Pityriasis rubra pilaris type IV-associated arthritis treated with adalimumab.

Pityriasis rubra pilaris (PRP) is a very rare disease from the hyperkeratotic papulosquamous disorder group. The rheumatologic manifestations of PRP are extremely rare and less known to clinicians than those of psoriasis. An interesting case of inflammatory arthritis in the hand of an adolescent patient who was successfully treated with adalimumab and compatible with the diagnosis of type IV circumscribed juvenile PRP is presented in the light of the literature.

Identification of a pathogenic CARD14 mutation in a 70-year-old woman with pityriasis rubra pilaris: when genetic diagnosis influences choice of treatment strategy.

Pityriasis rubra pilaris (PRP) is a rare dermatosis characterised by hyperkeratotic follicular papules, orange-red scaly plaques and palmoplantar keratoderma. The aetiology of the disease is in most cases unclear and treatment can be challenging. Familial cases of PRP may result from pathogenic variants in the caspase recruitment domain family member 14 (CARD14). We present a case of lifelong PRP in a 70-year-old woman, where genetic testing revealed a heterozygote missense variant c.412G>A, p.(Glu138Lys) in CARD14. Therapy with ustekinumab was initiated with remarkable effect, which improved the patient's quality of life significantly.

Lichen-planopilaris-like scarring pattern in a patient with alopecia and pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is an erythematous-desquamative dermatitis that is sometimes associated with non-scarring alopecia. Despite the fact that the disease can be disfiguring, scarring alopecia has rarely been described in this disease. Here, we present a 69-year-old woman who developed an erythrodermic episode of PRP associated with telogen effluvium that left an area of persistent alopecia of the scalp and resulted in hair loss in the eyebrows. The biopsy of that area of the scalp demonstrated a scarring alopecia with lichen-planopilaris-like features. Despite this histopathology, the alopecia responded well to treatment. This finding expands the context in which lichen planopilaris features can be found and demonstrates their good prognosis under early treatment.

Treatment of pityriasis rubra pilaris: a case series of 28 patients.

BACKGROUND: Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory dermatosis. Treatment is challenging and mostly based on scarce empirical evidence from large case series. OBJECTIVE: To investigate patient characteristics and therapy outcomes in patients with PRP. MATERIALS & METHODS: In this retrospective study, patients with PRP treated at the Department of Dermatology, University Medical Center Göttingen, between 01/2005 and 10/2018, were analysed according to patient and disease characteristics and treatment experience. RESULTS: PRP was diagnosed in 28 patients with a mean age of 57.3 years at onset; 20 were men (71.4%) and eight women (28.6%). Of all the patients, 89.3% had comorbid diseases; metabolic disorders were particularly frequent. Seventy-eight treatment courses were identified with acitretin as the most common therapy (28.2%). Excellent response was noted in 71.4% of treatment courses with methotrexate, followed by cyclosporine (27.3%) and acitretin (19.0%). Among biologics, ustekinumab (excellent response: 75.0%) and infliximab (50.0%) were particularly effective. CONCLUSION: This study is one of the few comprehensive larger case series of patients with PRP. Acitretin may be effective, particularly for type I PRP, and other retinoids, methotrexate or cyclosporine might also be considered early in the therapeutic algorithm. Moreover, our data adds to the growing evidence that biologics are effective and safe for the treatment of PRP.

The color of skin: orange diseases of the skin, nails, and mucosa.

Cutaneous disease can present with lesions of all colors of the visible spectrum. Lesions of the skin, nail, and mucous membranes with an orange color can be due to a variety of etiologies. The conditions may appear as purely orange, yellow-orange, red-orange, tan, or brown with an orange hue. The orange color may also present as a transient phase of a disease process. As with all dermatologic pathology, a key way to distinguish orange-colored lesions is by distribution and morphology. The sclera, palate, lips, gingiva, and nails may also be involved. A literature review using PubMed with keywords, including orange, skin, mucosa, cutaneous, xanthoderma, and granuloma, was conducted to gather all dermatologic conditions that can present with an orange color. The relevant diseases were categorized by etiology and include inflammatory, infectious, neoplastic, and exogenous causes.