Dermatitis herpetiformis

Abstract

Impetigo herpetiformis (IH) is a rare dermatosis which usually occurs during the third trimester of pregnancy. It is characterized by acute erythematosquamous plaques covered with tiny superficial pustules in a herpetiform distribution with less likely mucus membranes involvement (1). It can be associated with constitutional symptom s such as fever, chills, nausea, vomiting and diarrhea (2-4). Impetigo herpetiformis can cause serious complications to the mother and fetus which include: maternal hypocalcemia leading to delirium, convulsions, and tetany in the mother (2,5), and placental insufficiency leading to still birth, neonatal death or fetal abnormalities (1,4,6,7). Lesions are expected to disappear after birth but may recur during subsequent pregnancies at an earlier gestational age (8,9). Presented here is a case of IH occurring during the 37th week of gestation in a primigravida who failed to respond to oral steroid but successfully cleared with oral etretinate.

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Case Report

A 23-year-old female presented to our dermatology clinic seven days after her delivery of a healthy baby with a generalized pustular skin eruption. The condition started during the third trimester, when she developed an itchy erythematous papules and plaques on her abdomen. At that time she was diagnosed as a case of urticarial plaques and papules of pregnancy and was given a strong topical steroid, multivitamins and antihistamine. She improved temporarily. But at the 37th week of gestation, the patient noticed the appearance of widespread confluent erythemaous plaques studded with superficial pustules on her trunk, sparing the umbilicus (Figure 1). The eruption continued to increase in severity for two weeks, during which time she delivered a healthy baby. She had no history of psoriasis or any skin disease and there was no family history of psoriasis.

On examination, the patient was an ill-looking female with stable vital signs. The skin of her trunk had erythematous plaques covered with pin-head sized pustules. Some areas demonstrated central clearing, while others were impetiginzed. Palms, soles, and mucus membranes were free of any lesions. A general systemic examination was normal. The patient's hematological and biochemical studies were normal except for marked leukocytosis, which was mainly neutrophilic. Calcium level was normal and culture of the pustules was sterile. Skin biopsy showed spongiform pustules filled with neutrophils located just beneath the corneal layer but no acanthosis or parakeratosis (Figure 2). The papillary dermis was edematous with perivascular mononuclear cell infiltrates.

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

The final diagnosis was IH. Although the patient had already been on a daily dose of 70 mg prednisone for two weeks prior to her presentation, her eruption was worsening, with new lesions appearing. We started her on oral etretinae 35 mg daily after taking her consent for strict contraception precautions for a period of three years while we tapered off prednisone gradually over a ten day period. Within the first week after the start of etretinate the eruption dried and no new lesions appeared. Complete clearance was achieved within three weeks later. The patient was kept on 35 mg etretinate daily for 16 weeks and followed up in the clinic for a period of 24 weeks with no recurrence.

Discussion

Impetigo herpetiformis is a pustular skin disorder considered by some authors as a variant of generalized pustular psoriasis, while others consider it a different entity because of the differences in the presentation; the frequent absence of a history of psoriasis and the fact that the eruption resolves by the end of pregnancy period (4,10,11). The underlying etiological factors are still speculative but the role of high progesterone levels during the last trimester of pregnancy and the decreased calcium levels were suggested in the pathogenesis of IH (12). On the contrary, a case study of a patient with IH, in which skin-derived antileukoproteinase activity was measured, suggested a reduced amount of that enzyme contributed to the formation of epidermal pustules (13). This mechanism could apply to both generalized pustular psoriasis and IH, suggesting a final common pathway in the pathogenic mechanisms (14). Expected biochemical changes in IH include decreased serum calcium, albumin and vitamin D (2,4,9,15).

The condition normally resolves after pregnancy and may recur in subsequent pregnancies (8,9,15). However, treatment with systemic steroid at a dose of 60-80 mg daily is indicated to prevent complications to the mother or the fetus during pregnancy after which other treatments could be used such as Clofazimine (12), oral PUVA, topical steroids, methotrexate (16), sulfapyridine and sulfones (17), isotretinoin (18), and etretinate (which is now replaced by its counterpart acitretin, due to its shorter halflife). The responsiveness to oral retinoids in IH could be taken as evidence that it may represent a variant of generalized pustular psoriasis whose standard treatment consists of oral retinoids and PUVA. Besides, the worsening of the patient's condition while on oral steroids and the quick control of the eruption when she was started on etretinate signifies that the condition could not have possibly resolved spontaneously and an alternative treatment was needed to control the eruption. Since the patient had no recurrence while on progesterone-containing contraceptive pills, the suggestion of progesterone's role in the etiology is unlikely. Further case studies and reports could clarify more on the etiology of this rare disease.

References

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13. Kuijpers Al, et al. Exteremely low levels of epidermal skinderived antileucoproteinase in a patient with impetigo herpetiformis. Br J Dermatol 1997; 137:123-129.

14. Katsambas A, et al. Impetigo herpetiformis during the puerperium. Dermatol 1999; 198:400-402.

15. Oll F, et al. Impetigo herpetiformis with lowered serum level of vitamin D and its diminished intestinal absorption. Dermatologica 1982; 164:360-365.

16. Aka N, Kuscu NK, Yazicioglu E. Impetigo herpetiformis at the 36th week of gestation. Int J Gyn Obs 2000; 69(2):153-154.

17. Stone OJ. Sulfapyridine and sulfones decrease glycosamineglycans viscosity in dermatitis herpetiformis, ulcerative colitis and pyoderma gangrenosum. Med Hypotheses 1990; 31:99-103.

18. Breier-Maly J, et al. Generalized Pustular Psoriasis of Pregnency (Impetigo Herpetiformis). Dermatol 1999; 198:61-64.

IQBAL A BUKHARI MD

ASISSTANT PROFESSOR, DEPARTMENT OF DERMATOLOGY, KING FAISAL UNIVERSITY, COLLEGE OF MEDICINE AND MEDICAL SCIENCES

ALKHOBAR, SAUDI ARABIA

ADDRESS FOR CORRESPONDENCE:

Dr. Iqbal A. Bukhari

King Fahad Hospital of the University

P.O. Box 40189

Alkhobar 31952, Saudi Arabia

Phone: +1196638957886

Fax: +1196638949209

E-mail: consultant@dermatologyclinics.net

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