Febrile ulceronecrotic Mucha-Habermann disease with exuberant mucosal involvement: case report

Abstracts

A pitiríase liquenoide é dermatose incomum, idiopática, com espectro clínico-histopatológico onde inclui a forma varioliforme aguda (doença de Mucha-Habermann), sua variante febril úlceronecrótica e a forma crônica. Manifestações sistêmicas podem ocorrer na variante febril úlcero-necrótica, com relatos de casos fatais em adultos. Relata-se o caso de um paciente jovem, com diagnóstico clínico e histopatológico de doença de Mucha-Habermann, variante febril úlcero-necrótica, e acometimento mucoso exuberante, fato ocasional, mesmo nas formas mais graves de pitiríase liquenoide. Além do aspecto clínico inusitado, demonstra-se excelente resultado terapêutico, com a associação de prednisona e metotrexato.

Metotrexato; Pitiríase liquenóide; Prednisona

CASE REPORT

Febrile ulceronecrotic Mucha-Habermann disease with exuberant mucosal involvement - Case report^*

Moema Mignac Cumming^I; Adriana S. Salathiel^II; Mário Alberto S. Paino^III; Sérgio Delort^IV; Ana Maria Roselino^V

^I

^IIPh.D. studies under course – FMRP-USP - Ribeirão Preto (SP), Brazil

^IIIPathologist, Laboratório de Anatomia Patológica Dr. Mário A. S. Paino - Araraquara (SP), Brazil

^IVDermatologist, President of 6th Dermatology District of Ribeirão Preto - Ribeirão Preto (SP), Brazil

^VAssociate Professor, Division of Dermatology, Department of Clinical Practice, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo – São Paulo (SP), Brazil

Mailing Address

ABSTRACT

Pityriasis lichenoides is a rare idiopathic cutaneous disorder, with a clinical-histopathological spectrum comprising the acute varioliform form (Mucha-Habermann's disease), its febrile ulceronecrotic variant, and its chronic form. Systemic manifestations may occur in the febrile ulceronecrotic variant, with reports of adult mortality. The case of a young male patient with clinical and histopathological diagnosis of Mucha-Habermann's disease, febrile ulceronecrotic variant, with severe mucosal involvement - an occasional incidence even in the most severe forms of pityriasis lichenoides - is presented. In addition to the atypical clinical aspect, an excellent therapeutic result is shown with the association of prednisone and methotrexate.

Keywords: methotrexate; pityriasis lichenoides; prednisone

INTRODUCTION

Acute varioliform pityriasis lichenoides, also known as Mucha-Habermann disease, is an uncommon idiopathic dermatosis characterized by erythematous desquamative lesions frequently followed by papular-necrotic and hemorrhagic lesions, which typically progress to atrophic and dyschromic scars ¹. They affect both children and adults in their first decades of life. The rare febrile ulceronecrotic variant was initially described by Degos in 1966 and it is characterized by sudden onset of disseminated purpuric papules with necrotic core ulcers and systemic manifestations ^1-3.

CASE REPORT

Twenty-one year-old male patient, born in the state of Sao Paulo, university student, single, reported recurrent episodes of upper airway infections for the previous six months, having taken antibiotics that solved the manifestations. He went to a medical center in November 2007, reporting the presence of an ulcerated lesion on the lower lip for 45 days, followed by erythematous non-pruriginous lesions on the trunk, limbs and oral cavity, followed by febrile peaks (38.55º C), malaise and arthralgia.

The dermatological examination showed erythematous papules on the trunk, limbs, hand palms and feet soles, many of them centered by flap desquamation, without intact vesicles or blisters. There were also ulceronecrotic lesions of varioliform aspect, lenticular to nummular, on the dorsum, gluteus, limbs and genitals (Figure 1).

The patient was treated with antibiotic therapy and prednisone 0.3 mg/kg/Day in addition to topical care in another center. The cutaneous manifestations worsened, which made the physicians increase the prednisone dose to 1 mg/Kg/Day and broadening of antimicrobial spectrum. As there were no clinical responses, the patient was referred to us and the following tests were ordered: general lab tests, resulting in normal range responses; serology for hepatitis B and C, HIV and Epstein-Barr virus which were not reagent, which also applied to other viral infections. Cultures for microbial agents showed negative results and laboratory exams for vasculitis (including ANCA) excluded this hypothesis. Clinical pathology studies evidenced epidermis with exocytosis of lymphocytes with atypia, keratinocyte necrosis and vacuolar interface dermatitis; dermis with lymphocytarian moderate infiltrate, superficial and profound, perivascular and interstitial, with extravasation of red blood cells (Figure 2). Based on the hypothesis of acute varioliform pityriasis lichenoides, ulceronecrotic febrile form, the dose of prednisone was maintained and associated with methotrexate at weekly dose of 15 mg PO, with significant improvement within six weeks. After four months of treatment, the case progressed to resolution of the clinical manifestations, leading to interruption of treatment. He had a new mild episode one week before this report was made and the same treatment was reintroduced. He still has atrophic scars of varioliform aspect (Figure 3).

DISCUSSION

Mucha-Habermann disease, ulceronecrotic febrile form, is a destructive variant of pityriasis lichenoides, with presence of coalescent ulcerations and high temperature ^2-4. The associated symptoms include arthralgia, gastrointestinal tract and central nervous system affection, interstitial pneumonitis and lymphocytic myocarditis, which may lead to death. The distribution of lesions is similar to that of acute varioliform pityriasis lichenoides, with occasional involvement of oral mucosa ^1,3. The etiology remains unknown, but the most widely accepted hypothesis is that it indicates hypersensitivity reactions to infectious agents as underlying mechanisms for the development of the disease. In the literature, many pathogens have been associated with the disease, including HIV, varicella zoster, Epstein-Barr virus, cytomegalovirus, adenovirus, Staphylococcus, Streptococcus, Mycoplasma and Toxoplasma gondii.^1,2,5 In our case, there was previous history of repeated upper respiratory tract infection six months before the first labial necrotic lesion. Bacterial studies and viral serology were negative, and it was not possible to correlate them with the complete syndrome.

Some reports have shown the presence of clonal T lymphocyte populations in the blood and cutaneous lesions of patients with acute varioliform pityriasis lichenoides, suggesting that the disease is a lymphoproliferative disorder of T cells, despite the absence of morphologically atypical lymphocytes in cutaneous lesions ^3,5,6. This is another theory commonly used by many authors to explain the etiology of the disease.

For ulceronecrotic variant, there are many reports of favorable results with the use of systemic glucocorticoids, combining phototherapy and oral methotrexate, intravenous methotrexate, cyclosporine and gammaglobulin ^7-10. Taking into account the potentially serious course of the disease, it is necessary to have strict monitoring of the patient to prevent fatal outcomes.

In this case report, there was exuberant mucosa affection, which is an uncommon finding even in the most severe forms of the disease, combined with favorable response to corticoids with methotrexate.

REFERENCES

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• 8.  Pyrpasopoulou A, Athyros VG, Karagiannis A, Chrysomallis F, Zamboulis C. Intravenous immunoglobulins: a valuable asset in the treatment of a case of septic febrile ulceronecrotic Mucha-Habermann disease. Dermatology. 2007;215:164-5.
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Publication Dates

• Publication in this collection
  25 Feb 2010
• Date of issue
  Dec 2009

History

• Accepted
  06 Nov 2009
• Received
  20 Aug 2008