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.

Febrile Ulceronecotic Pityriasis Lichenoides et Varioliformis Acuta Successfully Treated with Oral Cyclosporin / 대한피부과학회지

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.

Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta in a child / 대한피부과학회지

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.

A Case of Pityriasis Lichenoides et Varioliformis Acuta in children: Treatment with roxithromycin / 대한피부과학회지

Pityriasis lichenoides et varioliformis acuta is a cutaneous disease of unknown origin characterized by an acute polymorphous eruption which heals with superficial scarring and pigmentation. It occurs mainly in young adults and less commonly in children. Although various types of therapy have been proposed for pityriasis lichenoides, including systemic steroids, tetracycline, methotrexate, sulfones, an UV radiation, there is no definitive treatment and there is few studies of appropriate, efficacious therapy in children. We report a case of pityriasis lichenoides et varioliformis acuta in children treated with roxithromycin.

A Case of Pityriasis Lichenoides et Varioliformis Acuta in children: Treatment with roxithromycin / 대한피부과학회지

Pityriasis lichenoides et varioliformis acuta is a cutaneous disease of unknown origin characterized by an acute polymorphous eruption which heals with superficial scarring and pigmentation. It occurs mainly in young adults and less commonly in children. Although various types of therapy have been proposed for pityriasis lichenoides, including systemic steroids, tetracycline, methotrexate, sulfones, an UV radiation, there is no definitive treatment and there is few studies of appropriate, efficacious therapy in children. We report a case of pityriasis lichenoides et varioliformis acuta in children treated with roxithromycin.

A clinical and histopathological study of pityriasis lichenoides et varioliformis acuta(PLEVA) / 대한피부과학회지

We reviewed the clinical features and the skin biopsy slides of 27 patients who were diagnosed as PLEVA clinically or histapathologically in order to evaluate the clinical characteristics and the specific histopathological findings of PLEVA. The results were as follows : l. 13 of these 27 cases(48%) were confirmed as PLEVA. 2. PLEVA was more frequent in males(85% ), most of whom were young adults. 3. In most cases of PLEVA, the predilection site was on the trunk and extremities, and mild pruritus was a characteristic complaint. 4. Most cases of PLEVA were treated with tetracycline or PUVA therapy with temporary remission, but this therapy did not decrease the rate of recurrence. 5. Some histopathological findings previously described as typical in PLEVA(eg, trapped RBC in the epidermis, exocytosis of lymphocyte, dermal hernorrhage and lymphocytic perivasculitis) seemed not to be specific and were often seen in a variety of other dermatoses. 6. The histopathological findings of linear parakeratosis(85%) and dyskeratotic cells in the middle and upper epidermis(92% ) seemed to be significant findings for PLEUA. 7. The above mentioned histopat,hological findings for PLEVA were frequently found from the vesicular lesions or necrotic hemorrhagic papules.

A Case of Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta / 대한피부과학회지

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.

A Case of Pityriasis Lichenoides et Varioliformis Acuta / 대한피부과학회지

We report herein a case of pityriasis lichenoides et variloiformis acuta in a 48- year-old man, who has wide spread erythematous macule, papule, vesicle, hemorrhagic vesicle and reddish-brown crust on the neck, trunk, axillae and both extremities. He was treated successfully with tetracycline 2. 0 gm a day for 4 weeks.