Body site distribution of pediatric-onset morphea and association with extracutaneous manifestations.

BACKGROUND: The distribution of pediatric-onset morphea and site-based likelihood for extracutaneous complications has not been well characterized. OBJECTIVE: To characterize the lesional distribution of pediatric-onset morphea and to determine the sites with the highest association of extracutaneous manifestations. METHODS: A retrospective cross-sectional study was performed. Using clinical photographs, morphea lesions were mapped onto body diagrams using customized software. RESULTS: A total of 823 patients with 2522 lesions were included. Lesions were more frequent on the superior (vs inferior) anterior aspect of the head and extensor (vs flexor) extremities. Linear morphea lesions were more likely on the head and neck, whereas plaque and generalized morphea lesions were more likely on the trunk. Musculoskeletal complications were more likely with lesions on the extensor (vs flexor) extremity (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.4), whereas neurologic manifestations were more likely with lesions on the anterior (vs posterior) (OR, 2.8; 95% CI, 1.7-4.6) and superior (vs inferior) aspect of the head (OR, 2.3; 95% CI, 1.6-3.4). LIMITATIONS: Retrospective nature and the inclusion of only patients with clinical photographs. CONCLUSION: The distribution of pediatric-onset morphea is not random and varies with body site and within individual body sites. The risk stratification of extracutaneous manifestations by body site may inform decisions about screening for extracutaneous manifestations, although prospective studies are needed.

WNT10A Mutation Causes Ectodermal Dysplasia in a Patient Mosaic for Turner Syndrome.

WNT10A plays a role in the proper proliferation and differentiation of ectodermal structures. Mutations in this gene can be responsible for a highly phenotypically variable range of disorders termed ectodermal dysplasias. Here, we describe the case of a five-year-old male patient who is mosaic for Turner syndrome (45,X [90%]/46,X isodicentric Y [10%]) and who presented to dermatology with anhidrosis, conical-shaped teeth, and a slowed rate of hair growth with genetic testing subsequently revealing a likely pathogenic heterozygous variant in WNT10A (c.682T>A; p.Phe228Ile). Future investigation into the WNT10A pathway, which is regulated downstream by ß-catenin, might allow topical therapeutics to be developed that promote normal ectodermal growth and differentiation. Current management for this patient includes precautions taken to prevent overheating and heat stroke and close dermatological and dental monitoring.

Cutis marmorata telangiectatica congenita successfully treated with intense pulsed light and pulse dyed laser therapy: a case report.

Cutis marmorata telangiectatica congenita (CMTC) is a rare vascular disorder characterized by blue-purple reticulated patches and plaques that can be localized or generalized. Associated skin atrophy and soft tissue hypoplasia is common while ulceration is relatively uncommon. As CMTC is exceedingly rare and spontaneous remission in childhood can occur in mild cases, evidence for treatment of severe, refractory disease is limited. We present the case of a four-year-old female with CMTC and associated painful, recalcitrant ulcers successfully treated with a combination of pulsed dye laser and intense pulsed light therapy.

Interferon-alpha induced psoriasis in a teenager.

Exacerbation of preexisting and development of new-onset psoriasis have been reported secondary to interferon treatment and, more recently, with newer biologic agents. We report a case of interferon-induced psoriasis in an adolescent with hepatitis C, a rarely reported medication reaction in children. This case highlights the importance of reviewing a patient's medication list when evaluating cutaneous disorders.

An elderly Filipino woman with follicular papules of the face and chest.

An elderly woman developed pruritic follicularpapules over a period of 5 years. A biopsy revealedfocal intraepidermal eosinophilic pustules, leading tothe diagnosis of Ofuji disease or eosinophilic pustularfolliculitis. A discussion of this condition is presented.

Pediatric disorders of pigmentation commonly seen in skin of color.

There are certain disorders of hyper- and hypopigmentation that are more common in children with skin of color. It is imperative that practitioners can swiftly and accurately diagnose these pigmentary disorders to prevent delay in treatment and enhance quality of life. Pigmentary disorders have many etiologies and may present similarly in the clinical setting; however treatment varies widely and is dependent on accurate diagnosis. Herein, we present a review of common disorders of hyperpigmentation and hypopigmentation in children with skin of color. We aim to provide physicians with information that can enhance clinical detection of common pigmentary disorders in this vulnerable population.

Pediatric patch testing: A 10-year retrospective study.

BACKGROUND: Patch testing is the gold standard for diagnosing allergic contact dermatitis in children. OBJECTIVE: To present a 10-year retrospective review of pediatric patch testing at our institution. METHODS: Data from patients 18 years or younger who presented for patch testing from February 1, 2005, to May 31, 2015, were entered into a database. Patch testing was performed according to North American Contact Dermatitis Group standards. A positive reaction was defined as any allergen for which the patient had a 1+ or greater reaction and provided avoidance information. RESULTS: A total of 157 patients with a mean (SD) age of 12.3 (4.1) years were evaluated. The 2 most frequent allergens were nickel and cobalt. Most patients were referred by dermatologists for suspected allergic contact dermatitis (72.3%). Dermatitis was present for more than 2 years in most cases (n = 67 [46.2%]), from 6 months to 2 years (n = 49 [33.8%]), and for less than 6 months (n = 29 [20.0%]). Males were more likely to have a positive reaction to fragrance mix 1 compared with females (P = .02). No significant association was found between age and allergen sensitivity. Those with a history of atopy were more likely to have a positive reaction to cobalt (P = .008) and chromium (P = .03). Sixty patients (38.2%) returned for follow-up, and 37 (60.7%) reported improvement; most (n = 54 [88.5%]) were being treated with topical corticosteroids. CONCLUSION: Our study of pediatric patch testing can provide information regarding common allergens in children, which can enhance patient care. Primary care practitioners may benefit from awareness of patch testing as an alternative to prescribing systemic immunosuppressants.

Tender, necrotic plaques of the glans penis due to calciphylaxis.

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare, but often fatal condition involving vascular calcification that can result in tissue ischemia and cutaneous necrosis. It is most often seen in patients with renal failure among many other occasionally reported etiologies. Below, we present a rare and challenging case of calciphylaxis involving the glans penis and right leg in a man with end stage renal disease on hemodialysis.

Pyoderma gangrenosum: A review

BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon neutrophilic dermatosis typically characterized by painful cutaneous ulceration's with hermorrhagic and purulent crust. PG has several different variants that often delay correct diagnosis. Clinical appearance and histopathology should suggest the diagnosis and exclude other diseases with similar ulceration's. The course of the disease is variable, often chronic, and occasionally severe and progressive. PG is frequently associated with other inflammatory diseases, most often inflammatory bowel disease and rheumatoid arthritis. Management can be challenging, with immunosuppression as the mainstay of treatment.