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Focal acantholytic dyskeratosis occurring in pityriasis rubra pilaris.

Focal acantholytic dyskeratosis (FAD) is a distinctive histologic pattern characterized by suprabasilar clefts surrounding dermal papillae (villi), acantholytic and dyskeratotic cells at all levels of the epidermis, hyperkeratosis, and parakeratosis. The features of FAD are typically seen in Darier's disease, warty dyskeratoma, and transient acantholytic dermatosis; they are also present in a variety of cutaneous neoplastic and nonneoplastic lesions. FAD, however, has not been previously described in lesions of inflammatory dermatoses. We report a case of FAD occurring in lesions of pityriasis rubra pilaris (PRP). To the best of our knowledge, this is the first reported case of this kind. We also review the pertinent literature.

Acantholysis↗

Pityriasis rubra pilaris with acantholysis and lichenoid histology.

Acantholytic foci have been reported several times in pityriasis rubra pilaris (PRP). Lichenoid tissue reactions were also mentioned in the literature regarding PRP. We report a 58-year-old patient who, after having colon cancer, had PRP with biopsies showing acantholytic lesions and a heavy lichenoid lymphocytic infiltration. Investigation by serial sectioning of the acantholytic lesion suggested an involvement of the intraepidermal eccrine duct and further investigation with carcinoembryonic antigen (CEA) staining demonstrated a CEA-positive eccrine duct in the acantholytic foci. We suggest that acantholysis in PRP is induced by proteolytic enzymes, urea, and other substances in eccrine sweat in keratin-plugged acrosyringia. This patient had a combination of three relatively rare features of PRP-acantholysis, lichenoid reaction, and a cancer background.

Acantholysis↗

Additional evidence that pityriasis rosea is associated with reactivation of human herpesvirus-6 and -7.

To elucidate the role of human herpesvirus (HHV)-6 and -7 (HHV-7) in pityriasis rosea (PR), we measured their DNA load in plasma, peripheral blood mononuclear cells (PBMC), and tissues using a calibrated quantitative real-time PCR assay. We also studied HHV-6- and HHV-7-specific antigens in skin by immunohistochemistry and anti-HHV-7 neutralizing activity using a syncytia-inhibition test. Plasma and PBMC were obtained from 31 PR patients (14 children, 17 adults), 12 patients with other dermatites, and 36 blood donors. Skin biopsies were obtained from 15 adults with PR and 12 with other dermatites. HHV-6 and HHV-7 DNA were detected in 17% and in 39% of PR plasmas, respectively, but in no controls. HHV-7 viremia was associated with a higher PBMC load and, in adults, with systemic symptoms. HHV-7, but not HHV-6, levels in PBMC were higher in PR patients than in controls. HHV-6 and HHV-7 antigens were found only in PR skin (17% and 67% of patients analyzed, respectively), indicating a productive infection. Syncytia-neutralizing antibodies were found in PR patients and controls, but their titers were lower in patients with HHV-7 viremia. These data confirm the causal association between PR and active HHV-7 or, to a lesser extent, HHV-6 infection.

Adult↗

Immunological studies of pityriasis rosea (Gibert).

In order to elucidate the immunological mechanism in the pathogenesis of pityriasis rosea, immunofluorescent studies were performed on sera obtained from forty patients with this disease. Antibodies against the cytoplasm of normal human epidermal cells were demonstrated in the sera of all patients. The antibody titer showed a tendency to increase within 3 weeks after onset of secondary eruptions and then to decrease gradually until the period of recovery. The immunoglobulin class was determined to be IgM. Furthermore, by the direct immunofluorescent technique, deposits of IgM in the epidermal cells of skin lesions were demonstrated in 3 of 6 herald lesions and in 1 of 4 secondary eruptions. It is suggested that anti-cytoplasmic antibodies produced by some unknown cause may induce the development of secondary eruptions of this disease.

Antibodies↗

Heliotherapy of pityriasis lichenoides chronica.

Fifteen patients with pityriasis lichenoides chronica (parapsoriasis guttata) were treated with sunlight irradiation during the summer months at seaside resorts under the supervision of the authors. Complete clearing occurred in 1 of the patients, almost complete clearing in 8, definte improvement in 4, temporary clearing in 2. All patients responded beneficially and no worsening was noted in these 15 cases. In an additional six cases who were instructed to expose themselves to sunlight similar results were obtained.

Adolescent↗

Pityriasis lichenoides et varioliformis acuta immunohistopathologic study.

Immunoglobulin M and complement (Clq, C3 and C9) were found to be deposited together in the walls of affected blood vessels and also in the basement membrane zone of skin lesions from two patients with pityriasis lichenoides et varioliformis acuta (PLVA). The fluorescence appeared to be most intensely distributed in blood vessels which were affected by inflammatory infiltrates and frequently showed hyaline or fibrinoid changes of their walls. Deposits of C3 activator, fibrin and albumin were not observed. These findings suggest that the disease process in PLVA is the result of a vasculitis mediated by an immune complex and possibly by activation of the classical complement pathway. In addition, serum IgM was slightly increased in both patients.

Adolescent↗

Pityriasis rosea in a patient with Behçet's disease treated with interferon alpha 2A.

Interferons have been used for the treatment of many diseases because of their immunomodulatory, antiviral, antitumoral, and antiproliferative effects. Systemic interferon alpha 2a (IFN-alpha 2a) is also efficacious in the management of Behçet's disease (BD). Many adverse cutaneous reactions related to IFN-alpha have been reported. In the light of the current literature, we report here the first case of Pityriasis rosea (PR) that developed while receiving IFN-alpha 2a for BD. However, since this is the first report on this association, further observations are needed to determine their clinical relevance.

Adult↗

Pityriasis rubra pilaris (PRP): report of four cases.

Four patients with pityriasis rubra pilaris are reported. The diagnosis in each was based upon well-recognized clinical features. Two of them, a mother and son, had the disease since childhood and were marked by relative remission in spring and exacerbation in autumn. Moderate to severe pruritus was a common dominator. Erythroderma was a presenting feature in one case. Although histopathology was considered imperative, it only supplemented the clinical expression. Vitamin A in heavy dosage, supplemented by vitamin E and stanozolol in tandem, was the mainstay of treatment.

Adult↗

Pityriasis lichenoides chronica presenting as hypopigmentation.

Seven black or dark skinned patients with pityriasis lichenoides chronica (PLC) are described, who presented with widespread hypopigmentation. The evidence on which a diagnosis of PLC was made is presented and the differential diagnosis discussed. The hypopigmentation was noted in some patients to be especially marked on the proximal parts of the limbs and axillary folds. A diagnosis of PLC should be considered in all dark skinned patients with a widespread hypopigmented rash.

Adolescent↗

Pityriasis rubra pilaris: epidermal cell kinetics.

Epidermal proliferative kinetics were studied in a patient with pityriasis rubra pilaris. Elevated proliferative indices (flash labelling and mitotic indices) were apparent compared with normal epidermis and measurements of the birth rate indicate that the rate of epidermal cell production was similar to that found in psoriasis, and equalled 11.8 cells/1000 germinative cells/h; this suggests a cell cycle time of 60 h. Contemporaneous measurements of the rate of entry into DNA synthesis showed values similar to the rate of entry into mitosis; hence there is no G2 cell loss as appears to occur in the hyperproliferative psoriatic epidermis.

Aged↗

Ultrastructure of pityriasis rubra pilaris with observations during retinoid (etretinate) treatment.

The light and electron microscopic structure of pityriasis rubra pilaris (PRP) is described in five patients. Hyperkeratosis, hypergranulosis, keratotic plugs in the follicular openings, acanthosis and focal parakeratosis were observed. A moderate perivascular infiltrate was seen in the upper dermis. Electron microscopy revealed moderately activated keratinocytes, a decreased number of tonofilaments and desmosomes, enlarged intercellular spaces, parakeratosis with lipid-like vacuoles and a large number of keratinosomes. Lymphoid cells were present in the epidermis in moderate numbers. At the dermo-epidermal junction, the basal lamina was focally split, containing gaps. Etretinate therapy produced moderate to marked clinical improvement. The histological picture improved but the typical signs of PRP, including follicular plugging, persisted. Ultrastructurally the cellular activity and the amount of hyperkeratosis and parakeratosis decreased, while increases in keratinosomes, intercellular substance, microvilli and desmosomes were observed during treatment.

Adolescent↗

Extensive pityriasis alba: a histological histochemical and ultrastructural study.

Nine patients with extensive pityriasis alba were studied using histopathological and histochemical techniques and electron microscopy. There was a reduction in the density of functional melanocytes in the affected areas without any change in their cytoplasmic activity. The melanosomes tended to be fewer and smaller, but their distribution pattern in the keratinocytes was normal. Melanosomal transfer to keratinocytes was generally not disturbed. The histology was non-specific. Hyperkeratosis and parakeratosis were not consistently present, and it seems unlikely that they played a significant role in the pathogenesis of the hypomelanosis. A variable degree of intercellular oedema and intracytoplasmic lipid droplets were present. The hypopigmentation may thus be due primarily to the reduced numbers of active melanocytes and a decrease in number and size of melanosomes in the affected skin.

Adolescent↗

Pityriasis rubra pilaris in the setting of HIV infection: clinical behaviour and association with explosive cystic acne.

The development of pityriasis rubra pilaris (PRP) in three patients with human immunodeficiency virus (HIV) infection is described. Two of the patients had onset of severe generalized cystic acne concomitant with their development of PRP. PRP and acne conglobata should be added to the group of cutaneous disorders that can present in a more virulent manner in the setting of HIV infection. The association of cystic acne with PRP and their response to treatment are discussed.

Acne Vulgaris↗

Pityriasis rubra pilaris and human immunodeficiency virus infection.

Recently, the occurrence of pityriasis rubra pilaris (PRP) has been reported in patients with HIV infection. It presents different clinical features, and has a poorer prognosis, than the classical adult type of PRP. We report the occurrence of severe PRP in an HIV-infected patient, and review the previously reported cases of this association. We propose the designation of a new category of PRP (type 6), characterized by the presence of HIV infection, usually without immunosuppression, a poor prognosis and response to treatment, and the development of nodulocystic and lichen spinulosus lesions.

Adult↗

Paraneoplastic pityriasis lichenoides in cutaneous lymphoma: case report and review of the literature on paraneoplastic reactions of the skin in lymphoma and leukaemia.

Paraneoplastic dermatoses are non-neoplastic skin disorders which occur in the context of an underlying malignant neoplasm. The classic paraneoplastic dermatoses are mostly associated with solid internal malignancies. They only rarely occur in the context of nodal or primary cutaneous lymphomas. Apart from these classic paraneoplastic dermatoses, there are additional skin disorders reported to occur in close association with haematological and lymphoproliferative disorders which can thus be regarded as paraneoplastic manifestations. We report for the first time two patients with pityriasis lichenoides et varioliformis acuta in association with mycosis fungoides. In addition, we review the literature on paraneoplastic dermatoses of the skin which have been described in patients with leukaemias and primary cutaneous lymphomas.

Adult↗

Tacrolimus ointment 0.1% in pityriasis alba: an open-label, randomized, placebo-controlled study.

BACKGROUND: Pityriasis alba (PA) is a frequent reason for dermatological consultation because of its chronic course, tendency to relapse and aesthetic impact. OBJECTIVES: In view of its strong association with atopic dermatitis, the objective of this open-label study was to assess the efficacy and safety of tacrolimus ointment in the treatment of PA compared with the efficacy of moisturizers. PATIENTS/METHODS: The study population consisted of 60 individuals of phototype III or IV according to Fitzpatrick's classification, aged 6-21 years. Patients were randomly assigned to one of two groups. Subjects in group A were instructed to apply tacrolimus ointment 0.1% twice daily, 12 h apart, on all hypopigmented macules. Standard moisturizers with SPF 20 sunscreen were used on all lesions applied at least 30 min apart from the tacrolimus ointment. Subjects in group B used solely the same moisturizers with sunscreen. Hypopigmented areas were evaluated at baseline and weeks 0, 3, 6 and 9 by investigators for scaling, hypopigmentation and pruritus on a scale of 0-3. Patient satisfaction was also recorded on a scale of 0-3. All adverse effects were recorded. RESULTS: A statistically significant improvement through time, in hypopigmentation, pruritus and scaling was observed in both groups during the course of 9 weeks. Hypopigmentation resolved from a baseline score of 2.38+/-0.64 to 1.15+/-0.54 at week 3, 0.46+/-0.51 at week 6 and 0.00+/-0.00 at week 9 for the group applying tacrolimus ointment 0.1%. The difference in improvement between the two groups was statistically significant on all three assessments for hypopigmentation (P<0.001), and for pruritus on week 6 and 9 assessments (P<0.05). Three patients (11.5%) in the tacrolimus group reported a mild transient sensation of burning. All patients in the tacrolimus group reported they were completely satisfied or just satisfied with the treatment compared with only 50% of patients using the placebo. CONCLUSIONS: Tacrolimus ointment 0.1% appears to be an effective and safe treatment for PA.

Adolescent↗

Pityriasis rubra pilaris in Singapore.

Fourteen patients with pityriasis rubra pilaris seen between 1981 and 1989 were evaluated retrospectively. There were nine male and five female patients. There was a bimodal age of onset in early childhood and in adulthood. All the patients had either follicular hyperkeratosis or hyperkeratosis of the palms and soles. Nine patients had erythroderma. The classical forms, either in the children or in the adult patients, were more likely to recover. Several treatment modalities were used and these included steroids, Vitamin A, etretinate, retinoic acid and ultraviolet light (UBV). Nine patients had complete recovery. Four of these patients received only steroids, two cleared while on vitamin A, two cleared while on etretinate and one cleared with methotrexate.

Adolescent↗