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Localized Facial Macules and Vesicles—Diagnosis

Localized Facial Macules and Vesicles—Diagnosis Diagnosis: Localized facial dermatitis herpetiformis (DH). Microscopic and laboratory findings and clinical course Direct immunofluorescence of the biopsy specimen from nonlesional skin revealed granular deposition of IgA concentrated at the dermoepidermal junction.The patient's hemoglobin levels had decreased over the past year to as low as 10.3 g/dL (reference range, 12.0-15.5 g/dL), with a hematocrit of 34% (referencerange, 35%-45%) and a ferritin value of less than 2 ng/mL (reference range, 15-290 ng/mL). Serum chemistry studies and liver function tests revealed noabnormalities. Antigliadin IgG and IgA antibody levels were markedly elevated. The patient's initial attempts to minimize the gluten in her diet had no effect on her dermatitis. After the diagnosis of DH was confirmed, dapsonetherapy (100 mg/d) was initiated. Within 36 hours of the first dose, the pruritus and burning sensation completely resolved. The patient continues to do wellwith dapsone therapy and plans to follow a gluten-free diet. Discussion Dermatitis herpetiformis was first described by Duhring1 in 1884. Since then, it has been more completely characterized clinically, histologically,and immunologically. Typically, DH presents with intensely pruritic erythematous papules or vesicles on the buttocks and extensor surfaces of the extremities.Histologically, dermal papillae contain neutrophilic infiltrates and edema, which may eventuate in a subepidermal blister. Direct immunofluorescence revealsgranular IgA deposits at the dermoepidermal junction in 100% of cases. Patients with DH exhibit a high frequency of HLA-A1, -B8, -DR3, and -DQw2 haplotypes.2 Duhring1 described the propensity of DH to affect the neck, trunk, and extremities, but noted that any anatomicalregion could be involved. His series did not describe any patients with lesions localized to the head or face. Trepanier3 reporteda case of bullous scalp lesions and, because of the patient's response to sulfone therapy, postulated that the diagnosis was localized DH. However,histologic analysis revealed an infiltrate with an eosinophilic predominance, and the results of direct immunofluorescence were negative. These observationsand the fact that the patient described was African American suggest that Trepanier's patient did not have DH.2 Komura and Imamura4 described a patient who had previously been diagnosed as having DH involving the trunk and extremitiesand who subsequently developed facial papular dermatitis. Histopathologic analysis revealed edematous dermal papillae infiltrated with neutrophils,lymphocytes, and eosinophils. Direct immunofluorescent studies showed fibrillar deposits of IgA along the dermoepidermal junction, consistent with DH. Helander and Jansen5 also reported a case of DH that was limited to the face. The findings of a biopsy and directimmunofluorescence were consistent with DH. The patient, who did not havesymptoms of a gluten-sensitive enteropathy, responded well to dapsone therapy. Although the confinement of lesions to the face is rare in DH, our patient presented with a history of pruritus and burning, along with evidence of small-bowelvillous atrophy, which suggested that she had DH. This suspicion was confirmed by the results of immunofluorescence testing and was additionally supportedby the dramatic response to dapsone therapy. The rare variant of DH in the present case lends credence to Duhring's assertion regarding the protean clinicalmanifestations of this disease.1 The diagnosis of DH merits consideration under the appropriate clinical circumstances, despitethe absence of classic lesional distribution. References 1. Duhring L Dermatitis herpetiformis JAMA. 1884;3225- 229Google ScholarCrossref 2. Otley CHall III RP Dermatitis herpetiformis Dermatol Clin. 1990;8759- 769PubMedGoogle Scholar 3. Trepanier Y Localized bullous dermatitis herpetiformis Arch Dermatol. 1970;10198- 99PubMedGoogle ScholarCrossref 4. Komura JImamura S Papular dermatitis herpetiformis: report of a case with localized, facial lesions Dermatologica. 1977;155350- 354PubMedGoogle ScholarCrossref 5. Helander IJansen CT Localized dermatitis herpetiformis J Am Acad Dermatol. 1987;161052- 1053PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Localized Facial Macules and Vesicles—Diagnosis

Archives of Dermatology , Volume 140 (3) – Mar 1, 2004

Localized Facial Macules and Vesicles—Diagnosis

Abstract

Diagnosis: Localized facial dermatitis herpetiformis (DH). Microscopic and laboratory findings and clinical course Direct immunofluorescence of the biopsy specimen from nonlesional skin revealed granular deposition of IgA concentrated at the dermoepidermal junction.The patient's hemoglobin levels had decreased over the past year to as low as 10.3 g/dL (reference range, 12.0-15.5 g/dL), with a hematocrit of 34% (referencerange, 35%-45%) and a ferritin value of less than 2 ng/mL (reference...
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Publisher
American Medical Association
Copyright
Copyright © 2004 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.140.3.353-h
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Localized facial dermatitis herpetiformis (DH). Microscopic and laboratory findings and clinical course Direct immunofluorescence of the biopsy specimen from nonlesional skin revealed granular deposition of IgA concentrated at the dermoepidermal junction.The patient's hemoglobin levels had decreased over the past year to as low as 10.3 g/dL (reference range, 12.0-15.5 g/dL), with a hematocrit of 34% (referencerange, 35%-45%) and a ferritin value of less than 2 ng/mL (reference range, 15-290 ng/mL). Serum chemistry studies and liver function tests revealed noabnormalities. Antigliadin IgG and IgA antibody levels were markedly elevated. The patient's initial attempts to minimize the gluten in her diet had no effect on her dermatitis. After the diagnosis of DH was confirmed, dapsonetherapy (100 mg/d) was initiated. Within 36 hours of the first dose, the pruritus and burning sensation completely resolved. The patient continues to do wellwith dapsone therapy and plans to follow a gluten-free diet. Discussion Dermatitis herpetiformis was first described by Duhring1 in 1884. Since then, it has been more completely characterized clinically, histologically,and immunologically. Typically, DH presents with intensely pruritic erythematous papules or vesicles on the buttocks and extensor surfaces of the extremities.Histologically, dermal papillae contain neutrophilic infiltrates and edema, which may eventuate in a subepidermal blister. Direct immunofluorescence revealsgranular IgA deposits at the dermoepidermal junction in 100% of cases. Patients with DH exhibit a high frequency of HLA-A1, -B8, -DR3, and -DQw2 haplotypes.2 Duhring1 described the propensity of DH to affect the neck, trunk, and extremities, but noted that any anatomicalregion could be involved. His series did not describe any patients with lesions localized to the head or face. Trepanier3 reporteda case of bullous scalp lesions and, because of the patient's response to sulfone therapy, postulated that the diagnosis was localized DH. However,histologic analysis revealed an infiltrate with an eosinophilic predominance, and the results of direct immunofluorescence were negative. These observationsand the fact that the patient described was African American suggest that Trepanier's patient did not have DH.2 Komura and Imamura4 described a patient who had previously been diagnosed as having DH involving the trunk and extremitiesand who subsequently developed facial papular dermatitis. Histopathologic analysis revealed edematous dermal papillae infiltrated with neutrophils,lymphocytes, and eosinophils. Direct immunofluorescent studies showed fibrillar deposits of IgA along the dermoepidermal junction, consistent with DH. Helander and Jansen5 also reported a case of DH that was limited to the face. The findings of a biopsy and directimmunofluorescence were consistent with DH. The patient, who did not havesymptoms of a gluten-sensitive enteropathy, responded well to dapsone therapy. Although the confinement of lesions to the face is rare in DH, our patient presented with a history of pruritus and burning, along with evidence of small-bowelvillous atrophy, which suggested that she had DH. This suspicion was confirmed by the results of immunofluorescence testing and was additionally supportedby the dramatic response to dapsone therapy. The rare variant of DH in the present case lends credence to Duhring's assertion regarding the protean clinicalmanifestations of this disease.1 The diagnosis of DH merits consideration under the appropriate clinical circumstances, despitethe absence of classic lesional distribution. References 1. Duhring L Dermatitis herpetiformis JAMA. 1884;3225- 229Google ScholarCrossref 2. Otley CHall III RP Dermatitis herpetiformis Dermatol Clin. 1990;8759- 769PubMedGoogle Scholar 3. Trepanier Y Localized bullous dermatitis herpetiformis Arch Dermatol. 1970;10198- 99PubMedGoogle ScholarCrossref 4. Komura JImamura S Papular dermatitis herpetiformis: report of a case with localized, facial lesions Dermatologica. 1977;155350- 354PubMedGoogle ScholarCrossref 5. Helander IJansen CT Localized dermatitis herpetiformis J Am Acad Dermatol. 1987;161052- 1053PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Mar 1, 2004

Keywords: face,vesicle,macule

References