Redefining Disease Severity with Special Area Involvement and Reflecting on Treatment Patterns in a Real-World Psoriasis Population.

BACKGROUND: The International Psoriasis Council (IPC) recommends an approach that considers body surface area (BSA), involvement in special areas, and treatment history for classifying patients as candidates for topical or systemic treatment. This study aimed to quantify the burden of psoriasis by describing BSA distribution, special area involvement, and treatments in a real-world population. METHODS: This retrospective cohort study included patients with psoriasis from the Optum® deidentified Electronic Health Records database with a BSA value (< 3%, 3-10%, and > 10%) recorded between 1 March 2014 and 1 September 2020. Treatments and special area involvement (face, scalp, palms/soles, nails, genitals) were identified within 90 days of the BSA value and stratified by BSA category. RESULTS: Among eligible patients (N = 5120), mean age was 51.4 years and 49.3% were women. The majority of patients (78.9%) were treated with any topical. Proportions of patients with BSA < 3%, 3-10%, and > 10% were 23.4%, 41.9%, and 34.6%, respectively; proportions with 0, 1, and 2+ special areas were 21.6%, 31.6%, and 45.7%, respectively; and 44.4%, 45.7%, and 45.9% of patients with BSA < 3%, 3-10%, and > 10%, respectively, had 2+ special areas. CONCLUSION: The IPC classification can likely identify many more patients who may benefit from systemic therapy than BSA alone.

Tumor Necrosis Factor-α Inhibitor Use in Psoriasis Patients With a First-degree Relative With Multiple Sclerosis.

Tumor necrosis factor (TNF)-α inhibitors are currently the gold standard for treating moderate to severe plaque psoriasis and other immune-mediated diseases. The presence of previously existing demyelinating disease is amongst the contraindications to their use. However, controversy surrounds the use of TNF-α inhibitors in patients who are more predisposed to developing multiple sclerosis (MS), specifically first-degree relatives of MS patients. In fact, the major guidelines committees' recommendations on this issue by the American Academy of Dermatology, the British Association of Dermatologists, and the European S3-Guidelines are not consistent. The data we present suggest that the number needed to treat is at least an order of magnitude smaller than the number needed to harm across all comparisons of anti-TNF-α agents and first-degree relative relationships. Based on these data, physicians could weigh the treatment options available and work closely with neurological colleagues when prescribing anti-TNF-α therapy in this patient population rather than practicing absolute prohibition of anti-TNF-α agents in patients who have a first-degree relative with MS.

Resident rounds part III: case report: a papulo- nodular eruption with systemic signs and symptoms.

This is a typical presentation of erythema nodosum leprosum in a patient with lepromatous leprosy who recently migrated from Micronesia. The clinical presentation, pathology findings, pathogenesis, and therapeutic options are reviewed here.

The spectrum of oculocutaneous disease: Part I. Infectious, inflammatory, and genetic causes of oculocutaneous disease.

Many skin diseases are associated with ocular findings, emphasizing the need for dermatologists to be fully aware of their presence, and as a result, avoid overlooking conditions with potentially major ocular complications, including blindness. We review important oculocutaneous disease associations with recommendations for the management of the ocular complications and appropriate referral to our ophthalmology colleagues. Part I of this 2-part review focuses on the infectious, inflammatory, and genetic relationships.

Cutaneous porphyrias part II: treatment strategies.

The porphyrias are diverse in pathophysiology, clinical presentation, severity, and prognosis, presenting a diagnostic and therapeutic challenge. Although not easily curable, the dermatological manifestations of these diseases, photosensitivity and associated cutaneous pathology, can be effectively prevented and managed. Sun avoidance is essential, and patient education regarding the irreversibility of photocutaneous damage is a necessary corollary. Beyond preventative measures, the care of fragile, vulnerable skin, and pain management, each of the porphyrias has a limited number of unique additional therapeutic options. Many of the treatments have been published only in small case series or anecdotal reports and do not have well-understood nor proven mechanisms of action. This article presents a comprehensive review of available therapeutic options and long-term management recommendations for the cutaneous porphyrias.

Cutaneous porphyrias part I: epidemiology, pathogenesis, presentation, diagnosis, and histopathology.

The porphyrias are a group of disorders characterized by defects in the heme biosynthesis pathway. Many present with skin findings including photosensitivity, bullae, hypertrichosis, and scarring. Systemic symptoms may include abdominal pain, neuropsychiatric changes, anemia, and liver disease. With advances in DNA analysis, researchers are discovering the underlying genetic causes of the porphyrias, enabling family members to be tested for genetic mutations. Here we present a comprehensive review of porphyria focusing on those with cutaneous manifestations. In Part I, we have included the epidemiology, pathogenesis, presentation, diagnosis, and histopathology. Treatment and management options will be discussed in Part II.

Dowling-Degos disease involving the vulva and back: case report and review of the literature.

Dowling-Degos disease is a rarely encountered pigmentary disorder in which small brown-to-black macules appear in a clustered or reticulated pattern primarily at flexural sites. It usually occurs as an autosomal dominant trait but sporadic cases have also been reported. Dowling-Degos disease is sometimes associated with other cutaneous abnormalities, many of which appear to occur as a result of abnormal follicular development. The histology is distinctive with marked, heavily pigmented, slender, and often branched, elongation of the rete ridges. Dowling-Degos disease is caused by one of several loss-of-function mutations in the keratin 5 gene. Similar mutations are found in patients with Galli-Galli disease and that disorder is now considered to be a subset of Dowling-Degos disease. Medical therapy is ineffective but two patients have responded well to ablative laser therapy. We report a patient with the sporadic form of the disease who developed pigmented macules in the rarely involved sites of the lower back and vulva. Her vulvar lesions were treated with Er:YAG laser ablation.