Mosaic Neurocutaneous Disorders and Their Causes

Importance Livedoid vasculopathy is a painful, ulcerative condition of the lower extremities for which no established treatment exists. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series. Objective To systematically review the treatment for livedoid vasculopathy and synthesize the available clinical data. Evidence Review A systematic review of the literature using Ovid MEDLINE (covering the period January 1, 1946, through June 9, 2017) and Ovid EMBASE (covering January 1, 1947, through June 9, 2017) databases was performed with a broad and inclusive search strategy along with a subsequent search of the references of retrieved articles. All case series reports published in the English language and in a peer-reviewed journal discussing the treatment for livedoid vasculopathy diagnosis were included. Findings A total of 29 case series reports published in the English language and in a peer-reviewed journal discussed the treatment for livedoid vasculopathy. These reports represented a total of 339 patients, of whom 230 (68%) were female and 69 (20%) were male; sex was not stated for 40 patients. Treatment with anticoagulants, antiplatelets, anabolic steroids, thrombolytics, hyperbaric oxygen, intravenous immunoglobulins, vitamin supplementation, UV light, and a combination of 1 or more of these among other therapies had a favorable outcome. Anticoagulants were the most commonly used monotherapy, achieving a favorable response in 62 of 63 patients (98%). Anabolic steroids, intravenous immunoglobulins, and antiplatelets were the second, third, and fourth most commonly used treatments, respectively. All of these therapies were associated with good clinical outcomes. Adverse events were observed in 44 patients (13%). Conclusions and Relevance A variety of treatments with varying degrees of success have been used to treat livedoid vasculopathy. Randomized clinical trials should be performed in the future to better establish these treatments in clinical practice.

https://jamanetwork.com/journals/jamadermatology/articlepdf/2663279/jamadermatology_micieli_2017_rv_170005.pdf

Treatment for Livedoid Vasculopathy: A Systematic Review.

that CTDs and RP could be important underlying disorders related to multi-MS. The causative pathogenesis of multi-MS in CTDs is unclear. In the present study, multi-MS was seen in association with SjS and SSc, which were frequently accompanied by RP and NFB, which could reflect microvascular damage. Thus, stress caused by ischemia could increase the melanin in the nail matrix. This was a retrospective study with a small number of Japanese patients visiting a university hospital. To determine the real frequency of CTDs in patients with multi-MS, it is necessary to assess a comparable population sample of the same age without other diseases, syndromes, or connective tissue diseases. However, CTDs and RP should receive more attention as underlying disorders associated with multi-MS. It is important to carefully evaluate the skin around the nails by dermoscopic examination and to assess the history of RP when patients with multi-MS are identified.

Combined acitretin and Candida antigen versus either agent alone in the treatment of recalcitrant warts

Background Without clear evidence, selecting among the existing immunotherapeutic options for warts remains challenging. Objective Through network meta-analyses, we aimed to evaluate the comparative efficacy of different intralesional immunotherapeutic modalities. Methods We included randomized controlled trials comparing intralesional immunotherapeutic modalities to cryotherapy, placebo, or imiquimod. All outcomes were presented as odds ratios (ORs) with 95% confidence intervals. Both conventional and network meta-analyses (with a frequentist approach) were conducted on R software. The P-score was used to rank different treatments. Results Network meta-analysis of 17 randomized controlled trials (1676 patients) showed that PPD (purified protein derivative vaccine, OR 39.56), MMR (measles, mumps, rubella vaccine, OR 17.46) and interferon β (OR 15.55) had the highest efficacy in terms of complete recovery at the primary site compared with placebo. Regarding complete recovery at the distant site, autoinoculation (OR 79.95), PPD (OR 42.95), and MMR (OR 15.39) were all statistically superior to placebo. According to the P-score, MMR was more effective than other modalities in reducing the recurrence rate at the same site. Limitations Relatively small sample size in some comparisons and variability in baseline characteristics. Conclusion PPD and MMR were the most effective in achieving complete primary and distant recovery (along with autoinoculation for distant recovery) and reducing the recurrence rate at the same site compared with cryotherapy and other immunotherapeutic modalities.

Intralesional immunotherapy for the treatment of warts: A network meta-analysis.

To estimate the antimicrobial effect of p-hydroxyphenyl acrylate (H5) derivatives on the basis of their molecular structure, the hydroxy and acryl groups of p-hydroxyphenyl acrylate were modified. The antimicrobial activity of the resulting compounds was assessed against a Gram-positive bacterium (Staphylococcus aureus), a Gram-negative bacterium (Pseudomonas aeruginosa), and fungi (Aspergillus fumigatus and Penicillium pinphilum) by the halo zone and the shake flask test. The antimicrobial activity of H5 was ascribed mainly to the acryl group. Compounds with acryl or acryloxy groups bound to the phenyl moiety were found to exhibit particularly high antimicrobial activities. The activities of phenyl acrylate and phenyl vinyl ketone were excellent as compared to aliphatic acrylates such as cyclohexyl acrylate and hexyl acrylate, indicating that the stereoelectronic effect of the phenyl group was important to the antimicrobial activity.

Antimicrobial activity of p-hydroxyphenyl acrylate derivatives.

INTRODUCTION Condyloma Acuminata (CA) is a common viral sexually transmitted disease. Although various treatment modalities are available for treating CA, but none of them can achieve 100% response rate. In a search for better response rate and less recurrence rate, the combination of cytotoxic agent Podophyllin with ablative liquid nitrogen cryotherapy was evaluated over cryotherapy alone. AIM To evaluate the synergistic effect of Podophyllin as a chemotherapeutic adjunct to an ablative therapy of liquid nitrogen cryotherapy versus liquid nitrogen cryotherapy alone in the treatment of CA. MATERIALS AND METHODS Sixty patients with multiple CA were randomly assigned to two groups in the study. Thirty patients in group A received double freeze thaw cycle of 25 seconds of liquid nitrogen cryotherapy. Thirty patients in Group B were subjected to liquid nitrogen cryotherapy in a similar manner followed by application of not more than 0.5 ml of 25% Podophyllin solution. All patients were followed up at 1, 4, 8, 12 and 24 weeks after the treatment to monitor the response to therapy and evaluation for any recurrence. When the number of unresponsive lesions were more than 30% of original lesions at 4 weeks follow-up, then the whole procedure was repeated again. RESULTS The complete response rate and the recurrence rate in the Group B in our study were comparable to Group A as the difference was statistically insignificant. But the differentiating point was that the similar results were obtained in Group B with an average1.2 sessions per patient in comparison to an average of 1.67 sessions per patient in Group A. CONCLUSION Cryotherapy represents a simple, safe and effective regimen for the treatment of multiple CA which in combination with Podophyllin is even more effective as a single session procedure; thereby shortening the treatment regimen.

A Comparative Study of Liquid Nitrogen Cryotherapy as Monotherapy versus in Combination with Podophyllin in the Treatment of Condyloma Acuminata.

Multiple squamous cell carcinoma (SCC) of the skin is an exceedingly rare entity. It has been reported in a few patients of psoriasis treated with oral psoralen therapy and UV radiation, immunodeficiency states, local intramuscular metastasis and cutaneous metastasis from vulvar SCC. We report the case of a 55-year-old man who reported with a painless non-healing warty growth on the lower back persisting for the last 2 years which was excised. Its histopathology proved it to be SCC with HPV changes. On examination, 4 similar lesions were found on the upper back; out of which 1 lesion exhibited marked dysplastic changes on histopathology, but without HPV changes. This case could be either multicentrically developed SCC due to HPV infection or cutaneous metastasis as carcinoma of unknown primary site i.e. metastasis occurring before primary tumor diagnosis.

Multiple cutaneous squamous cell carcinomas: primary carcinomas versus cutaneous metastasis.

Mal de meleda (MdM), a rare autosomal recessive genodermatosis is characterized by erythema and hyperkeratosis of the palms and soles with a sharp demarcation and that progress with age (progrediens) and extend to the dorsal aspects of the hands and feet (transgrediens). It has been associated with various conditions albeit rarely with congenial cataract. Ocular lens and the skin have the same embryological origins. We hereby present this novel case report of Mal de meleda in association with congenital posterior subcapsular cataract which to the best of our knowledge has not been reported from India before.

Mal de meleda with congenital cataract: A novel case report

Hypopigmented macules or guttate leukoderma is a rarely reported association of Darier's disease largely reported in dark colored individuals. So far, the pathogenesis of hypopigmented macules has been only partially explained by varying hypotheses such as post inflammatory phenomenon, an early or subclinical variant of disease, and genetic mosaicism. We describe a case of a 32-year-old male of Indian origin suffering from multigenerational Darier's disease associated with hypopigmented macules over the trunk. Classical warty papules and plaques were present in three generations thus highlighting autosomal dominant inheritance of the disease. The hypopigmented macules were found only in the proband thereby signifying its nonfamilial manner of inheritance.

Inheritance pattern of hypopigmented macules associated with familial Darier's disease

Vulvo-vagino-gingival syndrome was described as a distinctive pattern of erosive plurimucosal lichen planus (LP), and it is a clinical triad of vulval, vaginal, and gingival LP. It can lead to sequelae such as vaginal and urethral stenosis which can have severe implications on the quality of life. We report a case of a 40-year-old female who developed urethral, vaginal, as well as anal stenosis as a result of long-term exclusive mucosal LP involving vulvo-vaginal and anal mucosa along with oral LP without any other cutaneous involvement. This case is being reported because of the rare association of anal LP with vulvo-vagino-gingival syndrome and its gross similarity to lichen sclerosus.

Nidhi Sharma

Papers

A Comparative Study of Liquid Nitrogen Cryotherapy as Monotherapy versus in Combination with Podophyllin in the Treatment of Condyloma Acuminata.

Multiple cutaneous squamous cell carcinomas: primary carcinomas versus cutaneous metastasis.

Mal de meleda with congenital cataract: A novel case report

Inheritance pattern of hypopigmented macules associated with familial Darier's disease

Vulvo-vaginal ano-gingival syndrome: Another variant of mucosal lichen planus.