Case Of Pityriasis Rubra Pilaris Research Articles

Pityriasis rubra pilaris partially responsive to treatment with upadacitinib: A case report

Pityriasis rubra pilaris is a rare inflammatory dermatosis characterized by orange-red confluent plaques, hyperkeratotic follicular papules, palmoplantar keratoderma, and, in some cases, erythroderma. The etiology of pityriasis rubra pilaris is unclear. This condition is often treated with oral retinoids and topical corticosteroids, and more recently, biological agents have become the mainstay of treatment. However, there is a paucity of high-quality evidence on the safety and effectiveness of these agents, and the disease often remains refractory to therapy. Herein, we present a case of pityriasis rubra pilaris with a favorable response to treatment with upadacitinib, a Janus kinase inhibitor, which has not been previously reported in the literature for the management of this condition.

Pityriasis rubra pilaris (Devergie's disease) in a COVID-19 patient

Pityriasis rubra pilaris (Devergie's disease) is an idiopathic, papulosquamous inflammatory dermatosis characterized by progressive erythrodermia. The etiology of the disease is unknown, and most cases occur sporadically. Viral infections, impaired vitamin A metabolism, medications, autoimmune reactions, and malignancies are most common triggering factors. The diagnosis of Devergie's disease is based on the clinical manifestations of the disease and the results of a skin histological examination. The present paper discusses a clinical case of pityriasis rubra pilaris that occurred in a COVID-19, 40-year-old woman. For the purpose of differential diagnosis of pitiriasis with atopic dermatitis, erythrodermic form of psoriasis, generalized form of urticaria, it is necessary to perform immunohistochemical studies to identify the proliferation of immunocompetent cells. Based on the described clinical case, it seems possible to recommend testing for SARS-CoV-2 if a patient has Devergie's disease against the background of fever and intoxication syndrome.

A Case of Pityriasis Rubra Pilaris Successfully Treated with Apremilast

A Case of Pityriasis Rubra Pilaris Successfully Treated with Apremilast

Ptiríase rubra pilar: relato de um caso raro

OBJECTIVES: Report the case of pityriasis rubra pilaris (PRP) in a male patient at the age of 2 years and 10 months. METHODS: A review of the medical record as well as the literature regarding the present conditions, via PubMed database. RESULTS: Male patient, 2 years and 10 months, referred for the first year of appearance of pruritic erythematodescamative annular plaques in lower limbs with dissemination to the trunk, upper limbs, and face, associated with scaling in the plantar region and nail dystrophy. The biopsy presented stratum corneum parakeratotic with hypergranulosis and moderate acanthosis. The diagnosis of pityriasis rubra pilaris was considered conclusive. Isotretinoin and oral antihistamine were prescribed, having complete improvement. CONCLUSIONS: PRP remains a challenging disease, be it on its pathogeny, diagnosis or treatment. Randomized studies would aid on establishing a standardized treatment for PRP, improving the quality of life of patients suffering from this disease.

A clinico-epidemiological study of various keratinization disorders

<p class="abstract"><strong>Background:</strong> There is a vast spectrum of disorders with basic defect in the process of keratinization. There are various associations (genetic, autoimmune and environmental) with different keratinization disorders. The aims and objectives of this study to study the epidemiology, clinical features and associations in various keratinization disorders. </p><p class="abstract"><strong>Methods:</strong> A retrospective observational study of 500 patients was done in a tertiary care center. Detailed history was taken and clinical examination was done. Investigations and skin biopsy were performed when needed.<strong></strong></p><p class="abstract"><strong>Results:</strong> In our study of 500 cases of keratinizing disorders, there were 269 (53.8%) cases of psoriasis, 132 (26.4%) cases of palmoplantar keratoderma, 22 (4%) cases of phrynoderma, 19 (3.8%) cases of ichthyosis, 13 (2.6%) cases of acanthosis nigricans, 11 (2.2%) cases of porokeratosis, 7 (1.4%) cases of Darier’s disease, 3 (0.6%) Cases of pityriasis rubra pilaris, 2 (0.4%) cases each of pachyonychia congenita and erythron keratoderma. The most common age group affected was 51-60 years (19.6%). Males to female ratio was 1.13:1. Chronic plaque psoriasis (43.51%) was the most common variant of psoriasis. Psoriasis vulgaris (75%) was the most common cause of erythroderma. Histopathological findings in all patients whose biopsy was taken was consistent with clinical diagnosis. Non trans gradient (97.75%) was the most common type of palmoplantar keratoderma. Ichthyosis vulgaris (47.38%) was the most common type of ichthyosis.</p><p class="abstract"><strong>Conclusions:</strong> Heredity plays an important role in keratinization disorders. Also, various comorbidities have been associated with different keratinization disorders. Hence, we need to look for these factors while evaluating the patients of keratinization disorders.</p>

Case of pityriasis rubra pilaris with annular pattern as an early manifestation

Case of pityriasis rubra pilaris with annular pattern as an early manifestation

PITIRÍASE RUBRA PILAR

A pitiríase rubra pilar (PRP) é uma doença crônica, papuloescamosa, de etiologia desconhecida. Embora existam casos familiares, a maioria é adquirida. Afeta homens e mulheres na mesma proporção, apresentando incidência bimodal com picos na primeira e quinta décadas de vida. Relatamos um caso clínico de pitiríase rubra pilar na forma clássica do adulto, em um paciente do sexo masculino, 40 anos de idade, que apresentava pápulas eritemato descamativas foliculares crónicas, de quatro anos de evolução, no dorso dos quirodáctilos. Neste estudo aborda-se a evolução clínica da doença, diagnósticos diferenciais, achados histopatológicos e o tratamento com acitretina.

Pityriasis Rubra Pilar and hypothyroidism

Pityriasis Rubra Pilaris (PRP) is a chronic and rare papulosquamous disorder. Treatment of Pityriasis Rubra Pilaris is based on empiric evidence because of several doubts regarding its etiology and also because of its relative rarity, making randomized studies difficult to perform. Some factors suggest that the metabolism of vitamin A is involved in pathogenesis. We report a case of Pityriasis Rubra Pilaris associated with autoimmune hypothyroidism which presented rapid and complete response after thyroid hormone replacement, without any association with other systemic treatment. In literature there are only three other reports of significant improvement of the lesions after hormonal correction. Deficiency of thyroid hormone inhibits the conversion of carotene into vitamin A, which would be responsible for the occurrence of Pityriasis Rubra Pilaris in this patient.

Pityriasis rubra pilaris with the concomitant Leser — Trelat syndrome

The authors describe a rare case of pityriasis rubra pilaris with the concomitant Leser — Trelat syndrome. Taking into consideration contra-indications for UV therapy, Neotigason (acitretin) was prescribed in the dose of 25 mg a day. Nearly absolute regression of eruptions characteristic of pityriasis rubra pilaris and multiple seborrheic keratomas was observed.

A case of pityriasis rubra pilaris with associated focal acantholytic dyskeratosis complicated by Kaposi's varicelliform eruption

The clinical and histopathological diagnosis of pityriasis rubra pilaris (PRP) can be difficult because clinical findings are often subtle in early stages, and microscopic findings can overlap with those of other skin diseases. Focal acantholytic dyskeratosis (FAD) can rarely be seen in PRP and can mimic Darier's disease, Grover's disease or other disorders characterized by these histopathologic features. Kaposi's varicelliform eruption is a widespread infection due to herpes simplex virus (HSV) types 1 and 2, coxsackievirus A16 or vaccinia virus, occurring in a preexisting dermatosis; only one case has been reported in PRP. We report a patient with PRP whose biopsies showed both herpes simplex infection and FAD. A complete understanding of the mechanism behind this eruption evolved gradually, aided in great measure by the histopathologic findings.

Efalizumab treatment of a case of pityriasis rubra pilaris (PRP)

Efalizumab treatment of a case of pityriasis rubra pilaris (PRP)

Resolution of Pityriasis Rubra Pilaris-Induced Cicatricial Ectropion With Systemic Low-Dose Methotrexate

To report a case of pityriasis rubra pilaris (PRP)-induced bilateral lower eyelid cicatricial ectropion that resolved with systemic low-dose methotrexate. Observational case report. A retrospective case review of a patient with cicatricial ectropion caused by PRP. A 82-year-old female presented with bilateral lower eyelid cicatricial ectropion secondary to PRP. The patient did not systemically respond to conventional oral retinoid therapy. She was treated with low-dose methotrexate and experienced dramatic resolution of her signs and symptoms, including complete resolution of her bilateral cicatricial ectropion. Systemic treatment of PRP with low-dose methotrexate may result in successful treatment of cicatricial ectropion without surgery.

A case of pityriasis rubra pilaris associated with rapidly progressive finger joint destruction

A 40-year-old female noticed edema of the lower limbs in March 1995. Nephrotic syndrome due to membranous nephropathy was diagnosed and administration of high-dose corticosteroids resulted in incomplete remission. Progressively enlarging, red scaling skin lesions developed concomitantly from the scalp to the extremities. Pityriasis rubra pilaris (PRP) was diagnosed in 1996 in the Department of Dermatology at Sapporo Medical University hospital. Various treatments proved ineffective. Arthritis of the finger joints developed in July 1999, and proteinuria recurred in April 2000. She was admitted to our department in August 2000. Physical examination on admission revealed marked swelling of both distal interphalangeal (DIP) joints and the right fourth proximal interphalangeal (PIP) joint. Results of testing for antinuclear antibody, rheumatoid factor, and HLA-B27 were all negative. Radiography of the hands revealed destruction of the DIP and PIP joints where MRI indicated the presence of synovitis. Bone scintigraphy demonstrated accumulation in bilateral metatarso-phalangeal joints and the left sacroiliac joint. Arthritis associated with PRP was diagnosed, as both PRP and psoriasis represent keratinizing disorders of the skin and clinical features in the present case resembled those of psoriatic arthritis. Despite administration of high-dose corticosteroids, destruction of finger joints progressed rapidly. Administration of cyclosporine in April 2002 improved arthritic symptoms. Cases of PRP accompanied by arthritis need to be accumulated to allow analysis of the pathogenesis and clinical picture of this association.

Familial Pityriasis Rubra Pilaris

Familial pityriasis rubra pilaris is a rare autosomal dominant skin disorder. Four individuals from one family are described who demonstrate clinical features compatible with a diagnosis of familial pityriasis rubra pilaris. Results of light and electron microscopic, immunocytochemical, and biochemical analysis of skin biopsy specimens from three of these four individuals are presented. All affected individuals demonstrated erythematous scaly skin with follicular prominence and islands of sparing. Inheritance was consistent with an autosomal dominant trait. Light and electron microscopic findings were compatible with those reported in sporadic cases of pityriasis rubra pilaris. Immunocytochemistry showed suprabasal staining with monoclonal antibody AE1. Immunoblot analysis revealed abnormal keratins with K6/16 expression, the possibility of an abnormal K14 or K16, and a 45-kd acidic keratin not normally expressed in epidermis. Because similar biochemical analyses have not been reported previously in other cases of pityriasis rubra pilaris (familial or sporadic), comparisons cannot be made. The observations suggest that the cutaneous abnormality in this family extends beyond clinical and morphological alterations to abnormalities in biochemical markers of epidermal differentiation.

A Case of Pityriasis Rubra Pilaris Associated with Incidental Acantholysis

A Case of Pityriasis Rubra Pilaris Associated with Incidental Acantholysis

Horny Perifollicular Mucinosis

We present a case of pityriasis rubra pilaris (PRP)-like eruption, in association with human immunodeficiency virus (HIV) infection, clinically characterized by follicular papules with some elongated spines and comedo-like lesions. The lesions were located on the back, flanks, and proximal extremities. Orthokeratotic follicular plugs and perifollicular mucinous degeneration were consistently observed on three biopsies. The most characteristic histological features of PRP were absent. To our knowledge, this is the first reported case of this kind. Such a case could suggest an atypical PRP modified by HIV infection. It is also possible that it represents a new entity associated with advanced HIV infection (horny perifollicular mucinosis).

Pityriasis rubra pilaris

In a clinical study, 27 of 31 cases of pityriasis rubra pilaris (PRP) had two or more of the following clinical features: erythroderma, well-confined healthy islands inside the inflammatory areas, keratoderma of the palms and soles, or visible follicular hyperkeratosis. Histological features were: perifollicular parakeratosis in 26 cases, hyperkeratosis in 27 cases; the number of granular layers varied greatly from case to case and within one specimen; acanthosis was eczematous in 20 cases and psoriasiform in 8 cases. Erythroderma in PRP in this study was self-healing, lasting 2-6 months with one exception, but PRP must as a whole be considered a mild, chronic disease lasting on average 5.7 years. Complete recovery occurred in only 8 patients and 5 of these had had an erythrodermic onset. Serum vitamin A level and tolerance test were normal. No immunological aberrations could be found. The HLA phenotype frequencies in PRP patients did not differ significantly from those in a Finnish control population. The autoradiographic study of the epidermal cells showed an increase in the cell production in the epidermis.

Pityriasis rubra pilaris responsive to ascorbic acid.

SUMMARY A case of pityriasis rubra pilaris with concomitant ascorbic acid deficiency is presented in an elderly Negro woman. Vitamin shortage was incidental but treatment with vitamin C yielded favourable results; spontaneous resolution seemed unlikely. Addition of vitamin A in less than optimal dose checked a mild cutaneous relapse and enhanced further the involuting process. Conjoint administration of vitamins A and C may prove ideal in management of pityriasis rubra pilaris. The therapeutic value of ascorbic add in this dermatosis warrants further study.

PITYRIASIS RUBRA PILARIS: A CLINICAL STUDY

DESPITE the fact that pityriasis rubra pilaris was first described almost a hundred years ago, 1 there are still many gaps in knowledge of the clinical course, extracutaneous manifestations and pathogenesis of the disease. Our interest was recently drawn to this subject when we saw, within a relatively short time, 2 patients in whom a diagnosis of pityriasis rubra pilaris had been made, both of whom presented clinical evidence of concomitant neuromuscular disease. We felt, therefore, that a review of a larger group of cases of pityriasis rubra pilaris might shed further light on this interesting observation. This report represents a clinical analysis of 58 cases in which a diagnosis of pityriasis rubra pilaris was made at the Mayo Clinic. The first reported treatment of this disease with vitamins was that of Pettler, 2 who had 2 patients, both of whom had been on vitamin-

A CASE OF PITYRIASIS RUBRA PILARIS (DEVERGIE).

British Journal of DermatologyVolume 36, Issue 8-9 p. 373-374 A CASE OF PITYRIASIS RUBRA PILARIS (DEVERGIE). GEORGE PERNET M.D., GEORGE PERNET M.D.Search for more papers by this author GEORGE PERNET M.D., GEORGE PERNET M.D.Search for more papers by this author First published: August 1924 https://doi.org/10.1111/j.1365-2133.1924.tb09209.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Volume36, Issue8-9August 1924Pages 373-374 RelatedInformation