ABSTRACT
INTRODUCTION: Pemphigus herpetiformis is a rare entity that combines the clinical features of dermatitis herpetiformis with the immunologic and histological features of pemphigus. It generally affects adults and pediatric cases are uncommon. We report a case of pemphigus herpetiformis in a 6-year-old girl treated successfully with dapsone. OBSERVATION: A 6-year-old girl presented with a pruritic, erythematous and vesiculous eruption. Histological examination showed acantholysis of the medial and superficial parts of the epidermis. Direct immunofluorescence study revealed cell surface immunoglobulin G and C3 deposits and staining on the dermo-epidermal junction. The child was diagnosed as having pemphigus herpetiformis. DISCUSSION: Pemphigus herpetiformis produces clinically a pruritic and atypical eruption that resembles dermatitis herpetiformis, with standard histology and immunofluorescence patterns recalling those of pemphigus. Considered to have a good prognosis, it responds well to dapsone and/or to low dose oral corticosteroids. The age range of onset varies from 31 to 81 years according to studies, without a male or female predilection. This is a report of a 6-year-old girl presenting with pemphigus herpetiformis, successfully treated with dapsone for two years with no relapse.
Subject(s)
Pemphigus/diagnosis , Child , Dapsone/therapeutic use , Dermatitis Herpetiformis/diagnosis , Dermatologic Agents/therapeutic use , Female , Humans , Pemphigus/drug therapy , Pemphigus/pathology , Prednisone/therapeutic use , Treatment OutcomeABSTRACT
BACKGROUND: Dermatitis artefacta is a self-induced chronic disease in which the responsibility for the lesions is uniformly denied by the patient. The latter is not aware of the reason for self-destruction. The diagnosis of such a condition is difficult because of multiple clinical aspects and the fear of missing an organic disease. CASE REPORTS: We report on seven cases of dermatitis artefacta with a chronic recurrent course. The cutaneous findings, the means involved in provoking lesions, the treatment modalities as well as the patients' psychological profile may vary from one case to another. DISCUSSION: Criteria for diagnosis and management are discussed and compared with literature data. An inappropriate approach may cause the loss of the patient and the recurrence of the lesions. Psychotherapy is indicated even though prognosis is poor.
