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 y varioliforme aguda / Pityriasis lichenoides et varioliformis acuta

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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

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.

The role of cytotoxic skin-homing CD8+ lymphocytes in cutaneous cytotoxic T-cell lymphoma and pityriasis lichenoides.

BACKGROUND: Pityriasis lichenoides (PL) is a rare cutaneous lymphoproliferative disorder of unknown origin. Malignant transitions of PL have been described, but are very rare. We recently observed the fatal course of a 26-year-old patient who presented with a clinical picture resembling PL but had cytotoxic CD8+ T-cell lymphoma of the skin (CxCTL). This case prompted us to reinvestigate the role of cytotoxic T lymphocytes in PL and its relationship to antiviral immunity. METHODS: Skin biopsy specimens of 11 patients with PL and two biopsy specimens of CxCTL were included. In all, 5 biopsy specimens taken from healthy skin and 5 samples of varicella-zoster virus (VZV) skin lesions were analyzed for control purposes. The inflammatory infiltrate was characterized by immunohistochemistry using monoclonal antibodies against CD3, CD4, CD8, CD20, cutaneous lymphocyte-associated antigen (CLA), CCR4, CXCR3, Granzyme B, Tia-1, and MxA. Flow cytometry was used to analyze the expression of chemokine receptors on peripheral blood mononuclear cells in CxCTL. RESULTS: The CxCTL skin lesions were dominated by a dense infiltration of CD8+ cytotoxic lymphocytes with a skin-homing CLA+ CCR4+ phenotype. PL and VZV skin lesions were also characterized by a predominantly CD8+ T cellular infiltrate with strong expression of the cytotoxic molecules Granzyme B and Tia-1 and the skin-homing molecules CLA and CCR4. Coexpression analyses confirmed that skin CLA+ CD8+ cytotoxic T cells are present in CxCTL, VZV, and PL skin lesions. Strong lesional production of the antiviral protein MxA, which is specifically induced by type I interferons, could be found in all investigated disorders. The study was based on histologic, immunohistologic, and flow cytometric analyses in a limited number of patients, because of the rareness of the investigated diseases. CONCLUSION: Our results revealed a striking similarity between the immunohistologic picture of malignant CxCTL, benign PL, and VZV skin lesions. Strong expression of the antiviral protein MxA in all disorders supports the view that a common antiviral immune response pattern leads to aberrant skin recruitment of CLA+ CCR4+ cytotoxic T lymphocytes in PL and CxCTL.

Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta characterized by high fever and papulonecrotic skin lesions. Here we report a case of a 14-year-old boy with typical features of FUMHD and unusual manifestation of extensive skin necrosis in intertriginous regions including axillae, neck, inguinal and antecubital areas. Systemic administration of corticosteroid and erythromycin led to rapid healing of ulcerations without residual scar formation. Review of the literature showed male-predominance and favorable outcome in pediatric cases of FUMHD.

Pitiriasis liquenoide: reporte de once casos / Pityriasis lichenoides: eleven cases report

La pitiriasis liquenoide (PL) es una enfermedad poco frecuente y de etiología desconocida. Tiene dos formas clínicas que constituyen puntos extremos de presentación: PL aguda y crónica. El diagnóstico está basado en la sospecha clínica y el estudio histopatológico; no se dispone hasta el momento de un tratamiento curativo. El seguimiento periódico de estos pacientes es fundamental debido a la posibilidad de transformación hacia procesos linfoproliferativos. Presentamos once pacientes con diagnóstico clinicopatológico de PL, nueve de los cuales eran de sexo femenino. En cuatro casos pudimos deducir el motivo de las confusiones diagnósticas que demoraron el diagnóstico de estas presentaciones inusuales (AU)

Pitiriasis liquenoide: reporte de once casos / Pityriasis lichenoides: eleven cases report

La pitiriasis liquenoide (PL) es una enfermedad poco frecuente y de etiología desconocida. Tiene dos formas clínicas que constituyen puntos extremos de presentación: PL aguda y crónica. El diagnóstico está basado en la sospecha clínica y el estudio histopatológico; no se dispone hasta el momento de un tratamiento curativo. El seguimiento periódico de estos pacientes es fundamental debido a la posibilidad de transformación hacia procesos linfoproliferativos. Presentamos once pacientes con diagnóstico clinicopatológico de PL, nueve de los cuales eran de sexo femenino. En cuatro casos pudimos deducir el motivo de las confusiones diagnósticas que demoraron el diagnóstico de estas presentaciones inusuales

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.

Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature.

A 32-year-old male with febrile ulceronecrotic Mucha-Habermann disease (FUMHD) responsive to methotrexate is reported. This is a severe variant of pityriasis lichenoides et varioliformis acuta characterized by the acute onset of a widespread ulceronecrotic cutaneous eruption together with high fever and systemic involvement. To our knowledge, only 13 patients with FUMHD have been reported to date.