Frequency of occurrence of polymorphic light eruption in patients treated with photohardening and patients treated with phototherapy for other diseases.

BACKGROUND: Medical phototherapy can lead to the manifestation of polymorphic light eruption (PLE), though little is known about the frequency of such events. AIMS: The aim of this Austrian single center study was to retrospectively investigate over a 4-year time period the frequency of PLE in patients prone to the condition and patients with other diseases under phototherapy (mainly narrow-band and broad-band UVB). MATERIALS AND METHODS: The data for analysis were obtained from the electronic health and patient record database and patient files of the Photodermatology Unit, Department of Dermatology, Medical University of Graz, Austria. RESULTS: PLE occurred in 24.3% (18/74) of PLE patients but only 0.7% (3/421) of psoriasis patients under phototherapy (chi-square; P < 0.0001). PLE also occurred in 1.2% (3/257) of patients with atopic eczema, 0.8% (1/118) with prurigo, 3.5% (4/115, P = 0.0206) with parapsoriasis en plaques/mycosis fungoides, 7.4% (2/27, P = 0.0013) with granuloma anulare, 14.3% (1/7, P = 0.0002) with scleroderma, and 16.7% (1/6, P < 0.0001 vs. psoriasis) with pityriasis lichenoides chronica or pityriasis lichenoides eruptiva et varioliformis acuta. DISCUSSION AND CONCLUSION: These results are helpful for treatment allocation and risk estimation of PLE occurrence with regard to obtaining informed consent not only from PLE-prone patients but also from patients with other skin disorders commonly treated by phototherapy.

Pityriasis lichenoides: Long-term follow-up study.

BACKGROUND/OBJECTIVES: Pityriasis lichenoides is an uncommon papulosquamous disorder of unknown etiology. The objective of this study was to review the clinical features and treatment responses of individuals with pityriasis lichenoides seen at a tertiary referral center. METHODS: Seventy-five patients diagnosed with pityriasis lichenoides between 1997 and 2013 were reviewed, and 46 had long-term follow-up via telephone interviews. RESULTS: Fifty (67%) patients were diagnosed with pityriasis lichenoides chronica, 22 (29%) with pityriasis lichenoides et varioliformis acuta, and 3 (4%) with mixed pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta features. Mean ± standard deviation age at onset was 12 ± 13 years (median 8 years). Disease duration was significantly shorter for patients with pityriasis lichenoides et varioliformis acuta (35 ± 35 months) than for those with pityriasis lichenoides chronica (at least 78 ± 48 months). At long-term follow-up, 23 of 28 (82%) patients with pityriasis lichenoides chronica and 3 of 16 (19%) with pityriasis lichenoides et varioliformis acuta had active disease. None progressed to lymphomatoid papulosis or cutaneous T-cell lymphoma. Ten of 23 active pityriasis lichenoides chronica cases had residual pigmentary change independent of race and lasted at least 35 ± 20 months. The most effective treatments were phototherapy (47% response rate), heliotherapy (33%), topical corticosteroids (27%), and antibiotics (25%). CONCLUSION: Pityriasis lichenoides is a predominantly pediatric disorder. The time course of pityriasis lichenoides chronica is significantly longer than that of pityriasis lichenoides et varioliformis acuta. Pityriasis lichenoides chronica may persist with pigmentary alterations in the absence of other signs of active inflammation. Treatment response is often limited, particularly for patients with pityriasis lichenoides chronica.

Seasonal variations in dermatologic and dermatopathologic diagnoses: a retrospective 15-year analysis of dermatopathologic data.

BACKGROUND: Certain dermatologic conditions are known to show seasonal variations in frequency, the reasons for which are unclear but in some cases may be attributable to changes in ambient weather conditions. OBJECTIVES: The current study was conducted to determine whether seasonal trends might exist for dermatologic conditions including erythema multiforme, guttate psoriasis, erythema dyschromicum perstans (ashy dermatosis), pityriasis lichenoides, and pityriasis rosea. METHODS: Data were derived from a 15-year retrospective review of electronic records from a large dermatopathology laboratory located in the mid-Atlantic region of the USA. Numbers of diagnoses per month and "per season" were determined. Pairwise comparisons of seasonal data were made using two-sample t-tests with significance set at P ≤ 0.05. RESULTS: Perniosis (chilblains) was significantly more common in winter and spring (P = 0.001). Hand, foot, and mouth disease was statistically more prevalent in summer and autumn (P = 0.028). Erythema multiforme was most common in spring and summer (P = 0.004). Grover's disease was most common in winter and spring (P = 0.000039). Guttate psoriasis was non-significantly more common in winter and spring (P = 0.076). No statistically significant seasonal variation was found for erythema dyschromicum perstans (P = 0.899), pityriasis rosea (P = 0.727), or pityriasis lichenoides (P = 0.366). CONCLUSIONS: This study found statistically significant seasonal trends for several dermatologic conditions. The study was primarily epidemiologic and was not intended to address histopathologic differences that might underlie the seasonal variations observed. However, further investigation of seasonal differences in the histopathology of erythema multiforme may prove interesting.

Pityriasis Lichenoides in Childhood: Review of Clinical Presentation and Treatment Options.

Pityriasis lichenoides (PL) is a skin condition of unclear etiology that occurs not uncommonly in childhood. It is often classified into the acute form, pityriasis lichenoides et varioliformis acuta (PLEVA), and the chronic form, pityriasis lichenoides chronica (PLC). We performed a comprehensive review of the English-language literature using the PubMed database of all cases of childhood PL reported from 1962 to 2014 and summarized the epidemiology, clinical features, treatment options, and prognosis of this condition in children. The proposed etiologies are discussed, including its association with infectious agents, medications, and immunizations and evidence for PL as a lymphoproliferative disorder. We found an average age of PL onset of 6.5 years, with a slight (61%) male predominance. We also found that PLEVA and PLC tend to occur with equal frequency and that, in many cases, there is clinical and histopathologic overlap between the two phenotypes. When systemic therapy is indicated, we propose that oral erythromycin and narrowband ultraviolet B phototherapy should be first-line treatment options for children with PL since they have been shown to be effective and well tolerated. In most cases, PL follows a benign course with no greater risk of cutaneous T-cell lymphoma, although given the rare case reports of transformation, long-term follow-up of these patients is recommended.

Childhood pityriasis lichenoides and oral erythromycin.

Pityriasis lichenoides (PL) is not uncommon in childhood, but current knowledge about the efficacy of oral erythromycin therapy for its treatment in children is limited. To investigate the role of oral erythromycin therapy in the treatment of PL in children, the records of 24 children with PL who had been started on oral erythromycin treatment at our institution between 2005 and 2008 were retrospectively reviewed. The study included 24 patients (14 male, 10 female) with a median age of 7 years (range 2-14) of whom 15 (62.5%) had PL chronica (PLC), six (25%) PL et varioliformis acuta (PLEVA), and three (2.5%) PLEVA-PLC overlap. History of upper respiratory tract infection was reported in 33% (n = 8) of the patients. History of drug intake and vaccination was noted in 20% (n = 5) and 20% (n = 5), respectively. The disease began during spring (30%, n = 7) or fall (30%, n = 7) in the majority of patients. The median duration of the disease was 11 months (range 1-48 months). Fifteen (68.2%) patients had more than 100 lesions. Distribution was diffuse in 82% (n = 18) of the cases and peripheral in the remainder (n = 6). Oral erythromycin was started at a dosage of 30 to 50 mg/kg per day in three to four divided dosages for 1 to 4 months. Good response was recorded in 64% and 73% of patients in the first and second months of therapy, respectively. Response rate rose to 83% in the third month. In those for whom follow-up data were available (n = 16), relapse was recorded in 12.5% (n = 3). Oral erythromycin may be an effective and well-tolerated treatment option for PL in children and should be continued for at least 3 months.

Pitiriasis liquenoide / Pityriasis lichenoides

La Pitiriasis Liquenoide (PL) es una enfermedad de etiología desconocida, que afecta principalmente a niños y adultos jóvenes. Clínicamente se caracteriza por una erupción de pápulas rosas, naranjas o purpúricas que evolucionan hacia vesículas y posterior ulceración con costras hemorrágicas. Suele ser asintomática aunque en determinadas ocasiones puede aparecer intenso prurito, fiebre y alteración del estado general. Se distingue una forma aguda (Pitiriasis liquenoide y varioliforme aguda o enf. de Mucha-Habermann) y una forma crónica (Pitiriasis liquenoide crónica). Las opciones terapéuticas son multiples: corticoides tópicos, antibióticos, UVA, UVB, metotrexate, sulfonas, pentoxifilina…

Pityrisis lichenoides (PL) is a skin disorder of unkown etiology, that mainly affects children and young adults. Its characterized by eruptions of pink,orange or purpuric papules that undergo central vesiculation, may ulcerate and resolve with haemorrhagic crusts.The eruption may be asymptomaticor sometimes acompained by severe itching, fever and malaise.Pityriasis lichenoides is perhaps best considered a disease spectrum with acute and chronic types: pityriasis lichenoides et varioliformes acuta (PLEVA)and pityriasis lichenoides chronica (PLC).The treatmen includes topical corticosteroids, oral antibiotic, psoralen,ultraviolet A. UVB, methotrexate, sulphone, pentoxifyline…

A clinical and histopathological study of pityriasis lichenoides.

BACKGROUND: Pityriasis lichenoides is a papulosquamous disorder of unknown etiology with remissions and exacerbations. Histopathology helps greatly in the diagnosis of this condition. AIM: We studied clinical and histopathological features of pityriasis lichenoides in our patients. METHODS: This is a 3-year retrospective and prospective, descriptive study of all patients clinically diagnosed as pityriasis lichenoides and confirmed by histopathology. All patients were studied clinically and histopathologically. RESULTS: There were 51 (30 males and 21 females) cases of pityriasis lichenoides in the study period. The maximum number of cases, 14 (27.45%) were in their second decade of life. Pityriasis lichenoides chronica was diagnosed in 39 cases (76.47%) and pityriasis lichenoides et varioliformis acuta (PLEVA) in 12 cases (23.53%). Histopathologically, basal cell vacuolation and perivascular infiltrate were seen in all the cases. Exocytosis was seen in 45.1% of the cases. All the cases of PLEVA showed lymphocytic vasculitis albeit without fibrinoid deposition in the vessel walls. CONCLUSION: Pityriasis lichenoides is not a rare disorder, and is not a true lymphocytic vasculitis as blood vessel damage and fibrinoid deposition in the blood vessel walls were not seen in this study.

Pityriasis lichenoides in childhood: a retrospective review of 124 patients.

BACKGROUND: Pityriasis lichenoides (PL) occurs in all age groups, although predominantly in younger individuals. OBJECTIVE: We sought to study the clinical features of PL in children followed up at our institution. METHODS: The records of 124 children who were given the diagnosis of PL at our institution between 1993 and 2003 were retrospectively reviewed. RESULTS: PL chronica (PLC) was recorded in 37% of the cases, PL et varioliformis acuta (PLEVA) in 57.3%, and clinical features of both disorders were seen simultaneously in the remaining. The median age of onset was 60 months (range: 6-180 months), although the median age of onset of PLEVA (median: 60 months) was significantly younger than that of PLC (median: 72 months) (P = .03). The age distribution showed peaks at 2 to 3 years (24.8%) and 5 to 7 years (32%). A history of infection or drug intake preceded the skin manifestations in 30% and 11.2% of patients with PLC and PLEVA, respectively. The disease began most commonly during winter (35%) or fall (30%). The median duration was 20 months (range: 3-132 months) in patients with PLC and 18 months (range: 4-108 months) in patients with PLEVA. Involvement was diffuse in 74.2% of the patients, peripheral in 20.2%, and central in the remainder. The disease was recurrent in 77% of the patients (n = 80). Of the patients, 59% had pruritus, whereas 32% reported no symptoms; the remainder had fever, arthralgia, or both. Erythromycin estolate or ethylsuccinate was administered to 79.7% of the affected children; 66.6% of these showed at least a partial response. LIMITATIONS: The analyzed data were collected retrospectively and biopsies were not performed in all patients. CONCLUSIONS: PL is not an uncommon disease in childhood, with age peaks in the preschool and early school-age years. It is usually recurrent, and shows a seasonal variation with onset most often in the fall or winter. In childhood PL, erythromycin is an effective initial treatment choice.

Pityriasis lichenoides and its subtypes.

Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript.

Pityriasis lichenoides and its subtypes / Pityriasis lichenoides and its subtypes

Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript.

Mucha-Habermann disease and its febrile ulceronecrotic variant.

In 1916 Mucha and in 1925 Habermann reported an acute form of pityriasis lichenoides characterized by the abrupt onset of papulovesicular eruptions and gave the name, pityriasis lichenoides et varioliformis acuta (PLEVA) or Mucha-Habermann disease (MH). In 1966, Degos reported a rare febrile ulceronecrotic variant of MH. MH occurs mainly in young adults, while febrile ulceronecrotic Mucha-Habermann's disease (FUMHD) occurs more frequently in children. The etiology of MH remains obscure, but it may be the result of a hypersensitivity reaction to an infectious agent. Although clinical and histologic features of the disease in children are similar to those of adults, more diseases need to be differentiated in pediatric patients. In addition, a number of effective therapeutic options in adults with MH are unsuitable for use in pediatric patients, to whom beginning with oral antibiotics, usually erythromycin, is recommended. A summary of previously reported fifteen cases with FUMHD, including our case, is listed.