ABSTRACT
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.
ABSTRACT
La pitiriasis liquenoide crónica (PLC) es una enfermedad benigna, que se caracteriza por tener un curso gradual, con aparición de múltiples pápulas con escamas adherentes de predominio en tronco y extremidades proximales. Con el objetivo de describir la presentación clínica, discutir el diagnóstico, diagnósticos diferenciales, tratamiento y revisar la literatura se presenta el caso de un hombre de 88 años que presentó múltiples lesiones papuloescamosas en extremidades inferiores. Se realizó biopsia cutánea que confirmó el diagnóstico de PLC. El paciente recibió tratamiento con emulsión hidratante, clobetasol tópico y claritromicina con resolución completa de las lesiones. La pitiriasis liquenoide crónica es un trastorno inflamatorio poco frecuente, de etiología desconocida que ocurre más comúnmente en adultos jóvenes y niños. Si bien la clínica es sugerente, se requiere biopsia cutánea para su confirmación diagnóstica. Es un trastorno benigno, a menudo asintomático y autolimitado, por lo que se debe valorar su tratamiento paciente a paciente. Principalmente se utilizan corticoides tópicos y antibióticos orales (tetraciclinas y eritromicina). Se ha vinculado en escasas publicaciones con el desarrollo de linfoma cutáneo y como síndromes paraneoplásicos, por lo que se sugiere realizar seguimiento.
Chronic lichenoid pityriasis (PLC) is a benign disorder, characterized by a gradual course with the appearance of multiple squamous papules with adherent scales predominating in the trunk and proximal extremities. With the objective to describe its clinical presentation, diagnosis, differential diagnosis, treatment and review literature, we present an 88-yearold male with multiple lesions in the lower extremities of one year of evolution, with papules and adherent scales. A skin biopsy was performed that confirmed the diagnosis of PLC. The patient received treatment with moisturizing emulsion, clobetasol topical and clarithromycin with complete resolution of the lesions. Chronic lichenoid pityriasis is a rare inflammatory disease of unknown etiology that occurs most commonly in young adults and children. Although the clinic is suggestive, a skin biopsy is required for diagnostic confirmation. It is a benign disorder, often asymptomatic and self-limiting, so its patientto- patient treatment should be assessed. Topical corticosteroids and oral antibiotics (tetracyclines and erythromycin) are used. It has been linked in few publications with the development of cutaneous lymphoma and as paraneoplastic syndromes, so it is suggested to follow up.
Subject(s)
Humans , Male , Aged, 80 and over , Pityriasis Lichenoides/diagnosis , Pityriasis Lichenoides/pathology , Biopsy , Clobetasol/administration & dosage , Chronic Disease , Treatment Outcome , Pityriasis Lichenoides/drug therapy , Clarithromycin/administration & dosage , Anti-Inflammatory Agents/administration & dosage , Anti-Bacterial Agents/administration & dosageABSTRACT
Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA), or febrile ulcerative Mucha-Haberman disease (FUMHD) is very rare, but potentially lethal variants of PLEVA. This subtype is characterized by rapidly progressive ulceronecrotic lesions and systemic manifestations, such as high fever, gastrointestinal, neurological, cardiologic and pulmonary involvement, and rheumatologic manifestations. Several treatments with variable response have been challenged, such as systemic steroid, antibiotics, methotrexate, dapsone, cyclosporine and ultraviolet therapy. But there is no standard therapy for FUMHD to date. We report a 59-year-old woman with FUMHD, who recurred after discontinuation of systemic steroid, but successfully treated with oral cyclosporine.
Subject(s)
Female , Humans , Middle Aged , Anti-Bacterial Agents , Cyclosporine , Dapsone , Fever , Methotrexate , Pityriasis , Pityriasis Lichenoides , Ulcer , Ultraviolet TherapyABSTRACT
Febrile ulceronecrotic Mucha-Habermann's disease (FUMHD) is an unusual severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by abrupt onset of widespread ulceronecrotic cutaneous eruption associated with high fever and systemic symptoms. In most cases, a mild typical form of PLEVA was usually present prior to the acute fulminating course. We report a case of FUMHD in a 6-month-old infant, who presented widespread hemorrhagic papules and coalescent thickly crusted ulcerations, high fever, liver dysfunction, and diarrhea with histopathologic findings suggestive of PLEVA.
Subject(s)
Humans , Infant , Diarrhea , Fever , Liver Diseases , Pityriasis Lichenoides , UlcerABSTRACT
Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA) is a very rare cutaneous disorder clinically characterized by the acute onset of erythematous papules, vesicles and large coalescent ulceronecrotic lesions on the whole body accompanied by high fever and severe constitutional symptoms. In general, ulceronecrotic lesion is preceded by a typical rash of PLEVA. Histopathologically, it shows widespread epidermal necrosis and fibrinoid necrosis of blood vessel walls in addition to the superficial and deep perivascular lymphocytic infiltrations. Several regimens including systemic corticosteroid, antibiotics, methotrexate or ultraviolet therapy have been challenged in this disorder . We report a typical case of febrile ulceronecrotic PLEVA in a 10-year-old child who showed a dramatic response to methotrexate.
Subject(s)
Child , Humans , Anti-Bacterial Agents , Blood Vessels , Exanthema , Fever , Methotrexate , Necrosis , Pityriasis Lichenoides , Pityriasis , Ultraviolet TherapyABSTRACT
Febrile ulceronecrotic PLEVA is an unusually severe from of PLEVA, characterized by the sudden onset of diffuse ulceronecrotic eruption aasociated with high fever. A mild eruption precede the acute fulminating course, We observed a 13-year-old boy presenting the form of febrile ulceronecrotic PLEVA. The histologic features were those of PLEVA. He received the systemic corticosteroids with aupportive care. To recognize this disorder is important because of a possibly fatal outcome, So close attention and vigorous therapy are necessary.