ABSTRACT
INTRODUCTION: Kaposi-Juliusberg's syndrome is a severe herpes simplex virus cutaneous infection, accompanied by general signs. Darier's disease is a rare genodermatosis characterised by keratinisation disorders. OBSERVATION: In a 51 year-old patient suffering from Darier's disease since the age of 20, a diffuse vesicular eruption occurred following a herpes eruption on the lower lip. Culture of a vesicle revealed a type 1 Herpes simplex virus. The diagnosis of Kaposi-Juliusberg's syndrome was made. COMMENTS: Kaposi-Juliusberg's syndrome is a "classical" herpes complication of atopic dermatitis. It may also develop on other predisposing territories such as acantholytic dermatitis: pemphigus vulgaris, Hailey-Hailey's disease and Darier's disease. Although a rare complication and life threatening for the patient, Kaposi-Juliusberg's syndrome can now be treated efficiently with intravenous and subsequently oral aciclovir.
Subject(s)
Darier Disease/complications , Herpes Simplex/etiology , Herpes Simplex/pathology , Acyclovir/administration & dosage , Acyclovir/therapeutic use , Antiviral Agents/administration & dosage , Antiviral Agents/therapeutic use , Herpes Simplex/drug therapy , Humans , Male , Middle Aged , Prognosis , SyndromeSubject(s)
Erythema Nodosum , Erythema Nodosum/etiology , Pregnancy Complications , Adult , Erythema Nodosum/physiopathology , Female , Humans , PregnancySubject(s)
Intestinal Diseases/complications , Sweet Syndrome/complications , Anemia, Refractory, with Excess of Blasts/complications , Anemia, Refractory, with Excess of Blasts/diagnosis , Crohn Disease/diagnosis , Diagnosis, Differential , Humans , Intestinal Diseases/diagnosis , Intestinal Diseases/pathology , Intestine, Small/pathology , Leukemia, Myeloid, Acute/complications , Leukemia, Myeloid, Acute/diagnosis , Male , Middle Aged , Skin/pathology , Sweet Syndrome/pathologyABSTRACT
INTRODUCTION: We report five cases of pigmented erythroderma occurring during AIDS, noteworthy for its unusual hyperpigmented feature, its advent at the terminal stages of AIDS, and an CD8 cells dermal infiltrate. PATIENTS AND METHODS: It is a retrospective study of five patients infected with HIV: a woman infected by transfusion and four homosexual men, average 55 years old. No one was intravenous drug user. They were all severely immunocompromised; HTLV I/II serology was negative. Skin biopsies were studied with light microscopy (Hematoxylin-eosin) and immunohistochemical studies were performed on frozen sections. RESULTS: The patients had an erythroderma of particular interest because of the associated hyperpigmentation, the severe repercussion (pruritus, weight loss), and the difficulty in treating (except systemic corticosteroids). The histology demonstrated a mononuclear dermal lymphocytic infiltrate, without epidermotropism and atypical cytonuclear feature. The phenotype of the infiltrate was uniformly of the suppressor-cytotoxic subset (CD8+, CD4-). COMMENTS: Our cases are like those previously described as "Pseudo-Sezary", mimicking a lymphoma during AIDS. Numerous factors are probably the cause of this hyperpigmented erythroderma: HIV, CD8 cells... CONCLUSIONS: This severe skin disease, complicating AIDS, seems very particular, but not yet clearly defined. In practice, the problem remains the treatment of this severe erythroderma, because only the systemic corticosteroids are effective, but this is debatable during the treatment of AIDS.
