food-induced acute generalized exanthematous pustulosis in a pregnant woman

C a s e S t u d i e s

199July • August 2006

From the Department of Dermatology and Venereology, University Hospital, Pleven, Bulgaria

Address for correspondence: Sonya Valkova, MD, PhD, 130 Doiran Str, 5800 Pleven, Bulgaria E-mail:[email protected]

A 21-year-old woman of Romany origin, in the third trimester of her fourth pregnancy, was admitted to the hospital because of a generalized erythematous and pustular eruption and desquamation involving her face, neck, trunk, and extremities. The skin changes were accompanied by fever (100.4°F [38°C]) and malaise. The patient was convinced that the dermatitis was induced by the consumption of “spoilt” pork sausage (bad smell, changed taste) approximately 24 hours earlier. Clinical examination revealed a woman with phototype III skin, black eyes, and black hair, in good general health. Widespread, symmetrical, moderately intense erythema and isolated or coalescing targetoid lesions studded with discrete, pinhead-sized, nonfollicular pustules in the center or at the periphery were distributed over her face, trunk, groins, and upper and lower extremities (Figures 1). On the neck and abdomen, lamellar desquamation was observed (Figure 2). Palms, soles, scalp, mucous membranes, hair, and nails were not affected. Nikolsky’s sign was negative. The patient complained of very slight skin burning and itching. The pregnancy was proceeding without any complications and her obstetric status was normal. The woman had neither any accompanying diseases, nor previous personal or family history of psoriasis, nor any known allergies. She had taken no systemic medication (not even vitamins). She had three pregnancies; two ended with the delivery of healthy babies and one of them was aborted at her will. Laboratory studies revealed leukocytosis (13.2 × 109/L), neutrophilia (8 × 109/L), anemia (hemoglobin, 108 g/L), and an elevated erythrocyte sedimentation rate (68–110 mm/h). The results from the following investigations were normal: urinalysis, renal and hepatic function, serum albu-min, Ca, Na, K, aspartate aminotransferase titer, cryoprotein, hepatitis B surface antigen, and serum markers for syphilis. Bacterial and fungal cultures of pustular content were sterile. A skin biopsy specimen of lesional skin revealed subcorneal pustules containing leukocytes and necrotic keratinocytes and a mixed perivascular inflammatory infiltrate with isolated eosinophils in the dermis (Figure 3). The patient was treated with systemic methylprednisolone in gradually reduced doses, fluocinonide cream 0.05%, and emollients. As a result, her fever disappeared and her erythema faded. Frequent obstetric examination and cardiotocography were normal and showed no evidence of placental insufficiency. At 40 weeks’ gesta-tion, the patient spontaneously gave birth without any complications to a healthy boy. She was discharged with complete resolution of the skin lesions, preceded by massive desquamation of the epidermis. The 1-year follow-up of the patient revealed no relapses or new pustular eruptions.

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Food-Induced Acute Generalized Exanthematous Pustulosis in a Pregnant WomanSonya Valkova, MD, PhD

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200 July • August 2006

Our first impression was that this patient was presenting with impe-tigo herpetiformis (generalized pus-

tular psoriasis of pregnancy), a very rare,

life-threatening condition. It was ruled out because there was no personal or family his-tory of psoriasis, no other symptoms or signs characteristic of the disease, and no typical histologic data for psoriasis (parakeratosis, papillomatosis, and acanthosis). Our further differential diagnosis included other derma-toses of pregnancy and other pustular and/or bullous eruptions, e.g., impetigo contagiosa, pustular folliculitis of pregnancy, pustular eosinophilic folliculitis, erythema exudati-vum multiforme. The combination of clini-cal, laboratory, and histopathologic findings made them unlikely in the present case.

The final diagnosis of acute generalized exan-thematous pustulosis (AGEP) was based on the presence of most of the clinical and histologic criteria defined by investigators in 19911 and later delineated in a report in 2001.2 Although to the best of our knowledge neither of these reports included AGEP in a pregnant woman, there has been a report of AGEP in a pregnant woman induced by paracetamol (acetaminophen).3

Known etiologies of AGEP include systemic medication, recent viral infection, and expo-sure to mercury or UV radiation. In recent years, the list of AGEP inducers has been con-stantly increasing.4 Ingestion of an allergen by a previously sensitized person may result in a number of cutaneous reactions, e.g., urti-carial eruptions, erythema multiforme-like lesions, or vasculitis.5 There are a few reports of generalized pustular eruptions following the ingestion of an allergen.6,7 As our patient had no history of drug intake, recent infec-tion, or contact with mercury, we assumed that the disease developed as a consequence of the consumption of the pork sausage, because the dermatitis occurred shortly after its intake and resolved soon after treatment.

Recent studies suggest that AGEP is a well orchestrated cell-mediated immunologic reac-tion with both afferent and efferent pathways.8 T-lymphocytes play a central role in the activa-tion and recruitment of other cells in the skin, secreting diverse activating cytokines, such as granulocyte–macrophage colony-stimulating factor, regulated on activation normal T-cell expressed and secreted (“RANTES”), CXC che-mokine ligand-8, interferon γ, and interleukin-17. In the efferent arm, effector cells, mainly

Figure 2. Erythema and des-quamation on the trunk

Figure 1. Targetoid lesions with pustules and desquama-tion on the lower extremities


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201July • August 2006

neutrophils and sometimes eosinophils, are recruited to the skin by the aforementioned chemokines, attach by a number of adhe-sion molecules, and accumulate in the epi-dermis to form pustules. AGEP, a neutrophil cell-mediated immune reaction, fits into the immunologic background of pregnancy and is actually expected to develop in response to certain stimuli.3

This case is of special interest because the dis-ease developed in a pregnant woman and was food-induced. Recognition that AGEP may resemble impetigo herpetiformis is important so that AGEP, which resolves if the offending agent is discontinued, is not overtreated.

Acknowledgment: I thank Assistant Professor Evlogy Marinov, Department of Pathology, Medical University of Pleven, for his assistance in histopathology and for the photomicrographs.

REFERENCES 1 Roujeau JC, Bioulac-Sage P, Bourseau C, et al. Acute

generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol. 1991;127:1333–1338.

2 Sidoroff A, Halevy S, Bavinck JN. Acute generalized exanthematous pustulosis (AGEP)—a clinical reac-tion pattern. J Cutan Pathol. 2001;28:113–119.

3 Wohl Y, Goldberg I, Sharazi I, et al. A case of paracetamol-induced acute generalized exan-thematous pustulosis in a pregnant woman local-ized in the neck region. Skinmed. 2004;3:47–48.

4 Saissi EH, Beau-Salinas F, Jonville-Bera AP, et al. Drugs associated with acute generalized exan-thematic pustulosis [in French]. Ann Dermatol Venereol. 2003;130:612–618.

5 Rietschel RL, Fowler JF Jr. Fisher’s Contact Dermatitis. 4th ed. Baltimore, MD: Williams & Wilkins; 1995:114–129.

6 Park YM, Park JG, Kang H, et al. Acute gen-eralized exanthematous pustulosis induced by ingestion of lacquer chicken. Br J Dermatol. 2000;143:230–232.

7 Rustin M, Robinson T, Dowd P. Toxic pustulo-derma: a self-limiting eruption. Br J Dermatol. 1990;123:119–124.

8 Britschgi M, Pichler WJ. Acute generalized exan-thematous pustulosis, a clue to neutrophil-mediat-ed inflammatory processes orchestrated by T cells. Curr Opin Allergy Clin Immunol. 2002;2:325–331.

Figure 3. Subcorneal pus-tule containing leukocytes and necrotic keratinocytes and polymorphic infiltrate in the dermis (hematoxylin-eosin stain, original magnifi-cation ×100)


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food-induced acute generalized exanthematous pustulosis in a pregnant woman

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