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[Atypical scleromyxedema with a granulomatous histological pattern and delayed sclerosis]
G de Cambourg1, R Goussot1, C Wettlé1
1Clinique dermatologique, hôpitaux universitaires, université de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France.
Background:
Papular mucinosis is characterised by primary mucin deposition in the dermis. The classification distinguishes between the localised form and the systemic form, which alone can result in complications, but this classification occasionally proves to be inadequate. Herein we report the progression of papular mucinosis, initially atypical due to the absence of cutaneous sclerosis and of misleading granulomatous histological features, which subsequently developed into characteristic scleromyxedema.
Patients And Methods:
A 55-year-old male developed a rash comprising countless acral papules. Several biopsies were necessary before a diagnosis of papular mucinosis was made, due to the initial granulomatous appearance at histology. Tests showed monoclonal immunoglobulin of indeterminate significance, but, due to the absence of cutaneous sclerosis, we were able to conclude on typical localised papular mucinosis. Two years later, extensive sclerotic induration of the skin appeared and the diagnosis was modified to one of scleromyxedema. Treatment with intravenous immunoglobulins was given and proved efficacious, but relapse occurred on discontinuation of the therapy.
Discussion:
Papular mucinosis is a rare disease of unknown physiopathology. The disease classification distinguishes between the localised and systemic forms, but it occasionally proves to be inadequate. Our case suggests a continuum between the localised and systemic forms of the disease. Further, the initial biopsies of acral papules in our patient had a misleading granulomatous appearance, as has been reported numerous times for the systemic forms. This granulomatous histological variant thus appears to constitute a diagnostic criterion for scleromyxedema.
Insights
This case study shows papular mucinosis can progress to scleromyxedema, highlighting diagnostic challenges. The granulomatous histological variant is a key indicator for scleromyxedema, suggesting a disease continuum.
Area of Science:
- Dermatology
- Pathology
- Immunology
Background:
- Papular mucinosis involves dermal mucin deposition, classified as localized or systemic.
- Current classifications may not fully capture disease progression.
- Atypical presentations can complicate diagnosis.
Observation:
- A 55-year-old male presented with numerous acral papules.
- Initial biopsies showed granulomatous features, leading to a papular mucinosis diagnosis.
- Absence of cutaneous sclerosis initially suggested localized disease.
Findings:
- The patient later developed extensive skin sclerosis, reclassifying the condition as scleromyxedema.
- Monoclonal immunoglobulin of indeterminate significance was detected.
- Treatment with intravenous immunoglobulins was effective but not curative, with relapse upon discontinuation.
Implications:
- This case suggests a potential continuum between localized and systemic forms of papular mucinosis.
- The granulomatous histological variant in initial biopsies may be a diagnostic marker for scleromyxedema.
- Accurate diagnosis and classification are crucial for managing this rare condition.
