Localized papular mucinosis with IgA nephropathy: a case report
Peng Wang1, Hanjun Yang, Yuping Ran
1Department of Dermatovenereology, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wang Hou District, Chengdu, People's Republic of China, 610041.
Background:
Papular mucinosis is an idiopathic cutaneous mucinosis. It includes 3 clinicopathologic subsets according to the recent Rongioletti classification: a generalized papular form, a localized form, and atypical forms. The localized form is subdivided into 5 subtypes. The atypical form does not meet the criteria for either the generalized papular form or the localized forms and includes localized forms with monoclonal gammopathy and/or systemic symptoms.
Observations:
We describe a 37-year-old man who had multiple small, waxy papular lesions on the neck for 4 years. One year after the lesions first appeared, he developed IgA nephropathy. No paraproteinemia or thyroid dysfunction was observed. Histologic examination of the skin lesions showed mucin deposits in the upper dermis. Alcian blue-positive mucin was also found in the renal interstitium and around some vessels and capillary loops. A diagnosis of localized papular mucinosis with IgA nephropathy (atypical form) was made after the biopsy.
Conclusions:
The patient showed an unusual manifestation of localized papular mucinosis with the lesions confined to the neck and IgA nephropathy. Alcian blue staining is a useful way to determine kidney involvement of papular mucinosis.
Insights
This study details a rare case of localized papular mucinosis presenting with IgA nephropathy. Alcian blue staining effectively identified kidney involvement in this atypical skin condition.
Area of Science:
- Dermatology
- Nephrology
- Pathology
Background:
- Papular mucinosis is a rare idiopathic cutaneous mucinosis with distinct clinicopathologic subsets.
- The Rongioletti classification defines generalized papular, localized, and atypical forms, with localized forms having further subtypes.
- Atypical forms may present with monoclonal gammopathy or systemic symptoms, challenging traditional classifications.
Observation:
- A 37-year-old male presented with a 4-year history of papular lesions on the neck.
- IgA nephropathy developed one year after the onset of skin lesions; no paraproteinemia or thyroid dysfunction was noted.
- Histopathology revealed dermal mucin deposits in skin lesions and Alcian blue-positive mucin in the renal interstitium and vasculature.
Findings:
- The patient was diagnosed with an atypical form of localized papular mucinosis associated with IgA nephropathy.
- Skin lesions were confined to the neck, representing an unusual manifestation of the condition.
- Alcian blue staining proved valuable in detecting renal interstitial and vascular mucin deposition.
Implications:
- This case highlights an unusual presentation of localized papular mucinosis with concurrent IgA nephropathy.
- The findings underscore the utility of Alcian blue staining in assessing kidney involvement in papular mucinosis.
- Further research may elucidate the pathogenic links between cutaneous mucinosis and renal disease.
Related Concept Videos
01:24Nephrotic Syndrome I : Introduction
01:26Nephrotic Syndrome II : Assessment and Medical Management
01:15Transcytosis of IgG
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...