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.

Surgical Management of Cicatricial Ectropion in Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris is an idiopathic, papulosquamous dermatological disease. It is clinically and histologically distinct from, but may resemble, psoriasis. Pityriasis rubra pilaris can be self-limiting but may also run a protracted, relapsing course. Medical treatment may involve topical emollients, systemic retinoids, methotrexate, and/or tumor necrosis factor antagonists. Ocular complications include cicatricial ectropion. The authors describe the surgical management of 3 patients with cicatricial ectropion secondary to pityriasis rubra pilaris. All patients had procedures involving skin grafts; 1 patient required multiple operations. The management principles are discussed, including the role and timing of surgery, within the context of emerging treatments for pityriasis rubra pilaris. Patients with pityriasis rubra pilaris and cicatricial ectropion should be closely managed by both ophthalmologist and dermatologist. The eyelid position may improve with conservative or surgical measures. If surgery is required, the limitations of skin grafting under these circumstances should be anticipated and patients counseled appropriately.

Post-infectious pityriasis rubra pilaris in pediatric patients. / Pitiriasis rubra pilaris posinfecciosa en pacientes pediátricos.

Pityriasis rubra pilaris (PRP) is a rare skin condition. The etiology of PRP is unknown; however, it has been associated with infections, autoimmune diseases, and neoplasms. Here we describe the cases of 2 pediatric patients with PRP triggered by a respiratory syncytial virus infection concurrently with obstructive bronchial syndrome. PRP resolved after treatment with topical emollients, topical corticosteroids, and calcineurin inhibitors.

La pitiriasis rubra pilaris (PRP) es una enfermedad dermatológica poco frecuente. Se desconoce su etiología, sin embargo, se ha asociado a infecciones, enfermedades autoinmunes y neoplasias. Se describen los casos de dos pacientes pediátricos que presentaron PRP gatillada por una infección por virus sincicial respiratorio mientras cursaban un síndrome bronquial obstructivo. Los cuadros de PRP remitieron luego del tratamiento tópico con emolientes, corticoesteroides tópicos e inhibidores de la calcineurina.

New-onset vitiligo during long-term, stable infliximab treatment of pityriasis rubra pilaris.

Incidents of new-onset vitiligo attributed to infliximab therapy for rheumatoid arthritis and ulcerative colitis have been reported. Reported cases share a common theme in that symptoms manifested in close proximity to the initiation or significant dose increase of the medication. This case describes the presentation of infliximab-induced vitiligo in a patient using it for long-term treatment of stable pityriasis rubra pilaris. The patient was initiated and titrated to a stable dose of infliximab totaling 27 months' duration. He was able to achieve near-complete resolution of symptoms before developing depigmented patches consistent with vitiligo. Infliximab was discontinued. Tacrolimus 0.1% ointment and narrow-band ultraviolet B light successfully repigmented the patches. The association of discontinuing infliximab and resolution of vitiligo suggests infliximab had a role in this case. Though the mechanism of involvement is undetermined, infliximab may have induced an autoimmune process by paradoxically activating lymphocytes. Alternatively, infliximab antibodies may have led to the process by disrupting the normal balance of cytokines.

A case of pityriasis rubra pilaris associated with membranous nephropathy.

Pityriasis rubra pilaris (PRP) is a rare idiopathic dermatosis which may be associated with autoimmune diseases, HIV infection, and internal malignancies. Its association with renal diseases is, however, much less recognized. We report a case of PRP with associated membranous nephropathy (MN), which resolved spontaneously with resolution of the dermatosis. This is only the second reported association between PRP and MN of which we are aware. Further reports of such an association will strengthen the evidence for the two conditions being linked and may thereby shed light on the pathogenesis of both PRP and MN.

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.

Erythema gyratum repens associated with pityriasis rubra pilaris.

Erythema gyratum repens (EGR) is a rare cutaneous eruption characterized by serpiginous morphology and a migrating scaly border. It is one of the most specific cutaneous paraneoplastic phenomena, and is associated with malignancy in most cases. We report a 46-year-old Afro-Caribbean man with the unequivocal clinical and histological features of pityriasis rubra pilaris (PRP). However, despite improvement on oral acitretin, the morphology of the eruption evolved into the striking serpiginous rash of EGR. The histology findings, although nonspecific, were in keeping with the diagnosis of EGR. No evidence of malignancy was found. Only four cases of PRP evolving into EGR have been reported in the literature, and none was associated with malignancy. All previously reported cases of EGR have been described in white patients, making our case the first reported exception, to our knowledge. The possible role of retinoids in altering the rash of PRP to that of EGR is discussed.