Self-healing juvenile cutaneous mucinosis: Clinical and histopathologic findings of 9 patients : The relevance of long-term follow-up - 12/05/18
, Jérôme Coulombe, MD a, b, Franco Rongioletti, MD, PhD c, Marc Haspeslagh, MD d, e, Anne Dompmartin, MD, PhD f, Isabelle Melki, MD g, h, i, Rawane Dagher, MD j, Brigitte Bader-Meunier, MD h, Sylvie Fraitag, MD k, Christine Bodemer, MD, PhD a, iAbstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : child, cutaneous mucinosis of infancy, fasciitis, lichen myxoedematosus, mucinosis, nodules, papular mucinosis, self-healing juvenile cutaneous mucinosis
Abbreviation used : SHJCM
Plan
| Dr Luchsinger is currently affiliated with the Department of Pediatric Dermatology, University Children's Hospital Zurich, Switzerland. |
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| Funding sources: None. |
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| Conflicts of interest: None disclosed. |
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| Reprints not available from the author. |
Vol 78 - N° 6
P. 1164-1170 - juin 2018 Retour au numéro
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