PUVA-induced pityriasis lichenoides chronica-like papular lesions in patients with mycosis fungoides: a clinical, histopathological and immunohistochemical study.

Mycosis fungoides (MF) is the most common form of cutaneous T cell lymphoma (CTCL) with many clinical variants including papular and pityriasis lichenoides chronica (PLC)-like variants. During psoralen and ultraviolet A (PUVA) treatment of MF, PLC-like papular lesions were observed to appear. The exact nature of these lesions is not fully understood. This work aimed to study PLC-like papular lesions arising in MF patients receiving PUVA therapy clinically, histopathologically and immunohistochemically (using monoclonal antibodies against CD4 and CD8) and to compare them with lesions in classic PLC patients. Fifteen MF patients with PLC-like papular lesions arising during PUVA treatment were included and 15 patients with classic PLC served as controls. While the extent of these lesions significantly correlated with their duration (p < 0.05), it showed no significant correlation with the TNMB stage of MF, number of phototherapy sessions or cumulative UVA dose at which they started to appear. The response status of MF to PUVA did not affect their development. Compared to classic PLC, these lesions showed significantly more acute onset (p = 0.003). None of these lesions showed histopathological features essential to diagnose papular/PLC-like MF and no significant difference existed with regard to their histopathological and CD4/CD8 phenotypic features compared to classic PLC. Papular lesions mimicking PLC in MF patients receiving PUVA mostly represent an upgrading reaction with possible good prognostic implication.

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.

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.

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.

Successful therapy of cyclosporin A in pityriasis lichenoides et varioliformis acuta preceded by hand, foot and mouth disease.

To our knowledge, there are no previously published cases of enteroviral infection complicated by pityriasis lichenoides et varioliformis acuta (PLEVA). A 30-year-old woman is reported with a severe form of PLEVA, preceded by hand, foot and mouth disease. Immunosuppressive treatment with cyclosporin A resulted in rapid clinical improvement.

Pityriasis lichenoides et varioliformis acuta after influenza vaccine.

The etiology of pityriasis lichenoides is unknown. One of the accepted theories admits that PL is an inflammatory response to extrinsic antigens such as infectious agents, drugs and vaccines. In recent medical literature, only the MMR vaccine (Measles, Mumps and Rubella) was associated with the occurrence of this disease. We present a case of a male, 12 year old healthy patient who, five days after Influenza vaccination, developed erythematous papules on the trunk, abdomen and limbs, some with adherent crusts and associated systemic symptoms. This case report is notable for describing the first case of pityriasis lichenoides et varioliformis acuta associated with the vaccine against Influenza.

Pityriasis lichenoides et varioliformis acuta after influenza vaccine

The etiology of pityriasis lichenoides is unknown. One of the accepted theories admits that PL is an inflammatory response to extrinsic antigens such as infectious agents, drugs and vaccines. In recent medical literature, only the MMR vaccine (Measles, Mumps and Rubella) was associated with the occurrence of this disease. We present a case of a male, 12 year old healthy patient who, five days after Infl uenza vaccination, developed erythematous papules on the trunk, abdomen and limbs, some with adherent crusts and associated systemic symptoms. This case report is notable for describing the first case of pityriasis lichenoides et varioliformis acuta associated with the vaccine against Influenza.

Pitiriasis liquenoide y varioliforme aguda / Pityriasis lichenoides et varioliformis acuta

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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.

Pitiriasis liquenoide: descripción de un caso / Pityriasis lichenoides: description of a case

La pitiriasis liquenoide (PL) es una dermatosis inflamatoria adquirida, idiopática, infrecuente, benigna y autoinvolutiva, que tiene dos variantes: aguda y crónica, con base en las diferencias morfológicas, la evolución temporal y el curso de este trastorno. Éstas pueden considerarse como los dos extremos clínicos dentro del espectro de una misma enfermedad. Clínicamente la PL se caracteriza por el desarrollo de brotes recurrentes de pápulas eritemato-descamativas liquenoides, de superficie queratósica, que pueden persistir por semanas o meses (crónica), o bien brotar y recidivar con exacerbaciones agudas acompañadas, frecuentemente, por vésico-pústulas, ulceración, hemorragias y costras (aguda), que curan dejando cicatrices atróficas varioliformes. Presentamos el caso de un escolar de 9 años de edad con una PL con escasas costras hemáticas puntiformes y con máculas hipopigmentadas residuales.

Pityriasis lichenoides (PL) is an inflammatory acquired idiopathic, rare, benign and self-healing dermatosis, which has two variants: acute and chronic, based on morphological differences, evolution and course of the disorder that can be regarded as clinical extremes within the spectrum of the same disease. It is characterized by the development of recurrent outbreaks of scaly erythematous lichenoid papules, with keratotic surface, which can persist for weeks or months (chronic) or sprout with acute exacerbations frequently accompanied by vesico-pustules, ulceration, bleeding and crusting (acute) that heal leaving atrophic varioliformis scars. We report the case of a 9 years old boy who developed pityriasis lichenoides with few punctate hematic crusts and residual hypopigmented macules.

Febrile ulceronecrotic Mucha-Habermann's disease with pulmonary involvement.

We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 12-year-old boy. After 1 week of typical cutaneous appearance of pityriasis lichenoides et varioliformis acuta (PLEVA), he was given a percutaneous injection of measles virus vaccine, and then extensive polymorphous, papular, and ulcerohemorrhagic skin lesions developed along with intermittent high temperature, hepatic dysfunction, and hypoalbuminemia. Thoracic CT scan showed parenchymal nodular infiltration at the tip of vascular structures. Skin biopsy showed a combination of the classic features of PLEVA and an allergic vasculitis. We treated him with prednisolone and methotrexate (MTX) successfully. The evolution in our patient suggests that FUMHD may have been triggered by virus vaccination. We also emphasize that FUMHD in children may be successfully treated with a combination of systemic MTX and high-dose corticosteroids.

Presentación atípica de pitiriasis liquenoide aguda, reporte de un caso / Atypic presentation of pityriasis lichenoides et varioliformis acuta, a case report

Las pitiriasis liquenoides (PL) son dermatosis polimórficas adquiridas poco comunes que plantean diversos retos, ya que son entidades difíciles de diagnosticar, categorizar y tratar. La pitiriasis liquenoide varioliforme aguda (PLEVA), la pitiriasis liquenoide crónica (PLC) y la enfermedad febril ulceronecrótica de Mucha-Habermann (FUMHD) no son patologías diferentes, por el contrario se consideran manifestaciones diversas dentro de un mismo espectro. Desde el punto de vista histológico son consideradas dermatitis de interfase con infiltrado prominente de linfocitos. La ambigüedad más crítica dentro de este grupo de patologías es la etiología, ya que múltiples agentes han sido implicados sin lograr revelar el mecanismo patológico de esta condición. No existe una modalidad definida de tratamiento; se cree que la terapia combinada es el mejor abordaje para esta patología. Reportamos un caso de un paciente con pitiriasis liquenoide aguda cuya presentación inicial fue atípica lo que nos lleva a considerar diagnósticos como pitiriasis rosada o secundarismo luético.