Catherine S. Yang

Review of thalidomide use in the pediatric population.

Thalidomide is resurging in the management of adult rheumatologic skin conditions, especially lupus erythematosus. Although use in pediatric patients is reported since the 1990s, there are no systematic reviews describing treatment in children. Thalidomide has immunomodulatory and anti-tumor necrosis factor-α effects as well as antiangiogenic properties, making it useful for a broad spectrum of inflammatory disorders. Thalidomide is second-line treatment for aphthous stomatitis and chronic graft-versus-host disease in children and has been prescribed for many other conditions including actinic prurigo and epidermolysis bullosa pruriginosa. Systemic lupus erythematosus may be less responsive to thalidomide in children than adults. Peripheral neuropathy is observed in both idiosyncratic and dose-dependent relationships; children older than 12 years may be more susceptible to developing this adverse effect than younger patients. There are rare reports of thrombotic complications in children treated for nonmalignant indications. We review the mechanism of action and propose that thalidomide is an alternative treatment for patients who fail or have contraindications to anti-tumor necrosis factor-α biologics.

Aminolevulinic Acid Photodynamic Therapy in the Treatment of Erosive Pustular Dermatosis of the Scalp: A Case Series

IMPORTANCE Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory skin condition that develops on sun-damaged skin in older individuals. Patients with EPDS present a therapeutic challenge because medical treatments and surgical modalities have met with limited success. Methyl aminolevulinate photodynamic therapy has been effective in 1 case but induced the disease in others. OBSERVATIONS Eight patients with EPDS with mean (range) age 84 (67-93) years underwent gentle curettage of the hyperkeratotic lesions followed by aminolevulinic acid photodynamic therapy (PDT) 1 to 2 weeks later. Lesions resolved in 6 patients, whereas 2 patients had residual lesions at 6-week follow-up and underwent a second cycle of curettage and aminolevulinic acid PDT with resolution. One patient experienced a partial recurrence 5 months after the procedure and was successfully retreated with curettage + aminolevulinic acid PDT. No adverse effects were noted, and patients were satisfied with the treatment. CONCLUSIONS AND RELEVANCE This series of patients demonstrated EPDS successfully treated with PDT. Lesions resolved in all patients with a protocol that included curettage followed by aminolevulinic acid PDT. Therefore, this protocol represents an efficacious modality for EPDS.

Severe, ulcerative, lichenoid mucositis associated with secukinumab

Secukinumab is a new human monoclonal antibody targeting interleukin (IL)-17A, a cytokine involved in the pathogenesis of psoriasis. The US Food and Drug Administration approved secukinumab for psoriasis in 2015. Because the medication has been on the market for a short time, adverse events involving the oral mucosa are rarely reported. We report a case of severe, ulcerative, lichenoid mucositis associated with secukinumab use.

Lindsay’s Nails in Chronic Kidney Disease

A 59-year-old woman who had undergone kidney transplantation 16 years earlier presented with an incidental finding of nail changes that had been present for at least 3 years. She was undergoing treatment with prednisone, mycophenolate mofetil, and tacrolimus for immunosuppression.

Inflammatory and glandular skin disease in pregnancy

A switch from cell-mediated to humoral immunity (helper T 1 [Th1] to helper T 2 [Th2] shift) during gestation plays a key role in placental immune tolerance. As a result, skin diseases that are Th2 mediated often worsen, whereas skin diseases that are Th1 mediated often improve during gestation. Also, due to fluctuations in glandular activity, skin diseases involving sebaceous and eccrine glands may flare, whereas those involving apocrine glands may improve during pregnancy. Despite these trends, inflammatory and glandular skin diseases do not always follow the predicted pattern, and courses are often diverse. We review the gestational course of inflammatory skin diseases, such as atopic dermatitis (atopic eruption of pregnancy), psoriasis, impetigo herpetiformis, urticaria, erythema annulare centrifugum, pityriasis rosea, sarcoidosis, Sweet syndrome, and erythema nodosum, as well as glandular skin diseases, including acne vulgaris, acne rosacea, perioral dermatitis, hidradenitis suppurativa, Fox-Fordyce disease, hyperhidrosis, and miliaria. For each of these diseases, we discuss the pathogenesis, clinical presentation, and management with special consideration for maternal and fetal safety.

Linear IgA bullous dermatosis associated with metastatic renal cell carcinoma

Linear IgA bullous dermatosis (LABD) is a rare disorder characterized by tense bullae arranged annularly on the trunk and extremities. LABD is usually idiopathic or associated with medications, classically vancomycin and nonsteroidal anti-inflammatory drugs. Rarely, LABD has been reported in association with lymphoproliferative disorders, sarcomas, and carcinomas of solid organs, although it is controversial whether this is a causative association.

Dermal eosinophilic infiltrate in junctional epidermolysis bullosa

Junctional epidermolysis bullosa (JEB) is a rare genodermatosis characterized by a split in the lamina lucida usually because of mutations in LAMA3, LAMB3 and LAMC2 resulting in absence or reduction of laminin‐332. Rare subtypes of JEB have mutations in COL17A1, ITGB4, ITGA6 and ITGA3 leading to reduction or dysfunction of collagen XVII, integrin α6β4 and integrin α3. The classic finding under light microscopy is a paucicellular, subepidermal split. We describe the unusual presence of an eosinophilic infiltrate in the bullae and subjacent dermis in a neonate with JEB, generalized intermediate (formerly known as non‐Herlitz‐type JEB), discuss the histologic differential diagnosis for a subepidermal blister in a neonate, review the literature regarding cases of epidermolysis bullosa (EB) presenting with inflammatory infiltrates, and discuss mechanisms to explain these findings. This case highlights that eosinophils can rarely be seen in EB and should not mislead the dermatopathologist into diagnosing an autoimmune blistering disorder.

Should self-destructive behavior affect a patient's access to scarce medical resources?

A 35-year-old man with moderate plaque psoriasis and psoriatic arthritis that has been well controlled on methotrexate (MTX) 20 mg weekly for the last 4 months presents for follow-up. He recently heard about adalimumab, and asks about starting treatment with this drug. He explains that although he has abstained from drinking while taking MTX, he craves alcohol. He had previously been on acitretin for years, during which time he was usually abstinent, but during the transition from acitretin to MTX, he was frequently intoxicated. Today, his skin is clear. He has not had any side effects while on MTX, but he states, ‘‘I want to live the way I want to,’’ including engaging in binge drinking. What is the clinician’s best course of action? A. Agree to switch the patient to adalimumab because he wishes to freely drink alcohol and have less laboratory monitoring. B. Continue MTX because it has controlled his disease and has not caused any adverse effects. C. Advise the patient that he can safely drink less than 100 g of alcohol per week while taking MTX, but will still need laboratory monitoring. D. Tell the patient that you cannot continue treating him unless he enrolls in an alcohol treatment program.