Acne fulminans treatment: case report and literature review.

Acne fulminans (AF), a severe acne variant primarily evident in adolescent males, is characterized by the sudden onset of severe and often ulcerating acne with fever and polyarthritis. A case of a 14-year-old initially treated with clindamycin and surgical debridement, highlights the complexity of AF, including challenges in diagnosis, treatment, and the importance of early dermatological consultation. Successful management was achieved through systemic therapy with retinoids and corticosteroids, resulting in significant improvement. This case underscores the necessity of a coordinated effort among dermatologists, endocrinologists, and rheumatologists for effective AF treatment, illustrating the critical role of timely diagnosis and comprehensive care in managing this rare and challenging condition.

Plasma Cell Mucositis: A Clinical Conundrum.

Plasma cell mucositis (PCM) is an unusual disorder most evident in the accessible mucosa and usually reported in the upper aerodigestive tract, although it is named according to its specific anatomical site of involvement such as plasma cell cheilitis, plasma cell gingivitis, plasma cell vulvitis, and Zoon's balanitis. PCM reflects a dense polyclonal rather than a monoclonal plasma cell proliferation of unclear and unknown etiology. This perplexing disorder tends to be treated by avoiding possible triggers and intralesional and/or systemic steroids. In this work, we provide a review and update on PCM, which often represents a clinical conundrum.

Extramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis.

Extramammary Paget disease (EMPD) is a rare skin cancer of apocrine-rich skin that mimics common inflammatory and infectious dermatoses, leading to delays in diagnosis and increased patient morbidity. Better clinical recognition of this entity, multidisciplinary patient assessment, and deeper understanding of the underlying pathophysiology are essential to improve patient care and disease outcomes. It is important to distinguish primary intraepithelial/micro-invasive EMPD from invasive EMPD or cases with adenocarcinoma arising within EMPD. This 2-part continuing medical education series provides a complete picture of EMPD. Part 1 of this continuing medical education series reviews the epidemiology, oncogenesis, clinical and histopathologic presentation, workup, and prognosis of this rare cancer.

Netherton syndrome-A therapeutic challenge in childhood.

Key Clinical Message: High-dose intravenous immunoglobulin exhibits great potential in the treatment of Netherton syndrome. Abstract: Netherton syndrome (NS) is a rare autosomal recessive genodermatosis (OMIM #256500) characterized by superficial scaling, atopic manifestations, and multisystemic complications. It is caused by loss-of-function mutations in the SPINK5 gene, which encode a key kallikrein protease inhibitor. There are two subtypes of the syndrome that differ in clinical presentation and immune profile: ichthyosiform erythroderma and ichthyosis linearis circumflexa. NS is a multisystemic disease with numerous extracutaneous manifestations. Current therapy for patients with NS is mainly supportive, as there is no curative or specific treatment, especially for children with NS, but targeted therapies are being developed. We describe an 8-year-old boy with genetically proven NS treated with intravenous immunoglobulin for recurrent skin and systemic infections from infancy, growth retardation, and associated erythroderma. Under this therapy, his skin status, infectious exacerbations, and quality of life all improved. Knowledge of the cytokine-mediated pathogenesis of NS and the development of new biologic drugs open new possibilities for NS patients. However, the different therapeutic options have been applied in a limited number of cases, and variable responses have been shown. Randomized controlled trials with a sufficient number of patients stratified and treated according to their specific immune profile and clinical phenotype are needed to evaluate the safety and efficacy of treatment options for patients with NS.

From endometrium to fingernail bed: histological evaluation of a rare cutaneous metastasis.

In the dermatological spectrum of oncologic manifestations, cutaneous metastases from endometrial carcinoma stand as a rarity, given the tumour's predilection for neighbouring uterine regions. We present an exceptional case of a patient in her mid-50s, whereby an endometrial carcinoma, defying conventional pathways, manifested on the skin and nail of her distal fourth finger, an unusual site for cutaneous metastases, with a specific histology of the primary cancer.

Herpes zoster infection in pregnancy: features and consequences.

Herpes (varicella) zoster (HZ) infection occurs in 4 people per 1000 in the general US population (irrespective of prior varicella infection and vaccination status) each year and has been the subject of scientific inquiry for decades. The consequences of infection are myriad and may depend on the dermatome of involvement as well as host factors such as age, comorbidities, prior treatment or immunization, and immunologic status. Pregnancy is associated with an altered immune and hormonal status in the mother. While maternal HZ infection during pregnancy is not uncommon, the implications for both mother and child are not well established, although multiple studies of perinatal maternal HZ infection suggest no intrauterine transmission to the fetus. We review the current literature on herpes zoster infection in pregnancy, including epidemiology, diagnosis, potential immunologic sequelae, and strategies for prevention and treatment.

The role of scoliosis on the comorbidity and demographics of neurofibromatosis type 1 patients: A retrospective analysis of the National Inpatient Sample database.

Neurofibromatosis type 1 (NF1) is the most common neurocutaneous syndrome in the United States, affecting every 1 in 3000 individuals. NF1 occurs due to non-functional mutations in the NF1 gene, which expresses neurofibromin, a protein involved in tumour suppression. As a result, NF1 typically presents with non-cancerous neoplasm masses called neurofibromas across the body. Out of all NF1 abnormalities, the most common skeletal abnormality seen in around 10%-30% of NF1 patients is scoliosis, an improver curvature of the spine. However, there is a lack of research on the effects of scoliosis on demographics and morbidities of NF1 patients. We performed a national analysis to investigate the complex relationship between NF1 and scoliosis on patients' demographics and comorbidities. We conducted a retrospective cross-sectional analysis of the 2017 US National Inpatient Sample database using univariable Chi-square analysis and multivariable binary logistic regression analysis to determine the interplay of NF1 and scoliosis on patients' demographics and comorbidities. Our query resulted in 4635 total NF1 patients, of which 475 (10.25%) had scoliosis and 4160 (89.75%) did not. Demographic analysis showed that NF1 patients with scoliosis were typically younger, female and white compared to NF1 patients without scoliosis. Comorbidity analysis showed that NF1 patients with scoliosis were more likely to develop malignant brain neoplasms, epilepsy, hydrocephalus, pigmentation disorders, hypothyroidism, diabetes with chronic complications and coagulopathy disorders. NF1 patients with scoliosis were less likely to develop congestive heart failure, pulmonary circulation disease, peripheral vascular disease, paralysis, chronic pulmonary disease, lymphoma and psychosis. NF1 patients with scoliosis were predominantly younger, female, white patients. The presence of scoliosis in NF1 patients increases the risks for certain brain neoplasms and disorders but serves a protective effect against some pulmonary and cardiac complications.

Post-COVID-19 neuropsychiatric manifestations: a suggested therapeutic approach to 'long COVID' with azithromycin.

The devastating effects of the coronavirus disease (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may not end when the acute illness has terminated. A subset of COVID-19 patients may have symptoms that persist for months. This condition has been described as 'long COVID'. From a historical perspective, it has been recognized that serious long-term neurological sequelae have been associated with RNA viruses such as influenza viruses and coronaviruses. A potential intervention for early post-COVID-19 neuropsychiatric impairment may be the commonly employed, readily available, reasonably priced macrolide antibiotic, azithromycin. We have observed a favourable clinical response with azithromycin in three patients with neurological symptoms associated with long COVID-19. We recommend considering formal clinical trials using azithromycin for patients with post-COVID-19 infection neurological changes including 'COVID fog' or the more severe neurological symptoms that may later develop.

Revive the Dennie-Morgan Fold: A Forgotten Sign of Atopic Dermatitis in Children.

In 1980, Hanifin and Rajka (1) proposed major and minor diagnostic criteria for atopic dermatitis (AD). Major associations included pruritus, dry skin, and history of atopy. One minor feature included Dennie-Morgan Folds (DMFs), which manifest as secondary creases in the skin underneath the inferior eyelid, usually found in infants (2). In an attempt to refine these criteria, a study evaluating 210 patients with an existing AD diagnosis observed DMF in 84% of AD cases. A pediatric study in Bijapur, India on 174 children under 16 years of age with AD identified DMF to be the most prevalent minor criterion. DMFs were found in 71.8% of the study's population and was followed by palmar hyperlinearity and xerosis in prevalence (2). Although DMF pathophysiology remains unclear, we suggest a theory stemming from nocturnal pruritus (NP). The two leading causes of NP are AD and psoriasis, both of which interfere with the patient's sleep quality. Children with increased NP have greater sleep fragmentation and difficulty waking up in the morning (3). A lack of melatonin rhythmic secretions resulting in circadian misalignment may serve as an intermediary for DMF onset. As more blood perfuses the skin under the eyes, edematous fluid enhances dysregulation of the collagen fibers under the eyelids. NP also manifests as facial touching and rubbing or scratching the eyelids during sleep, which can aggravate tissue surrounding the eye (3). AD affects 15-20% of the pediatric population, whereas food allergies, some life-threatening, are found in up to 30% of children with AD, compared with 0.1-0.6% of children in the general population (2). Identifying DMF in children can facilitate the diagnosis of AD and thus lead to the necessary tests to determine whether conditions associated with AD exist, such as food allergy. DMFs are indicative of an AD diagnosis and can be especially critical for children who have life-threatening food allergies associated with their AD (3). One challenge in using DMF as a marker for AD are its different manifestations across ethnic and racial groups. In a study of 160 children aged 3-11 years in London, England, DMFs were present in 34/69 children classified as "black", regardless of whether the child presented with AD. In children classified as "white", only 11/44 had DMFs regardless of AD diagnosis. When cases of AD were excluded, 25% of the white children and 49% of black children had DMFs (4). In a separate study evaluating the differences in prevalence of AD minor characteristics between African Americans and European Americans, African Americans were more likely to have extensor involvement along with diffuse xerosis, palmar hyperlinearity, and DMFs (5). Furthermore, in individuals with darker skin tones, DMFs may be of greater importance, as other characteristics such as erythema may be harder to recognize (5). Life-threatening food allergies are rare, but represent serious sequelae associated with AD. DMFs can serve as a critically simple, easy-to-identify marker for AD and perhaps identify the condition before the presence of serious sequelae. DMFs may guide the clinician towards inquiring further about other AD-related symptoms, thus improving clinical assessment of a significant pediatric condition.

Plasma Cell Mucositis: A Clinical Conundrum.

Plasma cell mucositis (PCM) is an unusual disorder most evident in the accessible mucosa and usually reported in the upper aerodigestive tract, although it is named according to its specific anatomical site of involvement, such as plasma cell cheilitis, plasma cell gingivitis, plasma cell vulvitis, and Zoon's balanitis. PCM reflects a dense polyclonal, rather than a monoclonal, plasma cell proliferation of unclear and unknown etiology. This perplexing disorder tends to be treated by avoiding possible triggers and intralesional and/or systemic steroids. Herein we review and provide an update on PCM, which often represents a clinical conundrum.

Impétigo: actualización / Impetigo: an update

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Childhood dermatitis in the tropics: with special emphasis on infective dermatitis, a marker for infection with human T-cell leukemia virus-I

Eczema in the tropics is a common problem. Although it is a major cause of discomfort among children worldwide, a warm tropical climate often has important repercussions for children with dermatitis. The original description by Sweet empahasized that interest in tropical eczemas extended to the English, as children of West Indian immigrants in England were affected. Likewise, immigrants may carry these disorders with them to the United States. In Jamaica, a tropical country, the largest and most populous of the English-speaking Caribbena islands, eczema is by far the most common skin disorder seen in children attending dermatology clinics. Reports from other Caribbean islands suggest that this is true for the region as a whole. In 1981, Alabi and La Grenade reported that from 1971 to 1975 eczema accounted for 46.7 percent of skin rashes seen in children at the University Hospital of the West Indies. Review of the period 1988 to 1993 showed that 52 percent of the 601 children who attended the skin clinic for the first time had eczema, confirming the earlier finding. In this latter review, atopic eczema was the most common type of eczema (52 percent), followed by seborrheic eczema (20 percent) and infective dermatitis (10 percent). The remaining 18 percent had a variety of unclassified eczemas including pityriasis alba, discoid eczema, acute vesicular eczema of the hands and/or feet, and hyperkeratotic eczema of the feet.(AU)