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PubMed1994

Pityriasis rubra pilaris: a new proposed classification.

Clinical features of pityriasis rubra pilaris (PRP) were classified into four types based on a study of 168 cases. All patients, except one case of type IV, and 12 cases of type II, have salmon-coloured or erythematous thick plaques on the palms and soles, which extended beyond the dorsopalmar and plantar junctions. Twenty-one children and 11 adults presenting with such plaques were classified as type I PRP. Fifty-nine children and 27 adults presenting with circumscribed scaly erythematous patches on the elbows and knees were classified as type II. Twenty children and 16 adults with similar patches involving large areas of the trunk which were not generalized were classified as type III. Four children and 10 adults with exfoliative erythroderma associated with diffuse follicular plugging were classified as type IV. The diagnosis was confirmed by histopathology which showed follicular plugging and focal parakeratosis. However, this was not seen in type I. This new clinical classification facilitates recognition of this type of PRP despite the absence of follicular plugging. Two children, initially classified as type I, showed gradual progression to type II on follow-up, with two adults progressing to type III, and 1 adult to type IV. Similarly, two adults classified as type II, progressed to type III, and 1 adult into type IV. Associated findings were an adult of type II who developed localized pustular psoriasis and another adult who developed ovarian adenocarcinoma.

Adult↗
PubMed1994

Changing forms of juvenile pityriasis rubra pilaris--a case report.

Pityriasis rubra pilaris (PRP) is a rare disease affecting both males and females. The aetiology is unknown, but it has an ill-defined relationship with psoriasis. Within the spectrum of PRP certain disease patterns are recognized, and regarded by many as helpful prognostic indicators. Griffiths has suggested a clinically based classification based on a series of 98 patients seen at St John's Hospital, London between 1950 and 1972 (Table 1). Classical type 1 PRP is an erythematous squamous disorder typically showing follicular hyperkeratosis, perifollicular erythema and sharply demarcated islands of unaffected skin. The palms and soles become hyperkeratotic and often exhibit a characteristic orange hue. Type III PRP is the juvenile counterpart of classical type I PRP. The following case report describes a patient who presented with type III PRP but later went on to develop the type IV or circumscribed, juvenile onset PRP.

Child↗
PubMed1995

Pityriasis rubra pilaris as the initial manifestation of internal neoplasia.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder of unknown aetiology. There are only three cases reported occurring coincident with internal neoplasia. We report a case of this disorder presenting as the initial manifestation of a previously undiagnosed malignancy with metastases in the liver. The primary tumour was not detected. The patient had a complete remission of his PRP with topical steroids alone, but died of the malignancy.

Adult↗
PubMed2004

Classical juvenile pityriasis rubra pilaris in a patient with Down syndrome.

We report a case of classical juvenile pityriasis rubra pilaris (CJPRP) in a 15-year-old boy with Down syndrome. The rash was extensive, severe, accompanied by intense itching and responded to low-dose etretinate. Xerosis and cheilitis were also present, suggesting that keratinization defects associated with Down syndrome may be involved in the pathogenesis of CJPRP. PRP is a rare dermatological disorder and to the best of our knowledge, this is the first reported case of CJPRP in a patient with Down syndrome.

Adolescent↗
PubMed2004

Extracorporeal photochemotherapy for the treatment of exanthematic pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous skin disease of unknown aetiology that has been categorized into five clinical types based on age at onset, cutaneous features and prognosis. We present a patient with chronic exanthematic type II atypical adult PRP, whose skin status was significantly improved with monthly extracorporeal photochemotherapy (ECP). Various therapeutic regimens including narrow-band UV-B, bath PUVA therapy, systemic fumaric acid esters and systemic cyclosporin had failed. Oral retinoids could not be administered due to a type IIa hyperlipoproteinemia with profound hepatic steatosis and elevated liver transaminases. The observed clinical benefit may encourage future clinical studies analysing the effectiveness of ECP in otherwise unresponsive cases of type II PRP.

Adult↗
PubMed2006

Photosensitive pityriasis rubra pilaris.

Exacerbation of pityriasis rubra pilaris (PRP) with ultraviolet exposure is well recognized. However, the initial presentation of PRP in a photoexposed distribution is infrequently reported, and only rarely confirmed with phototesting. We describe such a case of photosensitive PRP. Phototesting revealed exquisite ultraviolet (UV) sensitivity, which was particularly marked to UVB, a feature shared with previously reported cases.

Aged↗
PubMed1987

Pityriasis rosea. An immunologic study.

Some immunologic studies were carried out for 31 patients with pityriasis rosea. Normal levels of serum IgG and IgA but higher levels of IgM were obtained. Serum C3 values were decreased while C4 values were normal. B lymphocytes showed no difference from the controls, whereas T-cell counts were significantly lower in the patients. The migration inhibition indices were higher in the patients than the controls. Direct immunofluorescence tests were negative in the studied three cases. The possible implication of a viral infection in the production of the reported changes was discussed.

Adolescent↗
PubMed2004

The role of human herpes virus 6 and 7 in the pathogenesis of pityriasis rosea.

Pityriasis rosea (PR) is an acute exanthematous inflammatory skin disease in which many epidemiological studies suggest a viral infection in its pathogenesis. Several viruses such as picornaviruses, parvoviruses and herpes viruses were accused as causative agents in PR. Human herpes virus-6 (HHV-6) and HHV-7 are newly discovered members of herpesviridae family. Several diseases including roseola infantum and PR have been linked with HHV-6 and HHV-7 in dermatology. We aimed to investigate the role of HHV-6 and HHV-7 in the pathogenesis of PR. Blood samples from 35 patients with acute PR and 30 healthy volunteers were examined to obtain titres for HHV-6 and HHV-7 antibodies. Anti-HHV-6 and -HHV-7 immunoglobulin (Ig) G antibodies were detected by IFA. The high titres of anti-HHV-6 IgG were determined in four patients and anti-HHV-7 IgG in only two of four patients. In our study, we haven't found any significant relationship between HHV-6, HHV-7 and PR. Our results were supported with the data of several previous studies.

Adolescent↗
PubMed2004

Pityriasis versicolor on the groin mimicking erythrasma.

Pityriasis versicolor (PV) is a widespread dermatomycosis caused by yeasts. Erythrasma is a superficial bacterial skin disease affecting the major folds of the body, particularly the groin. We report the case of a 45-year-old man, affected by PV, exclusively localized in the inguinal folds and in the inner surface of the thighs, characterized by lesions clinically reproducing erythrasma. The authors underline the possibility that PV mimics erythrasma and vice versa, especially in those countries in which both diseases are quite common, and stress the importance of performing a simple mycological examination to avoid gross diagnostic and therapeutic errors.

Antifungal Agents↗
PubMed1997

Pityriasis rubra pilaris and focal acantholytic dyskeratosis.

A 65-year-old man presented with a widespread erythematous maculopapular eruption. Skin biopsy showed spongiosis and focal acantholytic dyskeratosis consistent with Grover's disease. Clinically the eruption evolved to erythroderma with typical features of pityriasis rubra pilaris. On review of the histology, changes consistent with this diagnosis were also present in addition to the acantholytic dyskeratosis.

Acantholysis↗
PubMed2004

Paraneoplastic pityriasis rubra pilaris in association with bronchogenic carcinoma.

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis occasionally reported in association with an underlying malignancy. We report a 61-year-old woman in whom PRP preceded the diagnosis of bronchogenic carcinoma by approximately 11 months. The clinical picture in this case included atypical cutaneous signs and mucous membrane involvement. Curative treatment of the malignancy with radiotherapy led to resolution of the mucocutaneous changes within months. A presentation of PRP, particularly if atypical, or in an older patient, should prompt consideration of an associated internal malignancy.

Carcinoma, Bronchogenic↗
PubMed2006

Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab.

A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this. She was treated with a number of topical and systemic agents with minimal improvement or major side-effects. The patient was then treated with intravenous infliximab 5 mg/kg. She improved dramatically within 2 weeks and was no longer erythrodermic. Five further infusions resulted in additional improvement. Methotrexate was briefly added to the regime, but was ceased owing to nausea. Topical tar and keratolytics were used on the scalp. The patient was left with minimal disease activity and was maintained on emollients.

Abdomen↗
PubMed2004

Atypical pityriasis rosea and Hodgkin's disease.

Pityriasis rosea (PR)-like eruptions have been associated with several neoplasms and drugs. These eruptions may be atypical. To date, the association of Hodgkin's disease with PR-like eruptions has rarely been reported. We report a 37-year-old patient with clinical lesions of PR-like, systemic symptoms and lymphadenopathies, who was subsequently diagnosed with Hodgkin's disease.

Adult↗
PubMed2004

Vesicular pityriasis rosea: response to erythromycin treatment.

Pityriasis rosea (PR) is a relatively common disease although its aetiology has not yet been identified. It occurs worldwide and there is no racial susceptibility factor. It usually affects teenagers and young adults between 10 and 35 years of age. Typical PR is much easier to diagnose than the rare atypical forms. We report a rare case of vesicular PR in a black woman who had vesicular lesions limited to her palms and soles in addition to regular typical lesions. We devised an efficient oral erythromycin treatment for this patient.

Administration, Oral↗
PubMed2004

Circumscribed juvenile pityriasis rubra pilaris.

We report the case of a 20-year-old woman with a 10-year history of circumscribed juvenile-onset pityriasis rubra pilaris (PRP, type IV). Our patient had well-defined keratotic follicular papules on an erythematous base located on the extensor aspects of the extremities and dorsal aspects of the feet but no involvement of the palms and soles. Although most cases of type IV PRP follow a favourable course with spontaneous resolution of the lesions, this case demonstrates that circumscribed juvenile PRP can be more persistent and lasts several years.

Adult↗
PubMed2005

Atypical presentations of pityriasis rosea: case presentations.

Atypical cases of pityriasis rosea (PR) are fairly common and less readily recognized than typical eruptions. We present four patients for whom we believe atypical PR is the most likely diagnosis. A 33-year-old man had purpuric lesions bilaterally on the legs with classical rash on the trunk. A 28-year-old woman had intensely pruritic and urticarial lesions. A 10-year-old girl had hundreds of small papular lesions 1-3 mm in size. A pregnant woman aged 26 had oral haemorrhagic ulcers with classical PR eruption on her trunk. The oral ulcers erupted and remitted at the same time as the generalized eruption. We reviewed the literature and proposed a classification based on rash morphology, rash size, rash distribution, number of lesions, site of lesions, severity of symptoms and course of the eruption. We believe that it is difficult to make a clear division to define typical and atypical PR, and that it is important not to ascribe any unusual or atypical skin eruption with PR unless other dermatoses have been excluded.

Adult↗
PubMed2005

Progressive and extensive hypomelanosis and extensive pityriasis alba: same disease, different names?

We report the cases of five female patients with high skin phototype affected by relapsing, hypochromic, non-scaling macules occurring after the summer on the back and spreading over large areas of skin. Histological features disclosed decreased epidermal melanin. Psoralen plus ultraviolet A (PUVA) treatment proved to be beneficial, but new relapses were noted after stopping treatment. Clinical and histological features were consistent with the diagnosis of 'progressive and extensive hypomelanosis' described by Guillet in persons of mixed racial background. We discuss the differential diagnosis of the latter entity with respect to the other idiopathic acquired primitive hypomelanosis and hypothesize an overlapping with the so-called extensive pityriasis alba (EPA).

Adult↗