Skin, Merkelsson-Rosenthal syndrome [LMP74471]

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Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Neoplasia
Diagnosis: 
Merkelsson-Rosenthal syndrome
Species: 
Human
Organ System: 
Skin
Gender: 
Female
Age: 
50 years
Diagnosis Code: 
ICD-10: L92.8
Slide Contributor Information
Yoon, J., Al-Habeeb, A.

Keywords

Merkelsson-Rosenthal syndrome, non-necrotizing granulomatous infiltrates

Clinical History

50-year old with persistent nasolabial and lip swelling, not yet diagnosed.

Clinical Discussion

Merkelsson-Rosenthal syndrome (MRS) is a rare disorder, characterized by recurring facial paralysis, swelling of the face and lips, and fissured (“scrotal”) tongue (OMIM 155900). There may be an association with Ehlers-Danlos syndrome, but MRS remains poorly characterized.

Sections show non-necrotizing granulomata in the deep dermis, extending to the subcutis. Necrosis and abscesses are not identified. Occasional giant cells are seen.

Considering the numerous, deep granulomata, another consideration is use of dermal fillers. Use of these fillers for aesthetic purposes is gaining popularity, and this histological pattern would be fitting, although the clinical correlation did not support this consideration.

Related Content

Related External Links: 
OMIM - MELKERSSON-ROSENTHAL SYNDROME