{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T14:48:34Z","timestamp":1775486914888,"version":"3.50.1"},"reference-count":6,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,3,31]],"date-time":"2004-03-31T00:00:00Z","timestamp":1080691200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Dermatology"],"published-print":{"date-parts":[[2005,3]]},"abstract":"A 15\u2010year\u2010old, unmarried female presented to our dermatology department for an intensely pruritic skin rash that had appeared abruptly 3\u00a0days earlier. She had a remarkable medical history for a case of allergic rhinitis and several attacks of asthma in her early childhood. The condition waxed and waned initially but had improved in recent years. Physical examination revealed several erythematous plaques, papules studded with scattered pustules having diameters less than 0.3\u00a0mm. Conspicuous scratch marks had caused erythematous wheal\u2010like indurations also studded with pustules in a linear distribution across the waist, forearms (Fig.\u00a01<\/jats:ext-link>), and back (Fig.\u00a02<\/jats:ext-link>). Discrete papulopustules were present on the face, nape and neck. The patient was otherwise healthy. There were no other symptoms such as fever, malaise, weakness, or lymphadenopathy<\/jats:p>Papulopustulosis with adjacent linear Koebner form distribution over the flexor aspect of the forearm<\/jats:p><\/jats:caption>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p>The pathergy phenomenon observed on the right upper back<\/jats:p><\/jats:caption>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p>Laboratory results were normal for hepatic and renal functions, serum electrolytes, glucose, protein, erythrocyte sedimentation rate (8\u00a0mm\/h), and C\u2010reactive protein (0.355\u00a0mg\/l). A human immunodeficiency virus (HIV) antibody screen test was negative. Serum was positive for herpes simplex virus (HSV)\u20101 and HSV\u20102 IgG (in low titers), but negative for HSV\u20101 and HSV\u20102 IgM. White blood cell count revealed leukocytosis (11.2\u00a0\u00d7\u00a0103<\/jats:sup>\/l), with a differential count of 68% neutrophils, 27% lymphocytes, and 8% eosinophils. Serum IgA, IgG, and IgM were within normal limits, but the IgE level was elevated (677\u00a0mg\/dl). Cultures from peripheral blood and pustules were negative. A Tzank smear performed on the pustules showed no multinucleated giant cells. Fungal testing of skin scrapings from the initial lesion site gave negative results. Routine stool tests, including common pathogen and parasite screens, were negative, and urinalysis results were unremarkable.<\/jats:p>A biopsy specimen obtained from a skin pustule showed subcorneal eosinophilic and neutrophilic pustules in the follicular infundibulum with marked spongiosis of the follicular epithelium. (Fig.\u00a03<\/jats:ext-link>). There was a moderately dense superficial and deep perivascular mixed inflammatory cell infiltrate comprising eosinophils, neutrophils and lymphocytes. Migration of eosinophils and neutrophils through the vessel wall with variable luminal intramural fibrin deposition, histologically indicative of vasculopathy, was seen. There was concomitant slight perivascular dermal necrosis. (Fig.\u00a04<\/jats:ext-link>)<\/jats:p>There is a striking inflammatory reaction involving the follicle characterized by migration of eosinophils, lymphocytes and neutrophils into the outer root sheath epithelium of the follicle (there are perifollicular vasculopathic changes, including those occurring in the context of red cell extravasation)<\/jats:p><\/jats:caption>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p>The perifollicular microvasculature demonstrates endothelial cell hyperplasia with striking proplastic alterations of the endothelium leading to luminal attenuation (\u22c6). The perivascular connective tissue exhibits incipient necrobiotic change, fulfilling morphologic criteria warranting the designation of pathergy (\u2192)<\/jats:p><\/jats:caption>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p>Based on the clinical presentation and light microscopic findings on biopsy, a diagnosis of eosinophilic pustular folliculitis with pathergy was made. Systemic prednisolone 30\u00a0mg in divided doses was given. After 1\u00a0week of systemic corticosteroid therapy, the patient's condition was significantly improved and the patient was subsequently discharged. Two months later she had a relapse, upon which corticosteroid therapy was commenced leading to lesional resolution. The foci of eosinophilic folliculitis healed with areas of hyperpigmentation with variable scarring.<\/jats:p>","DOI":"10.1111\/j.1365-4632.2004.02097.x","type":"journal-article","created":{"date-parts":[[2005,3,8]],"date-time":"2005-03-08T14:54:59Z","timestamp":1110293699000},"page":"203-205","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Pathergy in atypical eosinophilic pustular folliculitis"],"prefix":"10.1111","volume":"44","author":[{"given":"Pai\u2010Jui","family":"Hsu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chien\u2010Jen","family":"Huang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meng\u2010Tse","family":"Wu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,3,31]]},"reference":[{"key":"e_1_2_2_2_2","first-page":"195","article-title":"Eosinophilic pustular folliculitis","volume":"50","author":"Ofuji S","year":"1970","journal-title":"Acta Derm Venereol"},{"key":"e_1_2_2_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(91)70144-Q"},{"key":"e_1_2_2_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-4362.1994.tb04943.x"},{"key":"e_1_2_2_5_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-4362.2000.00973.x"},{"key":"e_1_2_2_6_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1999.02701.x"},{"key":"e_1_2_2_7_2","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2002.118338"}],"container-title":["International Journal of Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-4632.2004.02097.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1365-4632.2004.02097.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,14]],"date-time":"2023-10-14T22:39:41Z","timestamp":1697323181000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1365-4632.2004.02097.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,3,31]]},"references-count":6,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2005,3]]}},"alternative-id":["10.1111\/j.1365-4632.2004.02097.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1365-4632.2004.02097.x","archive":["Portico"],"relation":{},"ISSN":["0011-9059","1365-4632"],"issn-type":[{"value":"0011-9059","type":"print"},{"value":"1365-4632","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,3,31]]},"assertion":[{"value":"2004-03-31","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}