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Treatment of classic pityriasis rubra pilaris.

BACKGROUND: Treatment of classic pityriasis rubra pilaris, which almost always progresses to a generalized erythroderma with marked, often disabling keratoderma of the palms and soles, remains problematic. OBJECTIVE: Our purpose was to evaluate the results of treatment in a recent period during which the retinoid group of drugs has been available. METHODS: The clinical course of 75 patients with classic pityriasis rubra pilaris seen from 1982 to 1992 was reviewed. RESULTS: Of 15 patients treated with isotretinoin, 10 had complete and 2 had partial clearing. Of six treated with etretinate, four had clearing. All eight patients treated with methotrexate had a favorable response. Other forms of treatment, including Goeckerman regimen, corticosteroids, vitamin A, and cyclosporine, were ineffective. CONCLUSION: Early diagnosis and early treatment with retinoids appear to offer the best chance for clearing of pityriasis rubra pilaris. If retinoids fail or cannot be used, methotrexate should be considered.

Adolescent↗

Pityriasis rosea--evidence for and against an infectious aetiology.

Pityriasis rosea, first named as such in 1860, probably holds the longest record for an exanthem suspected to be associated with an infection but for which an exact cause has not been found. The distinctly programmed clinical course, the lack of recurrence for most patients, and the presence of temporal case clustering provide the strongest evidence to support an infectious aetiology. Further support comes from seasonal variation and the association with respiratory tract infections, the unfavourable social and economic background of cases, and a history in some cases of contact with patients with pityriasis rosea. The apparent therapeutic efficacy of several treatment modalities does not provide strong evidence for or against an infectious aetiology. The roles of human herpesvirus 7 and to a lesser extent human herpesvirus 6 remain controversial. There exists reasonable evidence that pityriasis rosea is not associated with cytomegalovirus, Epstein-Barr virus, parvovirus B19, picornavirus, influenza and parainfluenza viruses, Legionella spp., Mycoplasma spp. and Chlamydia spp. infections. Evidence is also unsubstantiated as yet for alternative aetiological hypotheses such as autoimmunity, atopy, and genetic predisposition.

Bacterial Infections↗

Localized pityriasis rosea.

Pityriasis rosea is a relatively common skin disorder. In its typical form it is easily recognizable; however, atypical forms can pose diagnostic problems. We report a 44-year-old woman with an acute onset of a localized eruption on her left breast. The morphology of the rash and the time course were typical of pityriasis rosea. Localized pityriasis rosea is an unusual variant, which has been described previously.

Administration, Topical↗

Acral pityriasis lichenoides.

Two patients presented with acrally located papulosquamous lesions that were polymorphic and had an identical morphology to those of pityriasis lichenoides. The lesions remained localized to the feet and ankles for years. Multiple skin biopsies had the histopathological features of pityriasis lichenoides. In one biopsy syringotropic lymphocytes and early syringolymphoid hyperplasia were observed. This rare variant of pityriasis lichenoides may be under-recognized and misdiagnosed as it resembles a variety of papulosquamous disorders particularly psoriasis.

Administration, Topical↗

Histologic criteria for the diagnosis of pityriasis rubra pilaris.

Even though the clinical appearance of pityriasis rubra pilaris is often distinctive enough to allow specific diagnosis of it, criteria for its histologic diagnosis have not yet been well defined. On the basis of a study of 55 cases of pityriasis rubra pilaris, we found the following histologic features helpful for diagnosis throughout most of the course of the disease: alternating orthokeratosis and parakeratosis in both vertical and horizontal directions; focal or confluent hypergranulosis; thick suprapapillary plates; broad rete ridges; narrow dermal papillae; and sparse superficial perivascular infiltration, mostly of lymphocytes. Plugging of follicular infundibula by cornified cells was seen only in biopsy specimens that came from lesions that were clinically indubitably follicular. Psoriasis has many histologic features in common with pityriasis rubra pilaris, but evolving and fully developed lesions of psoriasis have neutrophils in mounds of parakeratosis, thin rete ridges, thin suprapapillary plates, broad dermal papillae, and mixed inflammatory cell infiltrates of variable density.

Adolescent↗

Pityriasis lichenoides: a cytotoxic T-cell-mediated skin disorder. Evidence of human parvovirus B19 DNA in nine cases.

BACKGROUND: Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) probably represent the polar ends of the same pathologic process, i.e. pityriasis lichenoides (PL), with intermediate forms in between. Previous studies have demonstrated that the inflammatory infiltrate in PLEVA is composed of cytotoxic suppressor T cells, whereas in PLC the helper/inducer T-cell population drives the immunological answer. Furthermore, monoclonal rearrangement of the T-cell receptor-gamma (TCR-gamma) genes was repeatedly found both in PLEVA and PLC. METHODS: Forty-one formalin-fixed, paraffin-embedded tissue specimens of 40 cases of PL were retrieved from the files of the authors. Immunophenotyping for cytotoxic granular proteins (Tia-1/GMP-17 and Granzyme B) and T-cell-related antigens (n = 41), TCR-gamma chain gene analysis (n = 30) and molecular investigations for parvovirus B19 (PVB19) DNA (n = 30) were performed. RESULTS: Overlapping immunophenotypes were observed in PLEVA and PLC. The dermal and epidermal T cells predominantly expressed CD2, CD3, CD8, and Tia-1 with a variable positivity for CD45RA, CD45RO, and Granzyme B. A monoclonal rearrangement pattern of the TCR-gamma genes was detected in three cases (10%). PVB19 DNA was found in nine cases (30%). T-cell monoclonality in conjunction with genomic PVB19 DNA was present in one case. CONCLUSIONS: Our results demonstrate that PL is a skin disorder mediated by the effector cytotoxic T-cell population. The identification of PVB19 DNA in nine cases may be interpreted ambiguously: PVB19 as a true pathogen or as an innocent bystander.

Adolescent↗

Pityriasis rotunda mimicking tinea cruris/corporis and erythrasma in an Indian patient.

Pityriasis rotunda is a rare disease characterized by perfectly round to oval, sharply defined, scaly, hypo/hyperpigmented patches of variable number and size located mainly over the trunk and proximal extremities. More than 95% of the reported cases in medical literature are from three countries/ethnic populations, namely Japan, South Africa (Bantu), and Italy (Sardinian islanders). To the best of my knowledge, no patient with the characteristic clinico-pathologic features has been reported from the Indian subcontinent. I report a 44-year-old man with eighteen pityriasis rotunda patches, persistent for nearly 20 years. The lesions in the groin and axillae closely resembled erythrasma and tinea, and he had received treatment for these conditions several times in the past. Histopathology of the skin biopsy showed thinning of the epidermis with a thinned-out granular layer and a sparse lymphomononuclear infiltrate in the dermis. A review of literature suggests that there are two subsets of the disease. The type I subset is comprised of pityriasis rotunda associated with systemic illness and is seen in Black or Oriental patients with no family history of the disease. The lesions tend to subside on treatment of the underlying illness. The type II subset patients are Caucasians as well as Blacks and Orientals with no underlying systemic illness. Familial occurrence is possible; lesions tend to be persistent and unresponsive to therapy.

Adult↗

Recent upper respiratory tract infection and pityriasis rosea: a case-control study of 249 matched pairs.

A sample of 249 patients was drawn from an original population of 939 patients with pityriasis rosea identified in an earlier population-based study of pityriasis rosea in Rochester, Minnesota. A control was selected for each case, matched for age, sex, and year of diagnosis. Comparison was made with regard to recent immunization, history of atopy, and recent infection. A statistically significant number of patients had experienced recent upper respiratory tract infection (P less than 0.005). The association between recent infection and pityriasis rosea is discussed.

Adult↗

Pityriasis rotunda as a cutaneous marker of hepatocellular carcinoma: a comparison with its prevalence in other diseases.

In an attempt to substantiate the claim that pityriasis rotunda may be a useful cutaneous marker of hepatocellular carcinoma in South African Blacks, the prevalence of the rash in 63 unselected South African Blacks with this tumour was compared to that in 63 matched patients with active tuberculosis, 63 with other malignant tumours, and 63 with various forms of chronic benign hepatic disease. The prevalence of pityriasis rotunda in hepatocellular carcinoma was 15.9%, which was appreciably greater (P = 0.0005) than the overall prevalence of the rash (2.6%) in the controls. The prevalence was 4.8% for tuberculosis (P = 0.038), 0% for other malignant diseases (P = 0.0007), and 3.2% for chronic benign hepatic disease (P = 0.015). We conclude that the presence of pityriasis rotunda is a useful pointer to the diagnosis of hepatocellular carcinoma in South African Blacks.

Adult↗

Topical treatment of pityriasis rubra pilaris with calcipotriol.

From a clinical, histological and therapeutic point of view, psoriasis and pityriasis rubra pilaris share important characteristics. Recently, calcipotriol has been shown to be an effective treatment in psoriasis, and we report three patients with pityriasis rubra pilaris who showed a favourable response to topical therapy with calcipotriol. In one case, analysis of markers for epidermal growth, differentiation and inflammation revealed reduction of suprabasal expression of keratin 16, and the number of T lymphocytes, monocytes and macrophages. It is of interest that a reduction of the recruitment of cycling epidermal cells, which is a consistent response pattern during treatment of psoriasis, was not observed during treatment of pityriasis rubra pilaris.

Administration, Topical↗

Pityriasis rosea in Nigerians.

One hundred thirty-eight Nigerian patients with pityriasis rosea were studied. Patients with the condition constituting 2.4% of all patients seen in two skin clinics in Northern Nigeria in a period of over three years. The proportional rate of pityriasis rosea parallels figures from other African studies, and is higher than values obtained in European series. Pyriasis rosea in Nigerians appears to be more florid and has a greater tendency to overstep the classic boundaries of distribution on the body, to affect the face and involve the oral cavity. The sex and age distribution was similar to European series, although some lowering of the age of onset of the condition was observed. No relationship between the frequency of pityriasis rosea and season was found.

Adolescent↗

Pityriasis rosea in Lagos.

A recent 5-year period study of the incidence of pityriasis rosea among dermatologic outpatients was conducted at the Lagos University Teaching Hospital. Three hundred fifty-two patients with pityriasis rosea were seen. The average annual incidence was 4.8 per 100 dermatologic patients. Women predominated by a margin of 1.2:1.0. More than 91% of the patients were between the ages of 5 and 35 years, with a peak at ages 10-14. The incidence of the disease was significantly higher in the early part of the rainy season. No declining incidence was observed over the years. A blood picture of a leukopenia with relative lymphocytosis was observed early in the disease. Ampicillin, when consumed after the onset of the eruption, made the patient with pityriasis rosea clinically worse.

Adolescent↗

Pityriasis alba: a histologic study.

BACKGROUND: Pityriasis alba is a common dermatitis, but only a few histologic studies have been reported and most of these maintain that its microscopic features are those of a chronic nonspecific dermatitis. METHODS: A retrospective light microscopic study was performed on biopsy specimens taken from 39 patients with localized pityriasis alba on face, arms and forearms without signs of atopy. Thirty-two patients were girls or women and seven were boys or men, with ages ranging from 7 to 37 years. The duration of illness varied from 1 month to 10 years. The clinical picture was characterized by three clinical stages: papular erythematous, papular hypochromic, and smooth hypochromic. RESULTS: The histologic study revealed features that were suggestive, though not pathognomonic, of the diagnosis. The most remarkable changes involved the pilary apparatus, which showed horny plugs, spongiotic edema, and atrophic sebaceous glands. There was also a disturbance of melanization secondary to epithelial damage. The tissue reaction was more conspicuous in the skin lesions with follicular papules. CONCLUSIONS: A histopathologic diagnosis of pityriasis alba may be proposed when the following features are seen in a biopsy specimen taken from a skin lesion with follicular papules: (1) irregular pigmentation by melanin of the basal layer; (2) follicular plugging; (3) follicular spongiosis; and (4) atrophic sebaceous glands.

Adolescent↗

UVB therapy of pityriasis lichenoides--our experience with 29 patients.

BACKGROUND: Pityriasis lichenoides comprises a clinical and pathological spectrum of disorders. So far no highly effective treatment has been reported. Previous small studies have suggested that ultraviolet B (UVB) is a good alternative. METHODS: This is a retrospective analysis of 29 pityriasis lichenoides patients treated in our institution with broad- or narrow-band UVB during the period 1996-2002. Twenty-one of these patients had one or more previous unsuccessful treatments. RESULTS: Complete response was achieved in 93.1% in both treatment groups, with 73% of them still relapse free after a mean follow-up of 58 and 38 months in broad- and narrow-band UVB treatment groups, respectively. Mild side-effects were observed in about one-third of the patients. CONCLUSION: We believe both forms of UVB are a good option for pityriasis lichenoides and should be considered as the first line in generalized cases interested in treatment.

Adult↗

Pigmenting pityriasis alba.

We conducted a prospective study of 20 patients with pigmenting pityriasis alba (PPA) over a period of two years. Characteristic morphology revealed a central zone of bluish hyperpigmentation surrounded by a hypopigmented, slightly scaly halo of variable width. All patients displayed lesions on the face. Concomitant extrafacial involvement was uncommon. A significant finding was an associated dermatophyte infection in 13 patients (65%). These patients all received griseofulvin 10 mg/kg/day for eight weeks, resulting in the resolution of PPA in seven within 4 to 20 weeks. These were also treated with 1% hydrocortisone. Biopsy specimens from two patients showed similar features, namely, a subacute dermatitis with variable pigment incontinence. Immunohistochemical labeling revealed a preponderance of T lymphocytes. Pigmenting pityriasis alba seems to be a variant of classic pityriasis alba showing a strong association with dermatophyte infection, especially tinea capitis. It may be related to lichenoid melanodermatitis.

Administration, Topical↗

Scanning electron microscopy of scales from pityriasis amiantacea.

The scanning electron microscope has been used to examine scales taken from the scalp of 3 patients with pityriasis amiantacea alone, 3 psoriatic patients with pityriasis amiantacea and one patient with both atopic dermatitis and pityriasis amiantacea. Samples from 2 patients were additionally studied by different fixation techniques and in the frozen hydrated state, but no cementing of the scales could be observed. There was no evidence of an infective agent.

Humans↗

Recurrent pityriasis rosea. New episodes every year for five years. A case report.

A case of recurrent pityriasis rosea in a 39-year-old woman is presented. She had her first attack of pityriasis rosea 5 years ago and new outbreaks followed every year, in the spring. Her husband had a severe attack of pityriasis rosea 6 years ago. All laboratory investigations were normal and no explanation for the many recurrences was found.

Adult↗

[Pityriasis rosea in secondary schools in Ouagadougou, Burkina Faso].

BACKGROUND: Pityriasis rosea is a spontaneously regressive benign erythematous squamous dermatosis. A better understanding of this condition in developing countries would be useful. PATIENTS AND METHODS: We conducted a cross section study based on one-day surveys in Ouagadougou, Burkina Faso, secondary schools. The stratified cluster sampling method described by Henderson was used. Six thousand pupils were examined to collect epidemiology and clinical data and to determine the presence of complications and results of complementary explorations performed. RESULTS: Thirty-six cases of pityriasis rosea were observed (0.6 p. 100). Prevalence was higher in children from an unfavorable social and economic background. A typical eruption was often observed with classical localizations and morphology in 86.1 p. 100 of the cases. Pruritus was often observed with an inaugural lesion (61.1 p. 100) predominantly on the upper limbs (54.6 p. 100) and the trunk (31.8 p. 100) measuring 1 to 3 cm. A typical second eruption was found in most cases, situated on the trunk (75 p. 100), distal portions of the limbs (64.4 p. 100), proximal portions of the limbs (33.3 p. 100) and the neck (30.5 p. 100). The eruption evolved for 1 week to 1 month in most cases (61 p. 100). In 55.5 p. 100 of the cases, therapeutic abstention was the rule. Secondary treatment-related complications occurred in 38.9 p. 100 of the cases. Stool studies generally demonstrate ameba cysts. Syphilis serology was negative. DISCUSSION: Our sampling technique in the secondary school setting provided a representative sample of the urban population of African cities. This work demonstrated the variability of prevalence by social and economic conditions and is in favor of an infections cause, demonstrating several similar features with other series reported in the literature. Generally, a stool study is not necessary and syphilis serology is negative in typical cases. Health care workers should be more aware of pityriasis rosea in order to avoid poorly adapted treatment.

Adolescent↗