Intramatricial platelet‐rich plasma therapy: A novel treatment modality in refractory nail disorders

Two patients, one with nail lichen striatus and second with idiopathic trachyonychia were treated with intramatriceal injections of platelet‐rich plasma. A total of 0.1 ml of the plasma solution was injected into the matrix of the involved nails at three weekly intervals. Follow‐up was done at each sitting and thereafter at 16 and 20 weeks. Assessment was done both photographically and by dermoscopy. Case 1 showed marked improvement within 3 weeks and Case 2 showed improvement within 6 weeks. No relapses were seen at 16 and 20 weeks of follow up. Intramatricial PRP is a safe and effective therapeutic modality in nail lichen striatus and idiopathic trachyonychia refractory to other treatment options. Further studies with larger sample size and controls are required to validate the results.     

Childhood Mycosis Fungoides: Experience of 28 Patients and Response to Phototherapy

Mycosis fungoides (MF), the most common cutaneous T‐cell lymphoma (CTCL), is rare in childhood. The prognosis and response to treatment are poorly described in children. The objective of the current study was to evaluate the response to phototherapy in a pediatric cohort. A retrospective cohort study of all patients diagnosed with MF before the age of 18 years and referred to the regional CTCL phototherapy service was performed between January 1990 and April 2012. Twenty‐eight patients were identified (13 boys, 15 girls). The mean age at presentation was 11.6 ± 3.9 years. The hypopigmented variant was noted in 79% of patients. All patients had stage I disease (IA = 10, IB = 17, unknown = 1). The median follow‐up after diagnosis was 43 months (range 6–274 mos). Narrowband ultraviolet B (NbUVB; 311 nm) was used as first‐line phototherapy in 18 patients and psoralen (bath) plus ultraviolet A (PUVA) was used in 8 patients. Complete or partial response was observed in 19 of 22 patients (86%). A further course of phototherapy was required in 7 of 12 patients (58%) treated with NbUVB after a median of 4 months (range 4–29 mos). A further course of phototherapy was required in four of eight patients (50%) successfully treated with PUVA after a median of 45.5 months (range 30–87 mos). No disease progression was noted over the follow‐up (median 43 mos). The majority of patients in our cohort had hypopigmented MF. Phototherapy offers an effective option for treatment of childhood MF, although the period of remission may be greater in patients treated with PUVA.     

Treatment of ingrown nail with a special device composed of shape‐memory alloy

Ingrown nail is a common nail problem resulting in pain and disability in daily life. Recently, a new treatment modality for an ingrown nail was reported that used a device composed of shape‐memory alloy, K‐D. The aim of the present study was to determine the efficacy, recurrence rate and complications of K‐D. Between June 2010 and September 2012, 24 patients (31 nails) underwent treatment of symptomatic incurved nails with a K‐D. Patients were evaluated at pretreatment and during every visit. The mean age of the patients involved was 43.4 years. The mean period of follow up was 161 days. The mean maintenance period was 41 days. The right first toenail was the most common site. Almost ingrown nails healed and the nail deformity was corrected after the procedure. Among the 31 nails, seven of the ingrown nails recurred during follow up (22.6% recurrence rate). The recurrence rate of the patients with stage 1, 2 and 3 ingrown nails was 22.2%, 33.3% and 14.2%, respectively. The majority of patients were very satisfied. There were no side‐effects in most patients except loss of nail in one patient. K‐D has some advantages such as simple application steps, no deformity after the procedure, high patient satisfaction and obvious effect compared to other non‐invasive and invasive methods.     

Disseminated cutaneous Mycobacterium kansasii infection presenting with Rosai–Dorfman disease‐like histological features in a patient carrying anti‐interferon‐γ autoantibodies

Typical cutaneous non‐tuberculous mycobacteria (NTM) infections show a histopathology pattern of granulomas with admixed Langhans giant cells, and abscesses may be observed in acute lesions. Herein, we describe a patient carrying a high titer of autoantibodies to interferon (IFN)‐γ with disseminated Mycobacterium kansasii infection presenting with emperipolesis and Rosai–Dorfman disease (RDD)‐like histopathological features characterized by remarkable, large, pale‐staining “RD cells”, which were CD68 and S100 positive and CD1a negative. The patient was misdiagnosed with RDD initially, but exhibited a poor response to all interventions. A re‐biopsy revealed Langhans‐type multinucleated giant cells; multiple definite acid‐fast bacilli were also found. M. kansasii was isolated from cultured tissues. Anti‐NTM treatment was initiated. After treatment, all lesions resolved almost completely within the following month. High‐titer anti‐IFN‐γ autoantibodies were detected during follow up, leading to the diagnosis of adult‐onset immunodeficiency syndrome. In conclusion, patients carrying high‐titer autoantibodies to IFN‐γ who also have a disseminated cutaneous M. kansasii infection may present with RDD‐like histopathological features, which may be a pitfall in the diagnosis of disseminated cutaneous NTM infections.     

Observer agreement in toenail disorders: implications for diagnosis and clinical research

Background Toenail disorders are frequent, especially onychomycosis. The interobserver variability of nail signs needs to be known before these signs can be confidently applied for diagnosis. Objectives To describe observer agreement in toenail findings as described by dermatologists in standard clinical practice, focusing on signs that could be useful for diagnosis of onychomycosis. Methods Prospective cross‐sectional study in five dermatology departments. Eighty‐six patients with abnormal toenails that could have onychomycosis as a differential diagnosis were independently examined by a pair of dermatologists using a predefined questionnaire, to describe the presence of 10 findings on previous history and 14 physical signs. Results Agreement was fine for previous history findings: it was very good (κ > 0·81) for previous diagnosis of diabetes, smoking and use of public dressing rooms or swimming pools. Agreement was good (κ 0·61–0·80) for immune suppression (drugs or cancer), previous diagnosis of fungal disease and worsening in the last year. It was moderate (κ 0·41–0·60) for previous diagnosis of arterial disease, trauma induced by work or sports, and distal vs. proximal or lateral vs. central start of the lesion. Agreement was worse for physical signs: we found good agreement for the presence of the same disease in fingernails, abnormal plantar desquamation, deformity causing nail trauma, and subungual hyperkeratosis. It was moderate for the presence of nail destruction, tinea interdigitalis, onycholysis, and the type of material obtained by subungual curettage (dust vs. hard). Agreement was fair (κ 0·21–0·40) for the presence of longitudinal or transverse striae, trachyonychia, pachyonychia, and change in colour of the nail plate. Pitting was too infrequent to allow for κ calculation. Chance expected agreement was between 51% and 84% for all signs except pitting. Conclusions Agreement is adequate for most signs. It is low for the presence of longitudinal or transverse striae, trachyonychia, and change in colour of the nail plate. Pitting is rare in toenails.     

Spontaneous Regression of a Nail Matrix Melanocytic Nevus in a Child

A 14‐year‐old Hispanic boy presented with a 0.25‐mm wide, sharply demarcated, dark brown band of longitudinal melanonychia of the left thumbnail. A clinical diagnosis of nail matrix nevus was made and the boy was scheduled for follow‐up. The band showed proximal fading after 6 months and had completely faded after 11 months, with the proximal nail fold showing small dark brown dots on dermoscopy. We documented the spontaneous regression of melanonychia over 11 months.     

Clinical Manifestations of Pediatric Psoriasis: Results of a Multicenter Study in the United States

The clinical features of pediatric psoriasis warrant further attention. A national study was conducted to determine the prevalence of scalp and nail involvement and a history of guttate psoriasis at onset according to age, sex, and disease severity. One hundred eighty‐one children ages 5 to 17 years with plaque psoriasis were enrolled in a multicenter, cross‐sectional study. Subjects and guardians were asked about a history of scalp and nail involvement and whether the initial presentation was guttate. Peak psoriasis severity was assessed and defined historically as mild psoriasis (MP) or severe psoriasis (SP) according to the Physician's Global Assessment and body surface area measures. One hundred forty‐three (79.0%) subjects reported a history of scalp involvement, and 71 (39.2%) described a history of nail involvement. Boys were less likely than girls to report a history of scalp involvement (odds ratio [OR] = 0.40, 95% confidence interval [CI] = 0.19–0.84) but more likely to have had nail involvement (OR = 3.01, 95% CI = 1.62–5.60). Scalp and nail involvement was not related to psoriasis severity. In contrast, subjects with SP (35.9%) more often reported a history of guttate lesions than did those with MP (21.8%) (p = .02). Antecedent streptococcal infection was more common in children with guttate than those with plaque psoriasis at onset (p = .02) but did not correlate with severity. Sex‐related differences in scalp and nail involvement suggest koebnerization. Preceding streptococcal infection predicts guttate morphology but not severity, and initial guttate morphology is associated with eventual greater severity of disease. More aggressive monitoring and management should be considered for guttate psoriasis, given its later association with more severe disease.     

Effects of non‐amputative wide local excision on the local control and prognosis of in situ and invasive subungual melanoma

Subungual melanomas (SUM) are rare, and amputation is often required. Non‐amputative wide local excision (WLE) of the nail unit with the periosteum of the distal phalanx, followed by skin graft, has been accepted for in situ or SUM of 0.5 mm or less thickness. However, previous reports have included a limited number of cases, and not all more than 0.5‐mm thick SUM exhibit invasion or attachment to the distal phalanx. The aim of the present study was to investigate the local recurrence and prognosis for in situ, minimally invasive and invasive SUM that were treated using WLE. We retrospectively reviewed 50 patients with in situ (n = 48) or minimally invasive SUM (n = 2) (in situ or minimally invasive group) and 12 patients with more than 0.5‐mm thick invasive SUM (invasive group) who were treated using WLE. All patients survived the follow‐up period (24–207 months), although four patients with in situ SUM experienced local recurrence at the lateral margin and re‐excision was required. In the invasive group, no patients experienced local recurrence, although one patient (8.3%) developed nodal metastasis at 86 months and regional lymph node dissection was required. WLE may provide acceptable local control for in situ and intermediate thickness SUM, without compromising the vital prognosis. However, a larger randomized prospective study with long‐term follow up is required to evaluate adequately the risks associated with a non‐amputative WLE for in situ and invasive SUM.     

Prospective study of topical 5‐aminolevulinic acid photodynamic therapy for the treatment of severe adolescent acne in Chinese patients

Acne vulgaris is one of the most common skin diseases in adolescents. In the present study, we aimed to evaluate the effectiveness and safety of topical 5‐aminolevulinic acid (ALA)‐mediated photodynamic therapy (PDT) for the treatment of severe acne in Chinese adolescent patients. Twenty‐one Chinese adolescent patients aged 12–18 years with Pillsbury III–IV severe facial acne were treated with three courses of ALA‐PDT. A 5% ALA lotion was applied topically for 60 min followed by irradiation with light‐emitting diode light at 633 nm with a light intensity of 75–80 mW/cm² and a light dose of 90–96 J/cm². Clinical assessment was conducted before and after each treatment, and at each follow‐up session. The total effective rates were 85.71%, 90.48%, and 95.23% after the three PDT sessions, and at the 4‐ and 8‐week follow ups, respectively. ALA‐PDT is an effective treatment for severe adolescent acne vulgaris, and is associated with mild and reversible side‐effects.     

Pediatric Dermatofibrosarcoma Protuberans in Madrid,Spain: Multi‐institutional Outcomes

Little is known about the incidence and management of dermatofibrosarcoma protuberans (DFSP) in children. We conducted a retrospective review of all patients younger than 18 years of age treated for DFSP over a period of 11 years (2000–2011) in Madrid, Spain. The sample consisted of 13 children. The average annual incidence of DFSP in the pediatric population corresponded to 1.02 cases per million person‐years (95% confidence interval 0.55, 1.73). Sites of involvement were diverse, with 15.3% of tumors found in acral locations. The median tumor size was 3.5 cm × 3 cm and the median time from apparent onset to diagnosis was 36 months. Histopathologic examination revealed conventional (77.0%), pigmented (15.4%), and myxoid (7.6%) variants. The mitotic index was consistently <5 per 10 high‐power fields. All lesions were removed using surgical excision. One patient developed a local recurrence because of initial affected margins; none developed metastases. The median duration of clinical follow‐up was 70.5 months. This study estimated the average annual incidence rate of DFSP in a population of patients younger than 18 years and reviewed the experience of several hospitals in the management of this tumor.     

Orange‐Brown Chromonychia and Kawasaki Disease: A Possible Novel Association?

A 4‐year‐old girl with clinical and laboratory signs of Kawasaki disease (KD) was hospitalized and given intravenous immunoglobulin plus aspirin therapy, with rapid defervescence and clinical improvement, and was discharged 48 hours after admission. At the time of her follow‐up echocardiography on day 14, orange‐brown pigmentation of the nail beds was noticed and confirmed with dermoscopy. No clear association between KD and orange‐brown chromonychia has been demonstrated, although reports and case series suggest a possible link between these two entities. We suggest that this particular finding might be encompassed in late (subacute) changes of extremities as part of KD diagnostic criteria.     

Pustular Psoriasis in Childhood and Adolescence: A 20‐Year Single‐Center Experience

Pustular psoriasis (PP) is rare in children. The small number of reported cases makes deciding on treatment and follow‐up challenging. The current study was an evaluation of treatment approaches and courses of PP in 18 children diagnosed and followed over a 20‐year period. From 1992 to 2011 we treated 1,447 children with psoriasis, 18 of whom had PP. Follow‐up was 2 to 19 years. At the time of initial manifestation of PP, our patients were 1.5 months to 16 years old. Seven patients had a previous history of psoriasis vulgaris. Three children entered long‐term remission after one pustular attack, 10 developed psoriasis vulgaris, 2 are currently under treatment, and 3 were lost to follow‐up. Treatment with acitretin, cyclosporine, or methotrexate was efficacious and well tolerated. PP is rare, but according to our experience, it has a good prognosis in children.     

Onychomycosis Does Not Always Require Systemic Treatment for Cure: A Trial Using Topical Therapy

Standard teaching dictates that systemic therapy is required for treatment of onychomycosis. It is unknown whether topical antifungal therapy is effective for pediatric nail infections. This prospective, randomized, double‐blind, vehicle‐controlled study was conducted in the Pediatric Dermatology Research Unit at Rady Children's Hospital to determine whether topical antifungal therapy is efficacious for pediatric onychomycosis. Forty patients ages 2 to 16 years with nonmatrix onychomycosis were randomized 1:3 to ciclopirox lacquer or vehicle lacquer. Ciclopirox lacquer or vehicle was applied daily for 32 weeks, with weekly removal of the lacquer and mechanical trimming. Those with poor response were crossed over to active drug at week 12. Thirty‐seven patients completed the 32‐week study, and follow‐up data were collected 1 year after completion of the study from 24 patients. Mycologic cure, effective treatment, and complete cure were assessed, as well as adverse events and effect on quality of life. Mycologic cure was 70% in the treated group and 20% in the vehicle arm (p = 0.03) at week 12. At end of the study (week 32), 77% of treated patients achieved mycologic cure and 71% effective treatment, compared with 22% of the control group. Ninety‐two percent of those who were cured and followed for 1 year remained clear. Topical antifungal lacquer (ciclopirox) can be an effective option for children with nonmatrix onychomycosis. Pediatric onychomycosis does not always require systemic therapy and responds better to topical therapy than does adult disease.     

Sirolimus therapy for kaposiform hemangioendothelioma with long‐term follow‐up

Mammalian target of rapamycin inhibitors have shown promising results in the management of kaposiform hemangioendothelioma (KHE). The purpose of this study was to present our experience involving sirolimus therapy for KHE. A retrospective study was conducted to review the medical documents of 26 patients with KHE who were treated with sirolimus at our hospital between March 2012 and December 2016. Fifteen males and 11 females manifested KHE in infancy with an average age of 2.9 ± 1.8 months. Multiple anatomical sites were involved. Four patients had multifocal lesions, while 22 patients had solitary lesions. Twenty‐five patients had Kasabach–Merritt phenomenon (KMP). Twenty patients completed sirolimus therapy in 28.3 ± 12.5 months. Nineteen KHE lesions reduced to small residuals with platelet counts reaching normal levels 3.7 ± 2.8 weeks after treatment; one KHE lesion had no response to therapy. One patient with multifocal lesions died due to a severe infection, although the patient had previously responded to sirolimus. Five patients remained in treatment and had good responses with normal platelet counts. Nineteen patients with anemia had normal hemoglobin levels after 3.5 ± 1.9 weeks of treatment. Mild side effects were observed. The median follow‐up time was 32 months (26–60 months), with no evidence of recurrences. Sirolimus was shown to be efficacious in the management of KHE with an average course of 28 months. The time‐to‐response was variable, with an average of 1 week. After 4 weeks of treatment, the platelet count and hemoglobin level had normalized. Multifocal KHE with KMP is more severe than solitary KHE.     

Novel treatment using thioglycolic acid for pincer nails

The authors developed a novel treatment using thioglycolic acid (TGA) to chemically soften pincer nails. The objective was to describe a new treatment method for pincer nails by applying TGA to soften the nail and then fix it in the correct position. A total of 104 patients (nine men and 95 women; mean age, 56 years) with 106 pincer nails underwent our treatment. A small hole was made on the markedly incurvated side of the pincer nail, and a super‐elastic wire was inserted into the hole and bent backwards; 5% TGA was then applied for 6–7 h prior to reduction. Favorable reduction was achieved in 66% of patients within 1 day of the procedure, in 30% within 2–4 days and in 4% 5 days or more later. No patient required surgery. No post‐procedure infection, rash, continuous pain or nail cut out was evident. In eight cases, recurrences of nail deformity were observed within the 1‐year follow‐up period. Our novel method, which consists of administrating TGA via a hole in the nail plate, is a useful treatment for pincer nails.     

Phosphaturic mesenchymal tumor,mixed connective tissue type,non‐phosphaturic variant: Report of a case and review of 32 cases from the Japanese published work

Phosphaturic mesenchymal tumor, mixed connective tissue type (PMTMCT) is a rare neoplasm that can cause tumor‐induced osteomalacia due to overproduction of a phosphaturic hormone, fibroblast growth factor 23 (FGF23). We report here a case of subcutaneous PMTMCT, non‐phosphaturic variant, in the sole. We also review 32 Japanese cases of PMTMCT reported in detail. They occurred in 16 men and 15 women (one was unknown), with ages ranging 20–73 years (median, 48). Tumors were found in soft tissue, bone and sinuses in 17, 11 and four, respectively. A history of long‐standing osteomalacia was noted in all cases except two non‐phosphaturic variant cases. Serum FGF23 level was elevated in 11 of 12 cases examined. In terms of follow‐up information, metastases were found in four patients, and two patients died of disease. In conclusion, PMTMCT is histologically a benign lesion; however, there may be rare metastatic and malignant cases. Wider recognition of the histological features of this unique neoplasm would aid its distinction from the large number of mesenchymal tumors for which it may be mistaken and should enable correct diagnosis of tumors with osteomalacia.     

Role of nail bed methotrexate injections in isolated nail psoriasis: conventional drug via an unconventional route

Nail psoriasis can be a debilitating condition; however, in patients with isolated nail involvement, the use of toxic systemic therapies such as methotrexate may not be justified. We report on 4 patients (30 involved nails between them), who were treated with injections of methotrexate (0.1 mL of a 25 mg/mL solution) into the nail bed at 3‐weekly intervals. Mean baseline Nail Psoriasis Severity Index (NAPSI) was 4.77 (range 2–8, cumulative score 143; n = 30); dropping successively at each visit to 2.43 (range 0–4, cumulative score 73; n = 30) at 15 weeks. The decline in mean NAPSI from 4.87 to 2.17 was statistically significant (P < 0.001; Friedman analysis). Reported adverse effects were pain, injection site pigmentation and nail bed haemorrhage. Administration of specific targeted therapy to the nail bed may help manage nail psoriasis effectively.     

Is Phototherapy Useful in the Treatment of Atopic Dermatitis in Asian Children? A 5‐Year Report from Singapore

The goal of the current study was to review a cohort of Asian children with atopic dermatitis (AD) treated using phototherapy at a tertiary dermatologic center in Singapore. We performed a retrospective review of patients younger than 16 years old with a clinical diagnosis of AD treated with phototherapy at the National Skin Center, Singapore, over a 5‐year period from 2004 to 2008. Twenty‐five patients were identified who were ages 7 to 15 years at the time they underwent phototherapy, with equal sex distribution. The duration of disease ranged from 2 to 14 years. Most patients had extensive disease involving more than 70% of their body surface area. Fifteen patients were treated with narrowband ultraviolet B (NBUVB) phototherapy, with 10 (66%) showing improvement of symptoms. The duration of therapy was 3 to 30 months (mean 11 mos), with a mean of 60 treatment sessions (range 12–104). Nine patients were treated with combined ultraviolet A (UVA) and NBUVB phototherapy. Five patients (55%) improved, with four having good response and one having mild improvement. The duration of therapy was 1–6 months (mean 3.6 mos), with an average of 22 treatment sessions (range 10–44). Two patients underwent combined UVA/broad‐band UVB (BBUVB) phototherapy. Both had worsening of disease requiring cessation of treatment. Phototherapy is a useful adjunct in the treatment of moderate to severe AD in children. More than half of the patients treated with NBUVB or combined UVA and NBUVB improved with phototherapy, but the usefulness of combined UVA and BBUVB requires further evaluation.     

Clinical follow‐up study of adult‐onset Still’s disease

Eighteen patients with adult‐onset Still’s disease have been followed up for 3–22 years in our department. Initial manifestations were fever with skin rash in 14 patients, fever, skin rash and sore throat in two, skin rash in one and arthralgia in one. During the follow‐up period, typical skin rash was seen in all patients, of them five patients (29%) revealed atypical skin rash simultaneously. Atypical rash included persistent erythema with pigmentation in two, persistent plaques and papules with linear erythema in two and edema of the eyelids mimicking dermatomyositis in one. Persistent papules and plaques revealed histologically characteristic features, such as dyskeratotic keratinocyte and liquefaction degeneration as well as a sparse superficial dermal infiltrate containing scattered neutrophils. In patients of chronic articular type and polycyclic systemic type, atypical skin rash, lymphadenopathy and hyperferritinemia were noted to be significantly higher than those of monocyclic type. These factors might be prognostic factors of adult‐onset Still’s disease in our study.     

Low‐dose methotrexate treatment for moderate‐to‐severe atopic dermatitis in adults

Background Atopic dermatitis (AD) is a common inflammatory skin disease. Methotrexate (MTX) was suggested as an effective treatment option in cases of moderate‐to‐severe atopic dermatitis. This study assessed the efficacy and safety of treatment with low weekly doses of methotrexate for moderate‐to‐severe AD in adults. Methods Twenty adult patients with moderate‐to‐severe AD were included in this retrospective study. Those patients were unresponsive to topical treatments, antihistamines and at least one of the second‐line treatments. MTX in low weekly doses of 10–25 mg was administered orally or intramuscularly with folic acid supplementation 5 mg per week for at least 8–12 weeks. The response to treatment was evaluated by change in SCORAD (SCORing Atopic Dermatitis), DLQI (Dermatology Quality of Life Index) and the global assessment of the clinical response score. Results After 8–12 weeks of treatment, we observed an objective response in most patients. There were 16 responders and 4 non‐responders. The mean SCORAD and DLQI decreased by 28.65 units (44.3%) and 10.15 units (43.5%), respectively. The first improvement was observed after a period ranging from 2 weeks to 3 months (mean 9.95 w ± 3.17). Treatment was more effective in adult onset AD than in childhood onset. Tolerance of treatment was good. However, nausea and an increase of liver enzymes were observed in 5 patients and 3 of them required a transient discontinuation of MTX. One patient developed peripheral neuropathy, which was resolved several weeks after the discontinuation of MTX. Conclusion MTX seems to be an effective and safe second‐line treatment for patients with moderate‐to‐severe atopic dermatitis. A randomized, controlled study is warranted.