Febrile ulceronecrotic Mucha-Habermann's disease with fatal outcome

Pietro Puddu, Giuseppe Cianchini, Laura Colonna, Carlo R. Girardelli, Giulio Ferranti, Ornella De Pita

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A 43-year-old woman was examined in October 1993 for a widespread, large, ulceronecrotic skin eruption with exception of the mucosa (Fig. 1). The skin lesions were round and regular, varied in size from 0.5 to 2 cm, and were partially covered by a brownish-black crust. The patient had a fever ranging from 38 to 40 °C, always preceded by shivering. She had presented with small exfoliating erythematous patches and itching, 8 months before being admired to our institute, and was treated with topical corticosteroids. After five and a half months, together with the beginning of fever, the above lesions became wet and were covered with squamous crusts. For 2 years the patient had been affected by perianal fistula, but had not referred to this pathology before. She also reported that she had been treated with antibiotic and corticosteroid intravenous therapies. A biopsy specimen from a lesion of the right leg showed a widely ulcerous fragment of the skin, characterized by a strong infiltration of lymphocytes, especially in the upper part of the dermis, with neutrophil granulocytes. The epidermis surrounding the ulcer showed acanthosis, orthokeratosic hyperkeratosis, and spreading lymphocyte exocytosis, with a large number of necrotic keratinocytes end the presence of melanophages (Figs 2 and 3). All the above data suggested a diagnosis of pityriasis lichenoides et varioliformis acute (PLEVA). Direct immunofluorescence carried out on perilesional skin showed the presence of fluorescent bodies on the superficial dermis using antisera anti- immunoglobulin (anti-IgM) and C1q. Laboratory tests showed the following values: erythrocyte sedimentation rate (ESR), 55 L/h; C-reactive protein, 85 mg/L; white blood cell (WBC), 9200 g/dL; platelets, 476,000; gamma glutamil transferase, 93 U/L; alkaline phosphatase, 185 IU/L; complement C3 fraction, 70.9 mg/L; CD4/CD8 lymphocyte ratio, 6.5; total IgE, 2493 ku/L. Human immunodeficiency virus (HIV), Epstein-Barr virus (EVB), cytomegalovirus, herpes simplex 1 and 2, toxoplasma, rickettsia, and hepatitis markers A, B, and C gave negative results, while blood cultures were positive for Staphylococcus aureus and Pseudomonas aeruginosa. Circulating immune complexes (as C1 binding system), 80 μm/eq/L (normal value, less than 20). Chest X-ray and total body computed axial tomography (CAT) scan were negative. Antimicrobial therapy with teicoplanin and trimethoprim + sulfamethoxazole for 15 days resulted in a reduction in microbial count in blood cultures, and therapy with prednisone (60 mg/day) for a further 20 days was started. The patient was discharged from hospital in reasonable physical condition, without fever and with an improvement of the skin lesions. Surgery for the treatment of her perianal fistula was not performed as her general condition had improved. The patient continued therapy at home with 50 mg prednisone and a broad spectrum antimicrobial for a further 20 days. One month later, the patient was admitted to hospital in a poor physical condition with fever (peaks of 40/41 °C), preceded by shivering, relapsing skin ulcers, weak respiration, and polypneia. The laboratory tests showed a serious hypoproteinemia, C-reactive protein concentration of 321 mg/L, and high transaminase levels. Blood analysis was positive for Staphylococcus aureus. Therapy was started with 60 mg prednisone, antimicrobials, and albumin, but 24 h later the patient fell into a coma and died from cardiogenic shock. Upon her family's request, no autopsy was performed.

Original languageEnglish
Pages (from-to)691-694
Number of pages4
JournalInternational Journal of Dermatology
Issue number9
Publication statusPublished - Sep 1997

ASJC Scopus subject areas

  • Dermatology


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