Dermatology Online Journal

Fatal outcome due to bacterial superinfection of eczema herpeticum in a patient with mycosis fungoides
Susana Mallo-García¹, Pablo Coto-Segura¹, Héctor Suárez-Casado², Luis Caminal², José Sánchez-del-Río³, Jorge Santos-Juanes¹
Dermatology Online Journal 14 (6): 21

1. Department of Dermatology II. Central University Hospital of Asturias, Oviedo, Spain. susana2505@yahoo.com
2. Department of Internal Medicine II. Central University Hospital of Asturias, Oviedo, Spain
3. Dermatology. Cabueñes Hospital. Gijón. Central University Hospital of Asturias, Oviedo, Spain

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Abstract

Kaposi varicelliform eruption or eczema herpeticum is well known to be associated with several chronic dermatoses, including atopic dermatitis, foliaceus pemphigus, seborrheic dermatitis, Darier disease, and congenital ichthyosiform erythroderma. Although less frequently, it has also been described in cases of mycosis fungoides and Sèzary syndrome. We would like to report an extremely rare case of a woman with a T-cell cutaneous lymphoma who developed disseminated cutaneous herpes simplex with S. aureus sepsis and a fatal outcome.

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A 73-year-old woman with syringomyelia, carotid stenosis, thrombocytopenia, and a right ovarian mucinous adenocarcinoma as pathological antecedents, was diagnosed with mycosis fungoides in October 2005. She had been treated with several different modalities, including narrow band UVB radiation, methothrexate, and prednisone with chlorambucil; her disease was poorly controlled.

Figure 1

Neither evidence of systemic involvement nor presence of Sèzary circulating cells was found. The patient required several hospital admissions in the last few months due to erythrodermia. She was then classified as T4N0M0, stage IIIA and was finally receiving prednisone and chlorambucil as her main treatment.

In January 2007 she was admitted to the hospital with general ill health, chills, temperature of up to 38.5ºC, and painful skin lesions. The cutaneous eruption began around the mouth and later extended to head and neck, trunk, and extremities; nearly total cutaneous surface involvement developed.

Upon physical examination (Fig. 1), the patient appeared acutely ill, and almost the entire skin surface was erythematous and covered with tiny vesicles. Coalescent vesicles over extensive areas were noted and yellowish crusts were apparent mainly over the face. Erosions were prominent on pressure or friction regions.

On admission, the temperature was 38.5ºC, the pulse rate was 110 beats per minute and the blood pressure was 85/65 mm Hg.

The blood tests disclosed the following values: Hemoglobin 10.1 g/dL, hematocrit 30.1 percent, 2200 leukocytes/mm³ (86% neutrophils, 12% lymphocytes), 165000 platelets/mm³, glycemia 132 mg/dL, BUN 65 mg/dL, serum creatinine 0.90 mg/dL, sodium 127 mmol/L, potassium 4.9 mmol/L, calcium 2.78 mmol/L, lactic dehydrogenase 628 U/L, AST 46 U/L and ALT 32 U/L.

Blood cultures grew S. Aureus. Viral culture from a vesicle grew out Herpes Simplex Virus I. The antibody tests for herpes virus type I (IgG and IgM) were positive. Also the skin biopsy showed cytopathic signs compatible with herpes virus type I.

Clinical diagnosis of disseminated herpes simplex infection was made. The patient was placed in isolation and treated with intravenous acyclovir (10 mg/kg/8 hours) and intravenous piperacillin tazobactam (4 gr/8 hours). In addition, IV fluids and analgesia with morphine were required. Despite treatment, there was progressive worsening and the patient died on the third day of hospitalization. Postmortem examination was not made.

This patient represents a very unusual manifestation of disseminated cutaneous herpes simple infection with secondary bacterial infection and sepsis in a patient with mycosis fungoides.

Kaposi varicelliform eruption or eczema herpeticum is associated with several chronic dermatoses, including atopic dermatitis, foliaceus pemphigus, seborrheic dermatitis, Darier disease, and congenital ichthyosiform erythroderma [1]. Although less frequently, it has also been described in cases of mycosis fungoides and Sèzary syndrome [2, 3]. Infections are a serious complication of cutaneous T-cell lymphoma [4], and are the cause of death in 27 to 60 percent of patients. The major bacterial pathogen has been found to be S. aureus, followed by Beta Hemolytic Streptococcus [3, 5, 6, 7]. Axelrod et al. determined the incidence of infections in patients with mycosis fungoides and Sèzary syndrome and found cutaneous bacterial infection the most common, followed by cutaneous herpes simples virus and herpes zoster. Local infections are most commonly seen; only 14 percent show cutaneous dissemination [4].

A disseminated viral infection, mainly due to herpes virus, as in the current case, may significantly worsen a patient's prognosis [4]. The combination of a Kaposi varicelliform eruption and a cutaneous T-cell lymphoma increases the susceptibility to secondary bacterial infection that might lead to sepsis and death. In such patients, it is extremely important to start early and aggressive antibacterial therapy to avoid the complications described above.

Cutaneous T-cell lymphoma should be included in the list of skin diseases that predispose to disseminated viral infections, particularly Kaposi varicelliform eruption.

References

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2. Scully RE, Galdabini JJ, McNeely BU: Case 37-1975, case records of Massachussetts General Hospital: Weekly clinicopathologic exercises. N Engl J Med 1975;293:598-603. PubMed

3. Segal RT, Watson W: Kaposi's varicelliform eruption in mycosis fungoides. Arch Dermatol 1978;114:1067-9. PubMed

4. Axelrod PI, Lorber B, Vonderheid EC. Infections complicating mycosis fungoides and Sèzary syndrome. JAMA 1992;267(10):1354-8. PubMed

5. Brion N, Guillaume JC, Dubertret L, Touraine R. Disseminated cutaneous herpes of the adult and Sèzary syndrome. Ann Dermatol Venereol 1981;108(6-7):517-21. PubMed

6. Goldgeier MH, Cohen SR, Braverman IM, Stenn KS. An unusual and fatal case of disseminated cutaneous herpes simplex. J Am Acad Dermatol 1981;4(2):176-80. PubMed

7. Taulbee KS, Johnson SC. Disseminated cutaneous herpes simplex infection in cutaneous T-Cell lymphoma. Arch Dermatol 1981;117:114-5. PubMed

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