghetti and meatballs," confirming the diagnosis.     

Pityriasis rubra pilaris as a systemic disease

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder of unknown etiology, initially described in 1835. It is characterized by keratotic follicular papules, well-demarcated salmon-colored erythematous scaly plaques interspersed with distinct islands of uninvolved skin, and palmoplantar keratoderma. Is PRP a systemic disease? Skin is mainly affected in PRP. Despite its clinical heterogeneity, PRP could be associated with a variety of rheumatologic, infectious, neoplastic, and other extracutaneous manifestations. We accept the hypothesis of not only an association but also a causative relation between skin and systemic manifestations with possible common underlying pathomechanisms such as systemic immunologic processes and superantigen mimicry.     

Keratosis pilaris and keratosis pilaris atrophicans faciei

Keratosis pilaris and ulerythema ophryogenes (keratosis pilaris atrophicans faciei) are hereditary disorders with altered follicular keratinization that show follicular, horny papules surrounded by an erythematous halo. Ulerythema ophryogenes is an uncommon variant of keratosis pilaris characterized by erythematous follicular papules of the eyebrows and cheeks followed by a gradual loss of hair. On the background of 15‐year‐old boy who presented with keratosis pilaris and ulerythema ophryogenes, we discuss the various clinical manifestations of keratosis pilaris.     

Pustular patch test reactions in atopic dermatitis.

Pustular patch test reactions to 5% nickel sulfate were regularly produced in patients with atopic dermatitis when patches were placed over areas of skin with (a) follicular papules, (b) erythema, (c) lichenification, and (d) minimal trauma. The pustular patch test reactions seldom occurred in the normal-appearing skin of these patients. However, if the skin was traumatized prior to the patch test, the reaction was produced in the normal-appearing skin of atopic as well as control individuals. No pustular reactions occurred in the follicular lesions of keratosis pilaris, supporting the view that follicular lesions of atopic dermatitis differ from keratosis pilaris. It is suggested that pustular patch test reactions are caused by primary irritation.     

儿童常见丘疹性疾病反射式共聚焦显微镜图像特征分析

目的 探讨反射式共聚焦显微镜（RCM）在儿童常见丘疹性疾病（光泽苔藓、扁平疣、线状苔藓、粟丘疹、传染性软疣、毛发苔藓）中的临床应用价值。方法 收集579例以丘疹为临床表现的门诊儿童病例,利用RCM分别观察皮损处及皮损周边正常皮肤的镜下结构,分析总结6种丘疹性疾病RCM图像的基本特征及鉴别要点。结果 经RCM检测后确诊光泽苔藓236例,扁平疣70例,线状苔藓123例,粟丘疹40例,传染性软疣53例,毛发苔藓57例。6种疾病均具有典型RCM特征,光泽苔藓可见扩大的真皮乳头,内有致密炎细胞及噬色素细胞浸润;扁平疣的颗粒层及棘层细胞呈同心圆状排列,似玫瑰花团样;线状苔藓棘层灶性水肿,基底细胞无或局灶性液化变性,真皮浅层中等量炎细胞呈簇状分布;粟丘疹在真皮可见境界清晰的圆形或椭圆形结构,内含高折光物质,且内容物折光不均匀;传染性软疣在RCM下可见完整的囊腔样结构,内有较高折光的软疣小体;毛发苔藓的毛囊漏斗部扩张,可见中低折光的近似角质样物质。6种疾病RCM下鉴别要点在于连续纵扫下皮损部位的结构特征（模式、折光度）。结论 RCM在儿童丘疹性疾病的诊断中具有较高临床应用价值。     

Severe Monilethrix Associated with Intractable Scalp Pruritus,Posterior Subcapsular Cataract,Brachiocephaly, and Distinct Facial Features: A New Variant of Monilethrix Syndrome?

Monilethrix is a rare developmental hair shaft defect characterized by small elliptical node-like deformities with increased hair fragility resulting in partial or diffuse alopecia. The disorder is usually transmitted in an autosomal dominant fashion with incomplete penetrance and variable expressivity, but autosomal recessive inheritance has also been reported. It is thought to be without systemic involvement, whereas keratosis pilaris and follicular papules are almost invariably associated features. We describe an instance of monilethrix in a 9-year-old boy from consanguineous parents, characterized by universal dystrophic alopecia associated with intractable scalp pruritus, diffuse keratosis pilaris, and bilateral posterior subcapsular cataracts. His disease was further characterized by physical underdevelopment and distinct features of hypertelorism, a wide-based nose, long philtrum, relatively large mouth with thick lower lip, enlarged forehead, small, receding chin, short neck, and rounded (ultrabrachycranial) skull. The findings in our patient suggest that "monilethrix syndrome" is an appropriate term for defining the instances of monilethrix associated with other abnormalities. We conclude that our patient may represent a new and severe, autosomal recessive variant of monilethrix syndrome.     

Keratosis pilaris rubra: a common but underrecognized condition

BACKGROUND: Keratosis pilaris is a common skin disorder of childhood that often improves with age. Less common variants of keratosis pilaris include keratosis pilaris atrophicans and atrophodermia vermiculata. OBSERVATIONS: In this case series from dermatology practices in the United States, Canada, Israel, and Australia, the clinical characteristics of 27 patients with keratosis pilaris rubra are described. Marked erythema with follicular prominence was noted in all patients, most commonly affecting the lateral aspects of the cheeks and the proximal arms and legs, with both more marked erythema and widespread extent of disease than in keratosis pilaris. The mean age at onset was 5 years (range, birth to 12 years). Sixty-three percent of patients were male. No patients had atrophy or scarring from their lesions. Various treatments were used, with minimal or no improvement in most cases. CONCLUSIONS: Keratosis pilaris rubra is a variant of keratosis pilaris, with more prominent erythema and with more widespread areas of skin involvement in some cases, but without the atrophy or hyperpigmentation noted in certain keratosis pilaris variants. It seems to be a relatively common but uncommonly reported condition.     

Dermatological manifestations in Noonan syndrome

Noonan syndrome (NS) is a genetic disorder affecting up to 1 in 1000 people. It is recognised by an unusual facial appearance, heart defects and small stature. Some people with features of NS also have lots of small dark brown flecks on their skin (lentigines), a condition formerly known as LEOPARD syndrome but now called NS with multiple lentigines (NSML). There are also some atypical (unusual) “Noonan-like” syndromes: this overlapping group of “Rasopathies” also includes Costello syndrome and cardio-facio-cutaneous syndrome. About 10 genes responsible for these conditions are now known, but the dermatological (skin) features of the different genetic types have never been clearly defined. Therefore, these French doctors, based at 10 different hospitals, systematically recorded the skin abnormalities seen in patients with any kind of NS attending their clinics over a 4-year period, and correlated them with NS subtype and gene. Of 129 patients, aged from 9 months to 77 years, three quarters had mutations (changes) in the PTPN11 gene and of these 65 were diagnosed as NS and 34 as NSML. Dermatological features in the NS patients were mild and non-specific such as easy bruising, raised hair follicles (keratosis pilaris), lax skin or wavy hair. NSML patients additionally had dozens of lentigines, larger brown patches and usually had particular PTPN11 mutations. NS patients with mutations in a different gene were more likely to have striking skin features including unusually curly hair, sparse eyebrows, marked keratosis pilaris or multiple moles. This research highlights the value of careful dermatological documentation in patients with genetic syndromes.     

Ulerythema Ophryogenes and Keratosis Pilaris in a Child with Monosomy 18p

Abstract: We report a 13-year-old boy with deletion of the short arm of chromosome 18 and follicular, partialy inflammatory, keratotic papules of the eyebrows, foreskin, and cheeks (ulerythema ophryogenes) as well as the shoulders, upper back, upper arms, and thighs (keratosis pilaris), initially diagnosed as atopic dermatitis. Over 100 patients with this genetic defect have been reported, and the 18p- syndrome is considered one of the most frequently occurring deletion syndromes. However, ulerythema ophryogenes and keratosis pilaris have not been described in any of these patients, although the association of the latter with other genetic abnormalities is well known. Keratosis pilaris is a relatively common genodermatosis of ectodermal origin, frequently occurring with ichthyosis or atopy; concomitance with ulerythema ophryogenes has also been reported. The association of chromosome 18p deletion defect and ulerythema ophryogenes may be helpful in future attempts to localize the gene defect responsible for follicular genokeratoses.     

High body mass index, dry scaly leg skin and atopic conditions are highly associated with keratosis pilaris

BACKGROUND: In a previous study we have found that young patients with insulin-dependent diabetes mellitus had a higher prevalence of keratosis pilaris (KP) than healthy controls, with a high correlation with body mass index (BMI) and ichthyosiform skin changes of the legs. Objectives: To investigate whether BMI, dry scaly legs and atopic conditions could be associated with KP in a healthy population of adolescents. METHODS: A total of 202 Jewish adolescents chosen at random among students undergoing a routine medical examination at school participated in the study. The patients filled in a questionnaire for data on ethnic origin, the presence or history of allergic rhinitis, asthma or atopic dermatitis, the presence of thyroid disease, diabetes or dry skin. A similar questionnaire was sent to the family physician for verification. A general inspection of the skin was made for the presence of KP; the lower legs were also examined for dry scaly skin and ichthyosiform skin changes. RESULTS: KP was present in 33 examinees (16%). Factors significantly associated with were dry scaly skin (p < 0.001, odds ratio, OR = 31.3, with 95% confidence interval, CI, 6.4-153.7), BMI >25 (p < 0.001, OR = 4.9, with 95% CI 2.2-11.2) and atopy (p = 0.001, OR = 4.5, with 95% CI 1.8-11.1). Conclusion: It therefore appears that KP is associated with multiple factors, including high BMI, leg skin dryness and atopic conditions.     

秃发性毛发角化症

报告1例秃发性毛发角化症。患者女,22岁。因全身泛发毛囊性红色丘疹8年,头皮瘢痕性秃发伴瘙痒4年就诊。临床表现为头部毛发稀疏,部分秃发区可见毛囊萎缩。面颊部毛细血管扩张,密集毛囊性粉刺样丘疹,四肢、躯干泛发粟米大毛囊性丘疹。枕部头皮组织病理检查：表皮角化过度,表皮突局灶性延长,毛囊口有角质栓,毛囊内外根鞘灶性萎缩或消失,局部多核巨细胞反应性增生,毛囊周围及真皮血管周围见较多淋巴细胞浸润。诊断：秃发性毛发角化症。     

Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum‐like papillary dermal elastolysis?

There are numerous acquired elastic tissue disorders, several of which present cutaneously with small yellow-to-white papules resembling plucked chicken skin. Differential diagnoses depend on the abnormalities within the network of elastic tissues. We report a case with distinct histologic features, which may represent a unique elastic tissue disorder or a variant of pseudoxanthoma elasticum-like papillary dermal elastolysis. Our patient's clinical presentation includes scattered 1–2 mm white-to-yellow papules without surface change on the upper back and neck region. Histology is characterized by foci of clumped, granular elastic tissue, which have replaced the oxytalan and elaunin fibers, alternating with foci of decreased concentrations of normal-appearing elastic fibers within the papillary dermis. Given its characteristics, we have termed this novel entity 'papillary dermal elastosis'.     

Trichostasis Spinulosa

Abstract: Trichostasis spinulosa is a common disorder of follicular hyperkeratosis that is often confused clinically with similar disorders, such as keratosis pilaris and eruptive vellus hair cysts. Six patients from the UTMB dermatology clinic who had trichostasis spinulosa are presented. Two of the six also had keratosis pilaris and one had eruptive vellus hair cysts. The present study was undertaken to compare and contrast the clinical presentation and histopathologic appearance of these three disorders. The results of the study and review of the literature revealed differences in distribution of lesions and microscopic appearance of follicular and histopathologic material.