Pityriasis rotunda. A clinical study in Jordan: experience of 10 years.

BACKGROUND: Pityriasis rotunda is a rare skin disease characterized by the presence of multiple, round or oval, sharply demarcated hyperpigmented scaly patches. It has been described in Japan, South Africa, and in some other countries. The cause of pityriasis rotunda is usually idiopathic but may be associated with certain internal malignancy or systemic diseases. OBJECTIVE: The aim of this study is to describe this rare entity in Jordan in a retrospective study during the last 10 years. Jordan, as a Middle Eastern country, had no previous reports about this rare disease. In addition, the intention was to report any systemic association with the disease. Some of the cases were examined by dermoscopy. No previous reports documented dermatoscopic features of the disease. RESULTS: We could report 23 cases during the last 10 years. Eighteen cases were females and five males, in an age range of 9-38 years. There were three familial cases. The dermatoscopic findings were well demarcated, hyperpigmented with brownish patches with polygonal scales. These scales were more defined, not homogeneous in color, and separated from each other by paler stria. CONCLUSIONS: The cases of the study seem to indicate the rarity of the disease in Jordan. Our case fits neatly into the benign form of the disease with lack of association with any underlying diseases.

Papulopustular rosacea, skin immunity and Demodex: pityriasis folliculorum as a missing link.

Papulopustular rosacea (PPR) is a common facial skin disease, characterized by erythema, telangiectasia, papules and pustules. Its physiopathology is still being discussed, but recently several molecular features of its inflammatory process have been identified: an overproduction of Toll-Like receptors 2, of a serine protease, and of abnormal forms of cathelicidin. The two factors which stimulate the Toll-like receptors to induce cathelicidin expression are skin infection and cutaneous barrier disruption: these two conditions are, at least theoretically, fulfilled by Demodex, which is present in high density in PPR and creates epithelial breaches by eating cells. So, the major pathogenic mechanisms of Demodex and its role in PPR are reviewed here in the context of these recent discoveries. In this review, the inflammatory process of PPR appears to be a consequence of the proliferation of Demodex, and strongly supports the hypothesis that: (1) in the first stage a specific (innate or acquired) immune defect against Demodex allows the proliferation of the mite; (2) in the second stage, probably when some mites penetrate into the dermis, the immune system is suddenly stimulated and gives rise to an exaggerated immune response against the Demodex, resulting in the papules and the pustules of the rosacea. In this context, it would be very interesting to study the immune molecular features of this first stage, named "pityriasis folliculorum", where the Demodex proliferate profusely with no, or a low immune reaction from the host: this entity appears to be a missing link in the understanding of rosacea.

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.

[Pityriasis rotunda]. / Pityriasis rotunda.

Pityriasis rotunda (PR) is a well-known skin disease in Japan, but it is seldom observed in Europe. Because it is often associated with an underlying, sometimes malignant disease, it is regarded by some authors as a clinical form of acquired ichthyosis. We report the case of a 20-year old male Congolese presenting with PR and ichthyosis vulgaris. This rarely described association raises the problem, discussed here, of whether PR belongs to ichthyosis vulgaris.

Inflammatory and papulosquamous disorders of the skin and eye.

We have discussed herein the dermatologic and ocular manifestations of several inflammatory diseases. Cooperation between ophthalmologists and dermatologists can significantly enhance patient care and comfort. It is hoped that this review will stimulate increased awareness of the prevalence of ocular findings in these diseases and encourage cooperation between these specialties for the benefit of our patients.

HIV seropositivity in association with pityriasis lichenoides et varioliformis acuta.

We describe a case of PLEVA in an asymptomatic, human immunodeficiency virus (HIV) positive patient. This association has not been previously described. The possible mechanisms involved are discussed.

Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis acuta in young children.

Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are related benign disorders without recognized association with cutaneous T-cell lymphoma (CTCL). We report the cases of two children with documented PLEVA evolving into CTCL over several years. One child had the clinical lesions of PLC but the dermatopathologic findings of PLEVA at age 2 years. At age 12 years, he had skin changes of poikiloderma atrophicans vasculare and dermatopathologic findings consistent with parapsoriasis en plaque. The second child presented at age 7 years with scaling dermatitis and dermatopathologic findings of PLEVA. At age 12 years, the histologic diagnosis was parapsoriasis. Monoclonal antibody studies performed on biopsy specimens from both patients revealed 70% to 100% cells staining with CD5, 80% to 90% staining with CD4, 30% to 50% staining with CD8, and an increase in CD1-staining cells in the papillary dermis, indicating a predominantly helper T-cell infiltrate. We believe that PLC and PLEVA may be part of the spectrum of CTCL. Furthermore, CTCL may be more common in young children than once thought.

Mucha-Habermann's disease and arthritis: possible association with reactivated Epstein-Barr virus infection.

We present a 12-year-old girl with skin lesions, arthritis and clinical response to tetracycline consistent with Mucha-Habermann's disease. She also showed serological evidence of reactivated Epstein-Barr virus (EBV) infection. We believe this represents the first such association of Mucha-Habermann's disease with EBV infection.

[Benign familial chronic pemphigus and pityriasis rosea. Clinical aspects and histology of the coexistence of both dermatoses]. / Pemphigus chronicus benignus familiaris und Pityriasis rosea. Klinik und Histologie bei Koexistenz beider Dermatosen.

We report the course of benign familial pemphigus (Hailey-Hailey disease) in a 45-year-old woman. While this condition was in remission the patient started to suffer from pityriasis rosea. A biopsy of this coexisting dermatosis revealed the typical features of Hailey-Hailey disease (suprabasal acantholysis) and of pityriasis rosea within the same lesion.

Pityriasis rosea eruption in secondary syphilis: an isomorphic phenomenon?

A 41-year-old man was referred because of a slightly pruritic eruption, and a classic case of pityriasis rosea was diagnosed on the first examination. Results of routine tests for syphilis were strongly positive, and on repeated examination the eruption had grown further. Skin biopsy specimens showed plasma cells and Treponema pallidum. All symptoms disappeared following antisyphilitic treatment. The possibility of an isomorphic response is discussed.

Pityriasis rotunda. A cutaneous sign of malignant disease in two patients.

The term pityriasis rotunda refers to strikingly circular scaly lesions, with the histologic appearance of ichthyosis vulgaris. Pityriasis rotunda has been described in the Japanese, South African blacks, and West Indian blacks. The condition occurs only in association with certain serious systemic illnesses, usually tuberculosis, or malignant neoplasms or less severe diseases of the female genital tract (eg, fibroids, ovarian cyst). We describe two South African blacks with pityriasis rotunda and neoplasms. The cutaneous lesions cleared when the primary neoplasm was treated.

Lymphoma-associated papulosis: lymphomatoid papulosis associated with lymphoma.

Lymphomatoid papulosis is a condition characterized by the continuing appearance of self-healing inflammatory papulonodules which histologically show atypical cells simulating a malignant process. We describe two patients who had features typical of lymphomatoid papulosis in whom a lymphoreticular malignancy developed. The first patient developed Hodgkin's disease 30 years after the onset of lesions that resembled lymphomatoid papulosis, and the second patient developed a lymphocytic lymphoma 11 months after the appearance of the self-healing papules. While most patients with lymphomatoid papulosis have a benign protracted course, "lymphoma-associated papulosis" represents the malignant end of this spectrum, in which lymphomatoid papulosis is associated with a lymphoma. All patients with lymphomatoid papulosis require close, long-term follow-up.