Isabelle Luchsinger1, Jérôme Coulombe2, Franco Rongioletti3, Marc Haspeslagh4, Anne Dompmartin5, Isabelle Melki6, Rawane Dagher7, Brigitte Bader-Meunier8, Sylvie Fraitag9, Christine Bodemer10. 1. Department of Dermatology, Centre National de Référence des Maladies Génétiques à Expression Cutanée (MAGEC), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. Electronic address: isabelle.luchsinger@kispi.uzh.ch. 2. Department of Dermatology, Centre National de Référence des Maladies Génétiques à Expression Cutanée (MAGEC), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Dermatology, Centre Hospitalier Universitaire Ste-Justine, Montréal, Quebec, Canada. 3. Department of Medical Science; Unit of Dermatology, University of Cagliari, Cagliari, Italy. 4. Dermatopathology Ghent, Ghent University Hospital, Ghent, Belgium; Department of Dermatology, Ghent University Hospital, Ghent, Belgium. 5. Department of Dermatology, Centre Hospitalier Universitaire de Caen, Caen, France. 6. Department of Pediatrics, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Immunology-Hematology and Rheumatology, Assistance Publique-Hôpitaux de Paris, Necker Hospital, Paris, France; Unité INSERM U 1163, University Paris-Descartes; Institut Imagine, Sorbonne Cité University, Paris, France. 7. Department of Pediatrics, Notre Dame De Secours University Hospital, Byblos, Lebanon. 8. Department of Pediatric Immunology-Hematology and Rheumatology, Assistance Publique-Hôpitaux de Paris, Necker Hospital, Paris, France. 9. Department of Pathology, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 10. Department of Dermatology, Centre National de Référence des Maladies Génétiques à Expression Cutanée (MAGEC), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; Unité INSERM U 1163, University Paris-Descartes; Institut Imagine, Sorbonne Cité University, Paris, France.
Abstract
BACKGROUND: Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder, and its pathogenesis and long-term prognosis are unknown. OBJECTIVE: To elucidate the clinical and histopathologic characteristics, pathogenesis, and outcome in patients with SHJCM. METHODS: Retrospective study of 9 patients with SHCJM. To complement initial findings, data collection forms were sent to the referring physicians. RESULTS: All patients had an acute onset of firm nodules. Of the 9 patients, 6 presented initially with waxy papules on the dorsum of the hands; 5 suffered from periorbital edema, and 6 had a febrile prodrome. Histopathologic assessment of the papules revealed dermal mucin deposition, whereas the nodules showed proliferative fasciitis-like features or nonspecific chronic lobular panniculitis. Laboratory studies elicited evidence of active viral infection in 2 patients (human herpes virus 6 and rotavirus). Seven cases had spontaneous resolution within 6 months, and 2 patients with incomplete resolution showed subsequent transition to fibroblastic rheumatism and an autoinflammatory rheumatologic disease, respectively. LIMITATIONS: This was a retrospective study with incomplete data from referring physicians. CONCLUSIONS: Although spontaneous complete regression is expected, patients with SHJCM need long-term follow-up because of the possible development of dematorheumatolgic conditions. The pathogenetic role of microbial agents deserves further investigation.
BACKGROUND: Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder, and its pathogenesis and long-term prognosis are unknown. OBJECTIVE: To elucidate the clinical and histopathologic characteristics, pathogenesis, and outcome in patients with SHJCM. METHODS: Retrospective study of 9 patients with SHCJM. To complement initial findings, data collection forms were sent to the referring physicians. RESULTS: All patients had an acute onset of firm nodules. Of the 9 patients, 6 presented initially with waxy papules on the dorsum of the hands; 5 suffered from periorbital edema, and 6 had a febrile prodrome. Histopathologic assessment of the papules revealed dermal mucin deposition, whereas the nodules showed proliferative fasciitis-like features or nonspecific chronic lobular panniculitis. Laboratory studies elicited evidence of active viral infection in 2 patients (human herpes virus 6 and rotavirus). Seven cases had spontaneous resolution within 6 months, and 2 patients with incomplete resolution showed subsequent transition to fibroblastic rheumatism and an autoinflammatory rheumatologic disease, respectively. LIMITATIONS: This was a retrospective study with incomplete data from referring physicians. CONCLUSIONS: Although spontaneous complete regression is expected, patients with SHJCM need long-term follow-up because of the possible development of dematorheumatolgic conditions. The pathogenetic role of microbial agents deserves further investigation.