Lymphomatoid Papulosis and Other Lymphoma-Like Diseases.

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica are the 2 main subtypes of pityriasis lichenoides. They represent the acute and chronic forms of the disease; both may have clonal T cells. Several treatment modalities are used, but it has been difficult to determine efficacy because of the possibility of spontaneous remission. Cutaneous CD30+ lymphoproliferative disorders constitute many cutaneous T-cell lymphomas and comprise lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma (ALCL). Both have an excellent prognosis. Lymphomatoid papulosis often only requires observation or treatment of symptoms. First-line therapies for primary cutaneous ALCL are surgical excision or radiotherapy.

An Atypical Presentation of PLEVA: Case Report and Review of the Literature

Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare, self-limited, cutaneous disorder of unknown etiology. Clinically, PLEVA is characterized by the sudden onset of scaly, erythematous macules and papules localized to the trunk and proximal extremities. We report the case of a patient who presented with multiple erythematous papules and plaques on the palms, forearms, and dorsal feet. J Drugs Dermatol. 2019;18(7):690-691.

Pityriasis Lichenoid-like Mycosis Fungoides in a 9-year-old Boy: A Case Report.

Dear Editors, Pityriasis lichenoides (PL)-like mycosis fungoides (MF) is a rare variant of MF, presenting clinical findings of PL but histological features of MF. It was first reported by Ko et al. (1) and only a few cases have been reported since (2-5). Herein we report the case of a boy with PL-like MF and review the related literature. A 9-year-old boy presented with a 1-year history of multiple pruritic crusted erythematous papules and scaly pink maculopatches on the face, trunk, and extremities (Figure 1, a and b). Histologic examination of a papule revealed lymphocytic epidermotropism and lymphocytes tagging the dermoepidermal junction. The nuclei of the lymphocytes were hyperchromatic and irregular (Figure 1, c and d). Immunohistochemically, the infiltrating lymphocytes revealed positivity for CD2, CD3, CD5, CD7, and CD8, but were negative for CD4, CD20, CD30, CD68, and CD163 (Figure 1, e-g). T-cell receptor gene rearrangement analysis (TCR-GRA) demonstrated the rearrangement of the gamma chain (Figure 1, h). PL-like MF was diagnosed. The patient was started on narrowband ultraviolet B (NBUVB) phototherapy. The skin lesions markedly improved after 6 months of treatment. There was no recurrence during the 2 years of follow-up. There has long been a controversy regarding whether PL is just an inflammatory dermatosis or a genuine T-cell lymphoproliferative disease. Wang et al. (2) proposed three categories for the relationship between PL and MF: (A) PL with a dominant T-cell clone, (B) PL subsequently progressing into MF, and (C) PL-like MF. In the first category, PL is a monoclonal T-cell-mediated inflammatory disorder, in which T-cell clones were found in about 50% of patients (6,7). The second category involves progression from long-term PL to MF (8,9). The average time-to-progression is about 8 years. It has been speculated that the PL-related immunologic microenvironment is favorable for developing a tumoral clone. Our patient presented with PL-like lesions clinically, while biopsy findings, results of immunohistochemistry, and TCR-GRA all suggested that this case was MF. Due to the short duration (only one year) of his lesions, we established the diagnosis of PL-like MF de novo, rather than evolution from PL to MF. The features of previously reported cases of PL-like MF and those of our patient are summarized in Table 1 (1-5). Men were predominant (18:7) among the total of 25 patients. Most patients were children or young adults (mean age of 23.4 years).The interval between presence of lesions and diagnosis varied from 1 month to 10 years. The cutaneous eruptions were all PL in appearance and almost all involved both the trunk and extremities. Pruritus was reported by approximately half of the patients. Histologically, the scaly papules were usually indistinguishable from classical MF, showing epidermotropism, haloed lymphocytes, lymphocytes aligning along the dermoepidermal junction, and Pautrier's microabscesses. Immunohistochemically, all tested cases demonstrated positivity for CD3 but were negative for CD20 and CD30. Cases with predominantly CD8-positive cells were twice as prevalent as cases with predominantly CD4-positive cells. TCR-GRA was performed in 20 cases, 15 of which revealed monoclonality. Most patients received psoralen combined with ultraviolet A or NBUVB phototherapy, and demonstrated either a complete or partial response. Recurrence was reported in only 2 cases (5). In summary, PL-like MF is a rare variant of MF. It has some features distinct from classic MF, such as a higher incidence in young men and predominantly CD8-positive T-cells infiltration. Phototherapy can be used as the first line of treatment. A good response and a favorable prognosis can be expected.

[Pityriasis lichenoides: not always a clear-cut diagnosis!] / Pithyriasis lichénoïde: diagnostic pas toujours aussi facile!

Pityriasis lichenoides is a rare inflammatory dermatosis occurring in young subjects. It evolves into rashes on the trunk and the limbs which regress spontaneously. We here report a particular case of pityriasis lichenoides whose diagnosis was adjusted due to the scar appearance of the lesions. The patient was treated with cyclin and phototherapy, with a favorable outcome.

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.

Use of Phototherapy in Children.

BACKGROUND: Phototherapy is a well-recognized treatment in adults and children. Previous articles have reported success in treating recalcitrant skin disorders such as atopic dermatitis (AD), psoriasis, pityriasis lichenoides chronica, and vitiligo in children. METHODS: This was a retrospective review over an 18-month period from June 2012 to December 2013 of all children receiving phototherapy in a tertiary pediatric dermatology center. RESULTS: Seventy-five patients 3 to 17 years of age (mean 10.6 years; 35 male, 40 female) were included. Forty-eight (64%) patients had AD and 21 (28%) had psoriasis. Seventy received narrowband ultraviolet B (NBUVB) treatment and five received hand and foot psoralen and ultraviolet A (PUVA) treatment. All patients with AD were treated with NBUVB and four (8.3%) were also treated with hand PUVA. After phototherapy, 76% had documented clear to almost clear skin. At the 12-month follow-up, 52% of the patients with AD remained clear. All 21 patients with psoriasis underwent NBUVB phototherapy. The clearance rate after phototherapy was 86%. At the 12-month follow-up, 43% of the patients with psoriasis remained clear. CONCLUSION: Phototherapy can reduce disease burden in individuals with severe AD and psoriasis and should be considered as a second-line therapy if standard topical regimens are unsuccessful.

Phototherapy for Pityriasis Lichenoides in the Pediatric Population: A Review of the Published Literature.

BACKGROUND: Pityriasis lichenoides (PL) is a dermatologic disorder that manifests in either the acute (pityriasis lichenoides et varioliformis acuta) or the chronic form (pityriasis lichenoides chronica, also known as parapsoriasis chronica). Traditional first-line therapy consists of corticosteroids or antibiotics; however, these treatments are often accompanied with multiple side effects and may be ineffective. OBJECTIVE: The goal of this study was to review the use of phototherapy for treating PL in the pediatric population. MATERIALS AND METHODS: We performed a systematic review of the literature in the National Library of Medicine's PubMed database and the SCOPUS database discussing phototherapy for treatment of PL in the pediatric population. The following search terms were used: 'pityriasis lichenoides', 'pityriasis lichenoides chronica', 'pityriasis lichenoides et varioliformis acuta', and 'febrile ulceronecrotic Mucha-Habermann disease'. RESULTS: The systematic search and screening of articles resulted in 14 articles including a total of 64 patients with PL treated with phototherapy. Three different modalities were utilized, with five studies using broadband ultraviolet B (BB-UVB) radiation, nine studies utilizing narrowband UVB (NB-UVB), and two studies employing psoralen with ultraviolet A (PUVA) therapy. Overall, the use of BB-UVB had an initial clearance rate of 89.6 % with 23.1 % recurrence, whereas NB-UVB cleared 73 % of the lesions with no recurrence, and PUVA therapy initially cleared 83 % of the lesions with 60 % recurrence. The side-effect profiles were similar and revealed limited toxicity. CONCLUSION: Phototherapy shows promising results and a favorable side-effect profile in the treatment of PL. Ultimately, large randomized controlled trials are needed to determine optimal treatments.

Pityriasis lichenoides chronica presenting with bilateral palmoplantar involvement.

Pityriasis lichenoides is an uncommon, acquired, idiopathic, self-limiting skin disease that poses a challenge to patients and clinicians to diagnose and treat. Several variants exist including pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC), and febrile ulcer-onecrotic Mucha-Habermann disease. Precise classification can be difficult due to an overlap of clinical and histologic features. In this case report we describe a patient with a rare presentation of PLC exhibiting bilateral palmoplantar involvement and mimicking psoriasis. We review the literature and discuss the clinical course, pathogenesis, and current treatment modalities of PLC.

Pitiriasis liquenoide crónica inducida por adalimumab en paciente con psoriasis y buena respuesta a metotrexato / Adalimumab-Induced Pityriasis Lichenoides Chronica That Responded Well to Methotrexate in a Patient With Psoriasis

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[Pityriasis Lichenoides: Case report and review of the literature]. / Pitiriasis liquenoide: presentación de un caso clínico y revisión de la literatura.

INTRODUCTION: Pityriasis lichenoides is a benign inflammatory disease of unknown etiology. There are two types of this condition: an acute form (PLEVA = pityriasis lichenoides et varioliformis acuta) and a chronic one (PLC = pityriasis lichenoid chronica). Both are more common in children and young adults. OBJECTIVE: To describe a case of PLC, discuss its clinical presentation, diagnosis, treatment and present a review of the literature. CASE REPORT: A seven-year-old child who presented with recurrent oligosymptomatic episodes of bright erythematous papular lesions in centripetal distribution, which subsided and left behind hypopigmented macules. The biopsy of the lesions confirmed chronic pityriasis lichenoid. During the 3 years of follow-up, multiple treatment regimens were used to reduce exacerbations, resulting in a partial response. CONCLUSION: PLC is a rare disease that represents a diagnostic and therapeutic challenge to the physician. The diagnosis of this condition is suspected clinically and confirmed by histology. It does not have specific treatment, but it responds well to corticosteroids, antibiotics, immunosuppressants and phototherapy with UVB narrowband (UVBnb). The latter has given the best results. It is important to monitor patients for risk of developing lymphoproliferative disorders.

Pitiriasis liquenoide: presentación de un caso clínico y revisión de la literatura / Pityriasis Lichenoides: Case report and review of the literature

Introducción: La pitiriasis liquenoide es una enfermedad inflamatoria benigna de causa desconocida. Tiene dos formas de presentación: una forma aguda (PLEVA, pitiriasis liquenoide y varioliforme aguda) y otra crónica (PLC, pitiriasis liquenoide crónica). Ambas son más frecuentes en niños y adultos jóvenes. Objetivo: Describir un caso de PLC, comentar su presentación clínica, diagnóstico y tratamiento, y revisar la literatura. Caso clínico: Escolar de 7 años que presentó episodios recurrentes de lesiones tipo pápulas eritematocostrosas brillantes de distribución centrípeta, oligosintomáticas, que desaparecían dejando máculas hipopigmentadas. La biopsia de las lesiones confirmó una pitiriasis liquenoide crónica. Durante los 3 años de seguimiento se realizaron múltiples esquemas de tratamiento para atenuar las reagudizaciones, logrando una respuesta parcial. Conclusión: La PLC es una enfermedad infrecuente que representa un desafío diagnóstico y terapéutico para el médico. El diagnóstico de esta entidad se sospecha por la clínica y se confirma con la histología. No tiene tratamiento específico pero presenta buena respuesta a corticoides, antibióticos, inmunosupresores y fototerapia UVB de banda angosta (UVB-nb). Esta última es la que ha reportado los mejores resultados. Es importante el seguimiento de los pacientes por el riesgo de desarrollar enfermedades linfoproliferativas.

Introduction: Pityriasis lichenoides is a benign inflammatory disease of unknown etiology. There are two types of this condition: an acute form (PLEVA = pityriasis lichenoides et varioliformis acuta) and a chronic one (PLC = pityriasis lichenoid chronica). Both are more common in children and young adults. Objective: To describe a case of PLC, discuss its clinical presentation, diagnosis, treatment and present a review of the literature. Case Report: A seven-year-old child who presented with recurrent oligosymptomatic episodes of bright erythematous papular lesions in centripetal distribution, which subsided and left behind hypopigmented macules. The biopsy of the lesions confirmed chronic pityriasis lichenoid. During the 3 years of follow-up, multiple treatment regimens were used to reduce exacerbations, resulting in a partial response. Conclusion: PLC is a rare disease that represents a diagnostic and therapeutic challenge to the physician. The diagnosis of this condition is suspected clinically and confirmed by histology. It does not have specific treatment, but it responds well to corticosteroids, antibiotics, immunosuppressants and phototherapy with UVB narrowband (UVBnb). The latter has given the best results. It is important to monitor patients for risk of developing lymphoproliferative disorders.

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.

Febrile ulceronecrotic Mucha-Habermann disease (pityriasis lichenoides et varioliformis acuta fulminans) associated with parvovirus infection.

Febrile ulceronecrotic Mucha-Habermann disease is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. It carries a great morbidity and is potentially fatal. The exact pathogenesis is not clear, and it has been proposed to be the result of hypersensitivity reaction to an infection. We report a patient with febrile ulceronecrotic Mucha-Habermann disease in a 12-year-old boy in whom the condition was most likely precipitated by parvovirus infection, and he showed a favorable response to a combination of prednisolone with narrow band ultraviolet B (NB-UVB) phototherapy.

Phototherapy for pityriasis lichenoides: our experience.

Pityriasis lichenoides is a benign disease that includes a continuous spectrum with two polar ends: pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC). Although its benign and self-limited character, treatment is required, both for itch relief and for cosmetic issues. The present study is a retrospective analysis of 13 patients (11 PLC and 2 PLEVA) treated in our institution with psoralen plus ultraviolet A (PUVA) or ultraviolet A combined with ultraviolet B (UVA/UVB) during the period 1998-2011. In the PUVA group, complete response was achieved in five patients and partial response in two. Total cumulative UVA dose was 84.4 J/cm(2). One patient quit therapy without therapeutic response. In the UVA/UVB group, complete response was achieved in two patients and partial response in an equal number of patients. One patient did not reach a significant improvement. Total cumulative doses were: 26.1 J/cm(2) for UVA and 3.62 J/cm(2) for UVB. There were no acute side effects in either therapeutic group. In the present study, PUVA phototherapy was preferred for patients with more widespread or long-evolving disease, while UVA/UVB was selected for patients who presented more recent disease or contraindications for PUVA therapy. Regardless of the absence of clinical guidelines, both therapeutic options proved to be successful, ascertaining phototherapy as an effective and safe option for pityriasis lichenoides patients.

Phototherapy in childhood.

BACKGROUND: Little data exist about the efficacy of phototherapy in childhood dermatoses. OBJECTIVE: To report our experience with pediatric patients treated with phototherapy. METHODS: The study included children

Pityriasis lichenoides: the differences between children and adults.

BACKGROUND: Pityriasis lichenoides (PL) is a skin disease that affects both children and adults. Anecdotally, it is said to run a more benign course in children, with a frequent tendency to self-resolution. However, to our knowledge, there have been no published studies comparing PL in both age groups. OBJECTIVE: To evaluate the clinicopathological features, overall efficacy of treatments and disease outcomes in children and adults diagnosed with PL. METHODS: A retrospective review of records was undertaken on all patients diagnosed with PL at two regional centres during an 8-year period (from 1998 to 2006). For each individual, data were collected on age, sex, number of lesions, lesional morphology and distribution, symptoms, histopathological features, treatment modalities (and response), overall follow-up and length of remission. RESULTS: We recorded 25 children (median age 8 years, range 2-18) and 32 adults (median age 40 years, range 20-65) with PL. All the children and adults had more than 20 scaly, papular lesions. Children had greater lesional body involvement than adults. Lesions on the legs and trunk were present in 23 children (92%) compared with 19 adults (59%) (P < 0.01) and facial involvement was observed more commonly in children (n = 10, 40%) compared with adults (n = 8, 25%). Dyspigmentation was significantly more common in children (n = 18, 72%) compared with adults (n = 6, 19%) (P < 0.001). Topical corticosteroids were used by 16 children (64%) and 18 adults (56%) but only half in each group found these effective. Eight children (32%) were treated with erythromycin, with only two (25%) clearing, and one of these subsequently relapsing. In contrast, four adults (13%) received antibiotics, with three (75%) clearing and none relapsing thereafter. Ultraviolet B phototherapy was used in eight children (32%), with seven (88%) completely or almost clearing, but four (57%) subsequently relapsed. Fourteen adults (44%) received phototherapy; 10 (71%) completely cleared and only two of these (20%) subsequently relapsed. Strikingly, after a median disease duration of 30 months, only five children (20%) went into complete remission compared with 25 adults (78%) (P < 0.001). CONCLUSIONS: This is the first study to compare PL in children and adults. Our findings suggest that, compared with adults, PL in children is more likely to run an unremitting course, with greater lesional distribution, more dyspigmentation and a poorer response to conventional treatment modalities.

Photoresponsive diseases.

Photoresponsive diseases are skin diseases or conditions that are known to respond with partial or complete clearing to ultraviolet exposure. Phototherapy may be used as a single therapy or as combination therapy with other treatments. Health care professionals providing phototherapy treatment are responsible for educating patients regarding the benefits and risks of phototherapy.

Pityriasis lichenoides: pathophysiology, classification, and treatment.

Pityriasis lichenoides (PL) is an uncommon, acquired spectrum of skin conditions that poses various challenges to patients as well as clinicians. It is a difficult and debatable disorder to diagnose, categorize, and treat. Besides these inherent obstacles, PL merits awareness because of its potential to progress to cutaneous lymphoma or an ulceronecrotic presentation, both of which carry a significant risk of mortality. The scope of PL presentations is delineated along a continuum of multiple variants including pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC), and febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Classification of these presentations as separate subsets is debatable in view of their overlapping clinical, histopathologic, and etiologic features. PLEVA generally presents as an acute-to-subacute skin eruption of multiple, small, red papules that develops into polymorphic lesions and vacillates with periods of varying remissions as well as possible sequelae of hyper/hypopigmentation and varicella-like scars. PLC has a more gradual manifestation of very small red-to-brown flat maculopapules with mica-like scale; it also follows a relapsing course but with long periods of remission. FUMHD is an acute and severe generalized eruption of purpuric and ulceronecrotic plaques with associated systemic involvement and a mortality rate of up to 25%; hence, it should be approached as a dermatologic emergency.Histopathological evaluation of PL usually reveals dermal, wedge-shaped, lymphocytic infiltrate, epidermal spongiosis, parakeratosis, and variable necrosis of keratinocytes. PLC demonstrates more subtle histology whereas, at the other end of the spectrum, febrile ulceronecrotic FUMHD exhibits the most exaggerated histological features. The pathogenic mechanism behind PL is unclear although infectious or drug-related hypersensitivity reactions versus premycotic lymphoproliferative disorder are the mainstay theories. The foremost therapies for PLEVA and PLC are phototherapy, systemic antibacterials, and topical corticosteroids. Aggressive treatment with immunosuppressant and/or immunomodulating agents as well as intensive supportive care are recommended for FUMHD. We first describe a representative case of a 14-year-old boy with PLC who was successfully treated with narrow-band UVB. We then review the pathophysiology, classification, and treatment of PL.

Pitiriasis liquenoide localizada / Localized pitiryasis lichenoides

La pitiriasis liquenoide es una enfermedad papulodescamativa de etiología desconocida frecuente en la edad pediátrica. Las lesiones suelen distribuirse de manera difusa en el tronco y las extremidades, y sólo excepcionalmente se han descrito casos de afectación localizada en un área limitada del cuerpo. Presentamos el caso de un niño de 9 años con brotes recurrentes de pitiriasis liquenoide de localización exclusiva en la zona inferior del abdomen

Pitiryasis lichenoides is a papulosquamous disorder of unknown etiology frequently seen in the pediatric population. The lesions are usually widespread on the trunk and extremities, and only exceptional cases of localized forms have been reported. We report a 9-year-old patient with recurrent crops of pitiryasis lichenoides lesions exclusively involving the lower abdomen

Phototherapy in pediatric patients.

The treatment of children with psoriasis, atopic dermatitis (AD), pityriasis lichenoides, and scleroderma poses a therapeutic problem because all therapeutic options are associated with numerous side effects. Therefore ultraviolet A and B (UVA and UVB) phototherapy is presented as a possible alternative to some of these therapies, primarily topical and systemic corticosteroids, in children. Our results in treating children with phototherapy and psoralen plus UVA (PUVA) bath phototherapy over the past 5 years are reported. UVB therapy (TL01) was used in 20 psoriatic children (6 boys, 14 girls; ages 6-14 years) during the stage of disease exacerbation and in 9 children (3 boys, 6 girls; ages 8-16 years) with pityriasis lichenoides. Combined UVA/UVB phototherapy was applied in 21 AD children (7 boys, 14 girls; ages 4-15 years). Photochemotherapy with local application of a PUVA bath was used in six children (2 boys, 4 girls; ages 9-16 years) with circumscribed scleroderma and in one girl with systemic scleroderma. All children received short courses of phototherapy with either no maintenance or short maintenance. All three therapeutic protocols resulted in a certain degree of improvement in most of the study patients. None of the patients exhibited any early phototherapy side effects. We conclude that phototherapy and PUVA bath are valuable and safe therapeutic options for selected children who do not respond to other treatments.