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PubMed2006

Correlation between HLA and pityriasis rosea susceptibility in Brazilian blacks.

OBJECTIVES: The human leucocyte antigen (HLA) has been related to susceptibility factors in several diseases. This study aimed to determine the potential genetic susceptibility of patients with pityriasis rosea (PR) through HLA molecular typing analysis. METHODS: The method of choice was polymerase chain reaction with sequence-specific primers (PCR-SSP) using low-resolution typing kits, with determination of the alleles class I (HLA-A, HLA-B and HLA-C) and class II (HLA-DRB1, DRB3, DRB4, DRB5 and DQB1) performed in 30 Afro-Brazilian PR-diagnosed patients and 45 healthy individuals as the control group (PR-C). RESULTS: Analysis of the HLA typing results showed that the relative risk (RR) of 4.00 [95% confidence interval (95% CI) 1.20-13.28, two-tailed P = 0.018] for allele HLA-DQB1*04 class II, detected in 33.3% of PR patients, was significant. By contrast, in the control group only 11.1% of subjects had that allele. Three out of six B*51 alleles and three out of six B*53 alleles detected in PR patients were found, together with the allele DQB1*04. CONCLUSION: We suggest that alleles DQB1*04 may be involved in the genetic susceptibility of PR based on the significant predominance of those alleles observed in the black PR patients. We also recommend that more studies are conducted on populations of other ethnic origins, preferentially with higher resolution techniques of DNA typing.

Adolescent↗
PubMed2006

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta with fatal outcome.

Ulceronecrotic Mucha-Habermann disease is a severe, febrile form of pityriasis lichenoides et varioliformis acuta. The condition may sometimes have a fatal outcome, especially in elderly patients. In this paper, we are reporting on a 60-year-old male patient who suffered from the eruption of erythematous, haemorrhagic, ulceronecrotic papules accompanied by high temperature. Erosions and ulceronecrotic papules covered over 80% of his body. Three weeks after his admittance to our hospital, clinical signs resembling ileus together with the leucocytosis occurred. The patient was transferred to the department of internal medicine and later to the surgery department. The fulminant course of the disease could not be stopped, and the patient died of severe intestinal and colon gangrene caused by the massive thrombosis of superior mesenteric artery. Up to date, only 23 cases of this severe form of the disease have been reported, and even with the early recognition, fulminant course may lead to death.

Colonic Diseases↗
PubMed2006

The detection of human herpesvirus-8 DNA in plasma and peripheral blood mononuclear cells in adult patients with pityriasis rosea by polymerase chain reaction.

BACKGROUND: Herpesvirus-like particles have been reported to be detectable by electron microscopy in lesional biopsy of patients with pityriasis rosea (PR). We report a study investigating the association of PR with human herpesvirus-8 (HHV-8) infection. METHODS: Our setting is a teaching clinic affiliated to a university. We recruited eight patients aged 28-47 years (mean: 34.5 years) diagnosed with PR during a one-year period. We collected acute blood specimens at presentation and convalescent blood specimens three to four weeks later. We also collected skin scrapings from the herald patch where present and from truncal secondary lesions. RESULTS: We detected HHV-8 DNA by a nested PCR (polymerase chain reaction) targeting, respectively, a 233-bp and a 160-bp fragment of ORF 26. PCR for HHV-8 DNA was negative in the peripheral blood mononuclear cells and plasma of acute and convalescent specimens of all patients, and negative in all skin scrapings. We detected anti-HHV-8 IgG and IgM antibodies by the indirect immunofluorescence. Four patients had IgG antibodies against HHV-8, but with no significant rise of titre. None were positive for anti-HHV-8 IgM antibody. CONCLUSION: We conclude that PR is not associated with HHV-8 infection.

Adult↗
PubMed1986

Pityriasis rubra pilaris in childhood: a long-term study of 29 cases.

Pityriasis rubra pilaris (PRP) is a dermatosis of unknown origin with a limited frequency in childhood (0.2% of our hospitalized pediatric patients). During the last 20 years we have observed 31 new cases; follow-up has been conducted in 29 children. The acute self-resolving form seems to be the most frequent in children, compared to adults. No cases of hereditary PRP have been observed, and no relationship between the severity of PRP and its prognosis has been reported. Since juvenile PRP has a relatively rapid course and a spontaneous resolution (a few months), it seems unnecessary to use potentially harmful drugs. Synthetic retinoids may be employed in patients whose disease is both persistent and disabling.

Adolescent↗
PubMed1987

Childhood-onset pityriasis rubra pilaris with immunologic abnormalities.

We examined a 6-year-old boy with seborrheic dermatitis like redness of the face, scaling of the scalp, and palmoplantar keratoderma. Skin biopsy showed hyperkeratosis, parakeratosis, follicular plugging, and mild upper dermal perifollicular mononuclear inflammatory infiltrate, which are compatible with the diagnosis of pityriasis rubra pilaris. Immunologic studies revealed normal values of peripheral B and T lymphocytes. The spontaneous activity of T suppressor cells was activated and the function of T helper cells was impaired, however.

Child↗
PubMedDate not supplied

Quality of life in children with pityriasis rosea: a prospective case control study.

Our aim was to investigate the effects on quality of life of pityriasis rosea (PR) in children and the concerns of their parents. We recruited all children ages 5-16 years with a diagnosis of PR in a primary care setting over a period of 30 months. Controls were the next child of the same sex and comparable age consulting for atopic dermatitis or for conditions unrelated to the skin. Information on parental concerns was also gathered. Ten children with PR and 20 control subjects were recruited. Total Children's Dermatology Life Quality Index (CDLQI) scores of children with PR (mean 3.50; 95% confidence interval [CI] 2.66-4.34) were significantly lower than those of children with atopic dermatitis (mean 7.70; 95% CI 5.59-9.81). Children with PR had significantly lower scores for symptoms and feelings, personal relationships, and sleep. Total CDLQI scores of children with PR were significantly higher than those of children with no active skin problem (mean 0.30; 95% CI -0.18 to 0.78). Significantly more parents of children with PR had concerns regarding the etiology, infectivity, relapse, and complications or dangers of the disease. We concluded that quality of life (QOL) of children with PR is significantly less affected than that of children with atopic dermatitis and significantly more affected than in children with no active skin problem. The major concerns of parents of children with PR are the etiology, infectivity, relapse, and complications or dangers. These results have important implications on whether active intervention is worthwhile for this disease.

Adolescent↗
PubMed1979

Pityriasis rosea in a patient with epidermolysis bullosa dystrophica.

A patient with clinical and histologic features of epidermolysis bullosa dystrophica-recessive (EBD-R) developed superimposed clinical lesions of pityriasis rosea (PR). Electron microscopy showed distinct "blebbing" of basal cells at the basement membrane zone in clinically normal skin of a non-predilected area, as well as in scarred skin from a predilected area and a lesion of PR. An increased susceptibility of the basement membrane zone to proteolytic enzymes is suggested as a possible hypothesis to explain these observations.

Adult↗
PubMed1996

Pityriasis rubra pilaris with acantholysis.

Two patients developed a papulosquamous eruption in a widespread distribution which progressed with islands of sparing of uninvolved skin characteristic clinically of adult-onset pityriasis rubra pilaris (PRP). Biopsies from both patients showed multiple areas of nonfollicular and follicular suprabasilar and intra-epidermal acantholysis with minimal dyskeratosis. They also showed the usual histologic features of PRP with a thickened orthokeratosis and parakeratosis, a retained and sometimes thickened granular cell layer, and psoriasiform epidermal hyperplasia with a perivascular lymphohistiocytic infiltrate in the superficial dermis. Two previous patients with PRP have been reported with nonfollicular, focal acantholytic dyskeratosis and both were interpreted as most likely representing an incidental finding. We believe the acantholysis in these two cases is related to the disease process, and in our second patient, was helpful in establishing the diagnosis.

Acantholysis↗
PubMed1997

The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis.

Pityriasis rubra pilaris (PRP) is an idiopathic erythematous scaling eruption which can be difficult to distinguish from psoriasis. We explored the clinical features, including background medical illnesses and potential triggers in patients with PRP, and also its histomorphologic spectrum. Patients with PRP were selected by natural language search of dermatopathology databases containing 250,000 cases accessioned over a 3-year period. A detailed medical history was obtained on each patient via office chart review and patient interviews. We compared the histologic findings in skin biopsies from patients with PRP to those of biopsies matched for site and age from control patients with psoriasis. Certain aspects of the clinical presentations encountered in the study group patients have not been previously emphasized, including eruptions compatible with a vesiculobullous disorder in 4 patients. Several patients had evidence of underlying iatrogenic or systemic immune dysregulatory states and certain triggers could be isolated such as emotional stress and prior exposure to microbial pathogens. Histomorphologic features predictive of PRP included follicular plugging, an increased granular cell layer, and acantholysis. While seen in a minority of biopsies of PRP, morphologic discriminators supportive of a diagnosis of psoriasis included neutrophils in the parakeratotic scale crust, and dermal papillae capillary ectasia with vessels lying in direct apposition to the epidermis. Other morphologic features were encountered in biopsies of PRP which have received little emphasis in the dermatopathology literature, including epithelial atrophy, significant dermal inflammation, the presence of eosinophils and plasma cells within the inflammatory infiltrates, an abnormal granular cell layer, and epidermolytic hyperkeratosis, albeit none were statistically signficant predictors of PRP over psoriasis. Nevertheless, their identification emphasizes the varied histologic spectrum of PRP. The almost ubiquitous finding of acantholysis and the presence of an abnormal granular cell layer in many biopsies of PRP suggests that abnormal keratins or defects in the retinol signalling pathway may be of pathogenetic importance.

Aged↗
PubMed2005

Photoaggravated pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous condition with an estimated incidence of one in 35,000 to one in 50,000. Psoralen and ultraviolet A (UVA) therapy has been used in its treatment but some patients are reported to be clinically photosensitive. We describe the photoinvestigation of a patient with PRP in whom sensitivity to broadband UVA was demonstrated.

Aged↗
PubMed2000

Three cases of pityriasis rubra pilaris successfully treated with cyclosporin A.

BACKGROUND: Pityriasis rubra pilaris (PRP) is reported to respond poorly to cyclosporin A (CsA). OBJECTIVE: We attempted to determine the efficacy of CsA in the treatment of classic adult-type PRP. METHODS: Three patients with classic adult-type PRP were treated with 5 mg/kg/day CsA. RESULTS: A sustained clinical response was achieved within 2-4 weeks of therapy. Relapses were noted when the CsA dose was decreased to 1.2 mg/kg/day. CONCLUSION: CsA should be considered in the treatment of classical adult-type PRP.

Adult↗
PubMed2002

Pityriasis rosea associated with imatinib (STI571, Gleevec).

A tyrosine kinase inhibitor (STI571, Gleevec) has recently been applied in the treatment of chronic myeloid leukemia. We present the first reported case of pityriasis rosea occurring as a reaction to Gleevec in a woman with blast crisis of this disorder. It is suggested that although coincidental, this exanthem may be due to this agent.

Adult↗
PubMed1978

Structure and biological function of human IgD. XVI. T and B lymphocytes in pityriasis rosea.

The relative frequency of peripheral blood T and B cells and their biological function(s) from a group of patients with pityriasis rosea (PR) was investigated during the acute and convalescent phases of the disease using rosetting, immunofluorescent tests, and in vitro cell culturing with anti-delta and anti-mu antibodies and phytohemagglutinin (PHA). The total number of immunoglobulin (Ig) bearing cells was significantly increased, in conjunction with a slight decrease in the T cell population. Lymphocytes with surface IgD, IgM, or both Ig, accounted for the increase in the B cell population. This increase was transient, since it was only observed during the acute phase of the disease. In spite of the increase in IgD/IgM bearing B cells, the mitogenic responsiveness of B or T lymphocytes to anti-delta, anti-mu, or PHA was similar to the same patients during the convalescent phase, or to normal donors. Similarly, the levels of serum IgM, IgD, IgG, IgA and IgE in PR remained constant and at a normal concentration throughout the experimental period. The significance of the transient increase in IgD/IgM bearing cells in the pathogenesis and etiology of PR and its possible impact on the immune system is discussed.

Adolescent↗
PubMed1997

Human herpesvirus 7 in patients with pityriasis rosea. Electron microscopy investigations and polymerase chain reaction in mononuclear cells, plasma and skin.

BACKGROUND: Clinical evidence suggests a viral etiology for pityriasis rosea (PR). OBJECTIVE: To evaluate human herpesvirus (HHV)-6 and HHV-7 as candidates for the etiology of PR. METHODS: Blood and skin tissue from 12 patients with acute PR, and 12 patients with other dermatoses were studied, as well as blood samples from 25 healthy persons. Serum interferon (IFN)-alpha and IFN-gamma were analyzed by ELISA. Analysis of morphological changes in cocultured peripheral blood mononuclear cells (PBMC) and electron microscopy (EM) to identify viral particles were performed. Polymerase chain reaction (PCR) with specific primers for HHV-6 and HHV-7 DNA sequences was performed on the plasma and PBMC of patients and healthy controls and on the skin of patients with PR and other skin diseases. RESULTS: PR plasma contained detectable IFN-alpha and IFN-gamma, whereas plasma from controls did not. PBMC from PR patients showed ballooning cells and syncytia after 7 days in culture whereas PBMC from controls and recovered PR patients did not. This cytopathic effect was also documented in a PR patient who relapsed and in Sup-T1 cell cultures inoculated with the cell-free supernatant from centrifuged cultured PBMC; in this supernatant, herpesvirus, virions were detected by EM, PCR identified HHV-7 DNA in PBMC, plasma and skin from all patients with active PR and in the PBMC only of 5 patients tested 10-14 months later. Weaker signals of HHV-7 DNA were detected in PBMC of 11 controls, but not in their plasma. Skin was negative for HHV-7 in all control specimens. CONCLUSIONS: Although the detection of HHV-7 DNA in PBMC and tissues does not prove directly a causal role, HHV-7 DNA in cell-free plasma corresponds to active replication which supports a causal relationship. We propose that PR is a clinical presentation of HHV-7 reactivation.

Cells, Cultured↗
PubMed1997

Pityriasis rotunda: a survey of 42 cases observed in Sardinia, Italy.

BACKGROUND: Pityriasis rotunda (PR) is an uncommon dermatosis characterized by multiple, round or oval, sharply demarcated scaling patches that are dyschromic and asymptomatic. It has been described in Japanese and in blacks, usually in association with certain infective or malignant systemic diseases. OBJECTIVE: The aim of this study is to further clarify this rare entity which in Italy seems to be confined to the island of Sardinia. METHODS: We studied 42 Sardinian patients, 22 males and 20 females, in an age range of 3-32 years. In 29 cases, the disease involved more than one family member. The patients were observed in Cagliari, the capital city of Sardinia. RESULTS: Bacterial, viral and fungal investigation yielded negative results. Haematochemical and immunological examination and thyroid, hypophyseal and adrenal hormones did not reveal any alterations. No systemic pathologies were found associated with the disease. CONCLUSIONS: The cases studied by us and those previously reported seem to indicate the presence of two distinct types of PR with significant prognostic differences.

Adolescent↗
PubMed1997

Pityriasis rubra pilaris as an initial presentation of hepatocellular carcinoma.

Pityriasis rubra pilaris (PRP) is a rare skin condition that is of unknown etiology. We present an unusual case of a patient with PRP as the sole initial manifestation of hepatocellular cancer. The course of the patient's illness and management of this case is discussed. Possible reasons for this association are presented.

Administration, Oral↗
PubMed1994

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta.

An unusually severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) with a fatal outcome in an 82-year-old woman is reported. After a period of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions developed, associated with intermittent high temperature and constitutional symptoms. Skin biopsies showed the typical histopathological changes of PLEVA. Early recognition of this severe variant of PLEVA is important, since the fulminating course can lead to death.

Aged↗