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.

Traction Alopecia: Clinical and Cultural Patterns.

BACKGROUND: Traction alopecia is common and preventable but frequently overlooked disorder. OBJECTIVE: To evaluate patients with traction alopecia. PATIENTS AND METHODS: This study was conducted at the Dermatology Center, Medical City, Baghdad, Iraq, during the period from November 2005 to October 2019. Demographic features like age, gender, disease duration and special hair styling practices and accessories were recorded. Clinical patterns were studied. RESULTS: Thirty female patients were included in this study. Their ages ranged from 6 to 47 years with mean age ± SD was 15.63 ± 9.806. Twenty-one (70%) were below the age of 16 years. No patient had tightly curled hair. All cases were asymptomatic apart from hair loss. The fringe sign was observed in 27 (90%) of cases. The response to therapy was poor. CONCLUSION: Traction alopecia is an important type of pressure-induced hair loss evident in children and adults with or without curly hair due mainly due to cultural hair styling practices with its frequency apparently increasing in recent years. The fringe sign is common and of diagnostic importance. It is a preventable form of hair loss which can be reversed if diagnosed early; otherwise, permanent scarring alopecia results. It represents a pressure phenomenon evident worldwide in both non-Sub-Saharan lineage and Sub-Saharan lineage individuals.

Autoeczematization: A Strange Id Reaction of the Skin.

Autoeczematization, or id reaction, is a disseminated eczematous reaction that occurs due to a release of antigen(s) after exposure to a primary stimulus, with the eczema spreading to a site distant from the original one. This seemingly perplexing generalization most commonly is associated with stasis dermatitis or tinea pedis, though it may be caused by a wide variety of other disorders. We postulate that T cells are reactive to keratinocyte antigens that are produced during keratinocyte damage, which induce this autoeczematization. Studies with current technology are needed to facilitate further understanding of this phenomenon.

Female pattern hair loss: A comprehensive review.

Female pattern hair loss is a common form of hair loss in women that increases in incidence with age. The etiology is unknown with numerous factors identified that influence its onset. Female pattern hair loss may be viewed as a marker for an increased risk of cardiovascular and metabolic disease. New treatments include microneedling, low-level laser therapy, and autologous fat transfer. This article focuses on the pathophysiology, diagnosis, systemic associations, and current treatments for female pattern hair loss, which is the most common cause of alopecia in women.

Generalized pustular figurate erythema: A newly delineated severe cutaneous drug reaction linked with hydroxychloroquine.

A severe cutaneous drug reaction resembling acute generalized exanthematous pustulosis resulting from ingestion of hydroxychloroquine has been documented. It is distinguishable by its longer incubation period, more varied morphology with initially urticarial and later targetoid and arcuate plaques, recalcitrance to therapy and longer duration. Given the anticipated surge in the use of hydroxychloroquine due to its reported benefits in those with coronavirus disease 2019, specific recognition of this entity is pivotal. We delineate it as generalized pustular figurate erythema.

The kerion: an angry tinea capitis.

Tinea capitis has a high incidence with a global changing pathogen distribution, making this condition a public health concern around the world. As the infection is initially asymptomatic, it is easily spread. Moreover, it is present in many fomites, including hairbrushes, pillows, and bedding. Prompt recognition and treatment is necessary for kerion, an inflammatory subtype characterized by tender boggy plaques with purulent drainage. Kerion is usually associated with infection by zoophilic dermatophytes, although other sources have been described. Treatment for this severe form of dermatophytic infection can be challenging. In addition to the use of topical treatments, oral administration of griseofulvin, terbinafine, itraconazole, or fluconazole is often required. Griseofulvin, the first-line treatment, may not completely eradicate pathogen colonization of the host and may contribute to reinfection and prevalence of infective but asymptomatic carriers. This review highlights new agents that are being evaluated for the treatment of kerion and typical tinea capitis, enhanced diagnostic criteria, and a grading system for kerion evaluation.

Efficacy and tolerability of a new topical nitric-zinc preparation for "difficult-to-treat" warts.

Treating plantar, periungual, and external genital warts can be challenging. A prospective study from four centers in Italy evaluated 37 immunocompetent patients with single or multiple warts and treated each lesion with a nitric-zinc topical solution composed of organic and inorganic acids meant to devitalize tissue and destroy HPV DNA in infected keratinocytes. Thirty of the 37 patients had external genital warts, two had plantar warts, two had palm and finger warts, and three had subungual warts for a total of 55 lesions treated. Nitric-zinc aqueous solution was applied over each wart utilizing a 30 µL capillary tube until a whitening response was observed. Additional applications as needed were accomplished at 2-week intervals until the wart was gone. In those with hand, plantar, and subungual warts, there was a 100% clearance after two to three sessions. Three with external genital warts had only a partial response and one no benefit after four applications. Thus, this approach was effective in external genital and other "difficult-to-treat" warts in 90% of patients after one to four applications. It also was easy to use with no adverse events noted.

Melasma.

Melasma is a common acquired hypermelanosis that primarily affects women of skin type IV-VI. It tends to appear on sun-exposed areas of face and neck. The exact pathogenesis is linked with many factors include ultraviolet radiation, pregnancy, hormonal activity, thyroid abnormalities, and medications. Melasma, which involves increased melanin production and melanocytosis, may be principally epidermal, dermal, or mixed. Mainstays of treatment include topical hypopigmenting agents, lasers, chemical peels, and dermabrasion.

Neonatal and infantile acne vulgaris: an update.

Acne may present in neonates, infants, and small children. Neonatal and infantile acne vulgaris are not considered to be rare. The presentation of acne in this patient population sometimes represents virilization and may portend later development of severe adolescent acne. Neonatal and infantile acne vulgaris must be distinguished from other cutaneous disorders seen in newborns and infants. Infantile acne tends to be more pleomorphic and inflammatory, thus requiring more vigorous therapy than neonatal acne.

Ulerythema ophryogenes: updates and insights.

Ulerythema ophryogenes is a rare cutaneous atrophic disorder that occasionally is associated with Noonan syndrome, de Lange syndrome, Rubinstein-Taybi syndrome, and cardiofaciocutaneous (CFC) syndrome. Often presenting in pediatric patients, the pathogenesis of ulerythema ophryogenes remains unclear, though several genetic causes have been suggested. Treatment recommendations remain anecdotal, but clearance has been noted as the patient ages. Although topical agents have been the mainstay of therapy, recent advancement in laser intervention for treatment of ulerythema ophryogenes is promising.

Lichen planus: an update and review.

Lichen planus (LP) is a papulosquamous eruption of the skin, scalp, nails, and mucous membranes. Although LP is more common in adults, it has become an established pediatric disorder. Its classic presentation is characterized by 4 p's: purple, polygonal, pruritic papules. Histopathologic examination reveals characteristic interface dermatitis. Although its pathogenesis is not fully understood, there is evidence that an imbalance of immunologic cellular reactivity is central. Lichen planus usually resolves within a few months. Treatment that primarily consists of topical and/or oral steroids will expedite recovery and alleviate symptoms. Resolution of this cutaneous disease often is accompanied by postinflammatory hyperpigmentation. Long-term sequelae of LP in the pediatric population are rare, but cutaneous atrophy and pterygium unguis may occur.

Pityriasis alba revisited: perspectives on an enigmatic disorder of childhood.

Pityriasis alba (PA) is a localized hypopigmented disorder of childhood with many existing clinical variants. It is more often detected in individuals with a darker complexion but may occur in individuals of all skin types. Atopy, xerosis, and mineral deficiencies are potential risk factors. Sun exposure exacerbates the contrast between normal and lesional skin, making lesions more visible and patients more likely to seek medical attention. Poor cutaneous hydration appears to be a common theme for most risk factors and may help elucidate the pathogenesis of this disorder. The end result of this mechanism is inappropriate melanosis manifesting as hypopigmentation. It must be differentiated from other disorders of hypopigmentation, such as pityriasis versicolor alba, vitiligo, nevus depigmentosus, and nevus anemicus. Alleviation of the various risk factors via patient education on proper skin care and hygiene, use of lubricants and emollients, topical corticosteroid therapy in the presence of inflammation, and the novel administration of topical anti-inflammatory drugs such as calcineurin inhibitors can play a crucial role in promoting remission or resolution.

Kaposi sarcoma and quinine: a potentially overlooked triggering factor in millions of Africans.

Kaposi sarcoma (KS)-associated herpesvirus, also known as human herpesvirus 8, is necessary but not sufficient for the development of KS. Lytic reactivation of human herpesvirus 8 may be important in KS pathogenesis. KS and its causative agent, KS-associated herpesvirus, have distinctive largely unexplained geographic distributions. We note the recent "oncoweed" hypothesis of biologic plants in the environment accounting for this reactivation. We believe that quinine and its derivatives might better explain the epidemiology of KS than oncoweeds. Indeed, we propose an "oncodrug" hypothesis, specifically with regard to quinine and its derivatives, a linkage first advanced by one of us (V. R.) and associates in 1984.

Trichotillomania: an important psychocutaneous disorder.

Trichotillomania (TTM) is a type of alopecia due to a psychocutaneous disorder, a self-induced illness classified as an impulse control disorder but with features of both obsessive-compulsive disorder (OCD) and addictive disorders. Although most common in children, this repetitive pulling out of one's own hair can occur at any age. The target usually is hair of the scalp, eyebrows, eyelashes, and pubic area using fingers, brushes, combs, and tweezers. Therapy for TTM can be challenging.

X-linked ichthyosis: an oculocutaneous genodermatosis.

X-linked ichthyosis (XLI) is an X-linked recessive disorder of cutaneous keratinization with possible extracutaneous manifestations. It was first described as a distinct type of ichthyosis in 1965. XLI is caused by a deficiency in steroid sulfatase activity, which results in abnormal desquamation and a retention hyperkeratosis. XLI is usually evident during the first few weeks of life as polygonal, loosely adherent translucent scales in a generalized distribution that desquamate widely. These are quickly replaced by large, dark brown, tightly adherent scales occurring primarily symmetrically on the extensor surfaces and the side of the trunk. In addition, extracutaneous manifestations such as corneal opacities, cryptorchidism, and abnormalities related to contiguous gene syndromes may be observed. Diagnosis of XLI is usually made clinically, as the histopathology is nonspecific, but confirmation may be obtained through either biochemical or genetic analysis. Treatment should focus on cutaneous hydration, lubrication, and keratolysis and includes topical moisturizers and topical retinoids.

Erythema toxicum neonatorum revisited.

Erythema toxicum neonatorum (ETN) is a common neonatal dermatologic disorder that usually is evident within the first 48 hours of life. Characteristic lesions include erythema, wheals, papules, and pustules. This transient rash resolves spontaneously without sequelae over the course of a week. Histologically, ETN shows an abundance of eosinophils. Although it has been recognized and described for centuries, the etiology and pathogenesis of ETN remain unclear.

Childhood vitiligo.

Vitiligo is a depigmentation disorder resulting from autoimmune destruction of cutaneous melanocytes. The psychosocial ramifications of this often disfiguring disease can be substantial. Half of the patients with vitiligo experience disease onset in childhood. Although largely similar to the disease in adults, pediatric vitiligo does have differences in epidemiology, associations, and treatment. We review vitiligo as it relates to the pediatric population, emphasizing key differences with adults and the latest treatment advances.

Corrective camouflage in pediatric dermatology.

Many dermatologic diseases, including vitiligo and other pigmentary disorders, vascular malformations, acne, and disfiguring scars from surgery or trauma, can be distressing to pediatric patients and can cause psychological alterations such as depression, loss of self-esteem, deterioration of quality of life, emotional distress, and, in some cases, body dysmorphic disorder. Corrective camouflage can help cover cutaneous unaesthetic disorders using a variety of water-resistant and light to very opaque products that provide effective and natural coverage. These products also can serve as concealers during medical treatment or after surgical procedures before healing is complete. Between May 2001 and July 2003. corrective camouflage was used on 15 children and adolescents (age range, 7-16 years; mean age, 14 years). The majority of patients were girls. Six patients had acne vulgaris; 4 had vitiligo; 2 had Becker nevus; and 1 each had striae distensae, allergic contact dermatitis. and postsurgical scarring. Parents of all patients were satisfied with the cosmetic cover results. We consider corrective makeup to be a well-received and valid adjunctive therapy for use during traditional long-term treatment and as a therapeutic alternative in patients in whom conventional therapy is ineffective.

Nevus spilus.

Nevus spilus (NS), also known as speckled lentiginous nevus (SLN), is a relatively common cutaneous lesion that is characterized by multiple pigmented macules or papules within a pigmented patch. It may be congenital or acquired; however, its etiology remains unknown. NS deserves its own place in the spectrum of classification of important melanocytic nevi; as a lentigo and melanocytic nevus, it has the slight potential to develop into melanoma. Accordingly, we recommend consideration of punch excisions of the speckles alone if excision of the entire NS is declined.