Acute lichenoid and varioliform pityriasis in a pediatric patient.

BACKGROUND: Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare dermatosis recognized as a benign condition of unknown etiopathogenesis. It is more common in pediatric patients and young adults and is characterized by multiple small or large erythematous plaques spread over the trunk and extremities. CASE REPORT: We describe the case of a 5-year-old male, previously healthy, with multiple erythematous lesions that disappeared leaving hypopigmented macules. The biopsy reported histological changes suggestive of mycosis fungoides. After a second revision of lamellae in this hospital, lymphocytic vasculitis (LV) with focal epidermal necrosis consistent with acute pityriasis lichenoides (PL) was identified. CONCLUSIONS: The existing knowledge about PLEVA lacks a consensus in specifying its classification, etiopathogenesis, diagnosis, and treatment, so this clinical condition represents a medical challenge. The diagnosis is made by clinical suspicion and confirmed by histology. The objective of this article was to report a case of PLEVA with an atypical presentation due to its histopathological findings, being the first report showing LV in children, as well as a review of the literature.

INTRODUCCIÓN: La pitiriasis liquenoide y varioliforme aguda (PLEVA) es una dermatosis poco frecuente, de etiopatogenia desconocida y evolución autolimitada. Es más común en pacientes pediátricos y adultos jóvenes, y está caracterizada por la presencia de múltiples placas eritematoescamosas pequeñas o grandes, diseminadas en el tronco y las extremidades. CASO CLÍNICO: Se describe el caso de un escolar de 5 años, de sexo masculino, previamente sano, que presentó múltiples cuadros de lesiones eritematosas que desaparecían dejando máculas hipopigmentadas. La biopsia reportó cambios histológicos sugestivos de micosis fungoide. Se realizó una segunda revisión de laminillas, identificando vasculitis linfocítica con necrosis epidérmica focal, consistente con pitiriasis liquenoide aguda. CONCLUSIONES: El conocimiento acerca de la PLEVA carece de un consenso que especifique su clasificación, etiopatogenia, diagnóstico y tratamiento, por lo que esta condición clínica representa un desafío médico. El diagnóstico se realiza por sospecha clínica y se confirma por histología. El objetivo de este artículo fue reportar un caso de PLEVA con presentación atípica por los hallazgos histopatológicos, siendo este el primer reporte de vasculitis linfocítica en niños, y además se realiza una revisión de la literatura.

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.

A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities.

Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose as in the early course they can mimic erythema multiforme or lymphomatoid papulosis. We report a case of a 38-year-old woman who presented with 90% body surface area involvement, fever, diarrhea, malaise and associated comorbidities. She was treated with systemic steroids and methotrexate but suffered a fatal outcome. So far, a total of 65 cases are reported in the literature.

The color of skin: white diseases of the skin, nails, and mucosa.

White diseases are a heterogenous group characterized by hypopigmentation or depigmentation. Skin and eye color are determined by the number and size of melanosomes present. Melanin is produced by melanosomes in the melanocytes present within the epidermis of the skin, uvea, and retinal pigmented epithelium (RPE). Conditions altering the number of melanocytes or concentration of melanin result in a lack of pigmentation, appearing as "white diseases" ranging from the well-known albinism and vitiligo to more esoteric white hand syndrome and Degos disease.

[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.

Severe Mucha-Habermann-Like Ulceronecrotic Skin Disease in T-Cell Acute Lymphoblastic Leukemia Responsive to Basiliximab and Stem Cell Transplant.

A 5-year-old girl with T-cell acute lymphoblastic leukemia (T-ALL) developed a progressive eruption of crusted papules and ulcerative plaques involving 80% of her body surface area with histopathology consistent with febrile ulceronecrotic Mucha-Habermann disease (FUMHD), although multiple specimens also contained clonal leukemic cells. Her skin disease was refractory to many classic treatments for FUMHD, including methotrexate, and became so severe that concern about superinfection prevented intensification of chemotherapy for her malignancy. The addition of basiliximab promoted gradual improvement of the skin, allowing for chemotherapy intensification and subsequent bone marrow transplantation, after which the eruption resolved completely. This report describes a severe case of FUMHD-like eruption associated with clonal leukemic cells that improved with basiliximab, suggesting anti-CD25 therapy as a novel treatment for ulceronecrotic skin disease in the setting of high interleukin-2 levels.

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.

[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.

Extreme dermatology--the intensive care skills of dermatologists in three case presentations of acute skin failure.

Dermatological emergencies include a number of clinical conditions usually accompanied by systemic symptoms that can lead to life-threatening complications. From the broad spectrum of life-threatening dermatoses, three cases are presented: a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a case of pemphigus vulgaris mimicking Stevens-Johnson syndrome (SJS), and a case of toxic epidermal necrolysis (TEN). Those cases were considered extreme, and presented to illustrate the positive outcome of timely intensive dermatological care. An interdisciplinary approach is essential in the diagnosis, treatment, management, and follow up of patients with life-threatening dermatoses.