Lipschutz ulcers in a 4-month-old girl.

Lipschutz ulcers, or non-sexually acquired genital ulcers, typically occur in older children and young adults. A diagnosis of Lipschutz ulcers can only be made after excluding common infectious and non-infectious causes of mucosal ulcers. Herein, we present the case of a 4-month-old girl with painful ulceration of the labia consistent with Lipschutz ulcers.

Autoimmunity in vitiligo: Therapeutic implications and opportunities.

Vitiligo is an acquired chronic pigmentary disorder affecting the melanocytes, mainly in the skin and mucosae. It occurs due to the dynamic interaction between genetic and environmental factors leading to autoimmune destruction of melanocytes. Defects in melanocyte adhesion and increased oxidative stress further augment the immune response in vitiligo. It is a cosmetically disfiguring condition with a substantial psychological burden. Its autoimmune nature with resultant chronicity, variable responses to therapeutic modalities, and frequent recurrences have further diminished the quality of life. Hence, treatment should aim to provide more extended remission periods, prevent recurrences, provide good cosmetic outcomes and ensure patient satisfaction. These treatment goals seem plausible with the recent progress in our understanding of the complex pathogenic mechanisms underlying vitiligo at a molecular and genetic level. We provide a literature review of the pathogenic mechanisms and the therapies targeting these mechanisms.

Efficacy of NB-UVB in Progressive Versus Non-Progressive Non-Segmental Vitiligo: A Prospective Comparative Study.

INTRODUCTION: Narrow-band (NB) ultraviolet B (UVB) phototherapy has been shown to halt disease progression in vitiligo, but whether there is any difference in the response to NB-UVB seen in patients with progressive vitiligo versus non-progressive vitiligo has not been evaluated. OBJECTIVES: To evaluate the effect of NB-UVB on progressive versus non-progressive non-segmental vitiligo. STUDY DESIGN: Prospective observational comparative study. DURATION: April 2016-November 2017. METHODS: Adult patients having non-segmental vitiligo involving 2-50% body surface area were divided into two subsets; patients developing >5 lesions in the last 1 month or >15 lesions in the last 3 months (progressive vitiligo, Group I) and patients with static disease for the last 6 months (non-progressive vitiligo, Group II). Both groups were treated with NB-UVB for 6 months (26 weeks) cumulatively and its efficacy in halting disease progression, re-pigmentation, side effects and psychosocial impact were evaluated. RESULTS: Nineteen out of 24 patients with progressive vitiligo had arrest of disease progression. Rest five patients developed lesions at a slower pace. Group II had earlier onset of re-pigmentation, while Group I had more NB-UVB fluence (34.73 J/cm2 vs 25.2 J/cm2, P value = 0.034), more time for the fluence to be fixed (P value = 0.001) and more pruritus (P value = 0.001). CONCLUSIONS: NB-UVB has the potential to halt disease progression in some patients with progressive vitiligo; but is associated with more total NB-UVB fluence and time taken for fixing it. Progressive vitiligo patients have more pruritus as compared to patients with non-progressive vitiligo.

Cutaneous Signs in Dermatological Diseases; An Overview.

Cutaneous signs in dermatology are pathognomonic or diagnostic for certain conditions. A knowledge of these signs is a useful skill, which must not be forgotten in the recent trend towards relying on molecular biology and other investigative tools including histological findings to make a diagnosis. The aim of this review is to summarize all cutaneous signs in dermatology in a systematic way that would benefit dermatologists, trainee or experienced, in identifying diseases and sharpening their clinical skills. A keyword search for the terms "cutaneous signs," "sign" AND "dermatology" was conducted through PUBMED, Google, and the major textbooks in dermatology (i.e., Rooks Textbook of Dermatology; Fitzpatrick's Dermatology in General Medicine and Dermatology). References were searched thoroughly for all cutaneous signs described. One hundred and nineteen signs were noted and summarized under the following categories: autoimmune, infective, inflammatory, neoplastic, genetic, trauma/miscellaneous. This review would serve as a good reference for those wanting to improve their clinical acumen in diagnosing dermatological disease.

Validation of the diagnostic criteria for segmental vitiligo.

BACKGROUND: Segmental vitiligo has a different clinical course and prognosis as compared to nonsegmental vitiligo, which necessitates its correct diagnosis. It may be difficult to distinguish segmental vitiligo from the limited or focal types of nonsegmental vitiligo. OBJECTIVE: To validate the previously proposed diagnostic criteria for segmental vitiligo. METHODS: This was a cross-sectional validation study involving patients with limited vitiligo. The diagnostic criteria were used to classify vitiligo lesions as segmental or nonsegmental, and was compared with the experts' diagnosis, which was considered as the "gold standard". RESULTS: The study included 200 patients with 225 vitiligo lesions. As per the diagnostic criteria, 146 vitiligo lesions were classified as segmental and 79 as nonsegmental. The experts classified 147 vitiligo lesions as segmental and 39 as nonsegmental, while the diagnosis either was labeled "unsure" or could not be agreed upon for 39 lesions. As compared with the experts' opinions ("for sure" cases, n = 186), the sensitivity and specificity of the diagnostic criteria was 91.8% (95% confidence interval [CI]: 86.2%-95.7%) and 100% (95% CI: 91%-100%), respectively. The positive predictive value was 100% (95% CI: 97.3-100%), while the negative predictive value was 76.5% (95% CI: 62.5%-87.2%). There was a 93.5% agreement between the clinical criteria and experts' opinions (k = 0.83, P < 0.001). LIMITATION: The diagnostic criteria were compared with the experts' opinion in the absence of an established diagnostic "gold standard". CONCLUSIONS: The proposed diagnostic criteria for segmental vitiligo performed well, and can be used in clinical practice, as well as in research settings.

Follicular Becker's Nevus: A New Clinical Variant.

Becker's nevus, also known as pigmented hairy epidermal nevus, is characteristically described as a unilateral, hairy, light to dark brown macule with sharply outlined but irregular border. The etiopathogenesis of Becker's nevus is still not clearly understood. Perifollicular pigmentation has been described earlier by some authors. But, Becker's nevus presenting exclusively with follicular lesions has not been described. We are reporting a series of patients of Becker's nevus with follicular lesions. The diagnosis in all the patients was made after clinicopathological correlation. Follicular epithelium may hold a significant role in the etiopathogenesis of Becker's nevus.

Xanthoma disseminatum with neurological involvement and optic atrophy: improvement with cladribine.

A 9-year-old boy presented with multiple hyperpigmented papules over flexors with polyuria, polydipsia and progressive loss of vision. Histopathology of papule suggested a diagnosis of non-Langerhans cell histiocytosis and systemic evaluation showed central diabetes insipidus and optic atrophy. With a diagnosis of xanthoma disseminatum with significant neurological involvement, he received cladribine therapy and showed significant improvement in both cutaneous and nervous system lesions.

Unsatisfactory response to sirolimus in Maffucci syndrome-associated spindle cell hemangiomas.

Maffucci syndrome is characterized by multiple benign vascular anomalies and enchondromas present on the distal extremities. Effective treatment options are currently not available for Maffucci syndrome-associated vascular lesions. Sirolimus is a mTOR pathway inhibitor, and has been tried successfully in the treatment of various vascular anomalies. We treated a 23-year-old female with Maffucci syndrome-associated spindle cell hemangiomas with oral sirolimus (2mg/day, 0.04mg/kg/day). There was improvement in pain, but no change in colour or size of the vascular nodules. In view of unsatisfactory response and treatment-related adverse effects (oral aphthae, mild transaminitis), sirolimus was stopped after 6 months.