Self-healing Juvenile Cutaneous Mucinosis: A Case Report In The Middle East
Geagea, C., Youssef, N., & Wakim, G. (2019). Self-Healing Juvenile Cutaneous Mucinosis: A Case Report in the Middle East. The American journal of case reports, 20, 65–69. https://doi.org/10.12659/AJCR.910047
View Original Source →Abstract
BACKGROUND Self-healing juvenile cutaneous mucinosis (SHJCM) is a rarely diagnosed disease worldwide, with less than 20 reported cases in the literature. It is characterized by a rather benign course in juvenile patients with nodular and mucinous skin eruption and edema. CASE REPORT A 12-year-old male patient previously healthy presented to the pediatrics clinic with a 1-week history of bilateral palmer pruritus and plantar tenderness upon walking, preceded by eruption of erythematous patch on his neck. The disease course evolved to include facial edema, erythema, nodular skin eruptions with a completely negative initial workup. The patient was labelled as a juvenile idiopathic arthritis patient and doomed to be a candidate for corticosteroid therapy. Upon further workup, a skin biopsy was taken and SHJCM was diagnosed. Complete resolution of symptoms was witnessed on symptomatic treatment after 5 months of diagnosis. CONCLUSIONS To our knowledge, this is the second case of SHJCM reported in the Middle East and the first to be reported in Lebanon. It is also the first case reported to have the longest follow-up period; 10 years of follow-up with no new findings or relapse. SHJCM is a rare disease whereby awareness of its features and presentation may help in diagnosing it and preventing unnecessary testing and aggressive treatment for a rather benign disease.
Case Details
Disease Location
Skin
Personal Characteristics
12-year-old male
Clinical Characteristics
Present for a 1-week history of bilateral palmar pruritus and plantar pain. Three days prior to presentation, the parents also noticed the appearance of a non-pruritic, erythematous patch on the posterior aspect of his neck. The erythematous patch extended from the neck to the upper back. One week after the initial presentation, the patient returned with frontal, periorbital, and upper lip edema and erythema. Peri-articular nodules appeared on the proximal interphalangeal joints bilaterally with a reported increase in pruritus and pain in the soles upon ambulation. Clinical diagnosis of juvenile idiopathic arthritis was evoked despite negative serologies: antinuclear antibody (ana), anti-scl (anti- topoisomerase), rheumatoid factor (rf). The patient was started on nonsteroidal anti-inflammatory drugs and desloratadine. Three weeks after initial presentation, the patient was no longer able to walk due to the plantar edema and knee pain. Similar nodules also appeared on his nose, fingers, and left elbow. The parents also reported bluish discoloration of the fingertips. Excisional biopsy of an elbow nodule showed fibrovascular proliferation in a septolobular pattern, reminiscent of proliferative fasciitis, set within myxoid stroma, dissociating and surrounding some fatty islands. The tissue also showed polymorphous inflammatory cells and essentially numerous large gangliocyte-like cells. These features were most consistent with the diagnosis of self-healing juvenile cutaneous mucinosis
Remission Characteristics
Within 5 months of onset of symptoms, the patient had complete resolution of his nodules with no more pain.
Treatment & Mechanisms
Clinical Treatment
Biopsy
Non-Clinical Treatment
None reported