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Focal dermal hypoplasia with an initial inflammatory phase
M Mann1, R Weintraub, K Hashimoto
1Department of Dermatology and Syphilology, Wayne State University School of Medicine, Detroit, Michigan 48201.
Abstract:
We report a case of focal dermal hypoplasia with an initial inflammatory phase. Although our patient had dark red macules and papules and red linear atrophic lesions at birth, her lesions were somewhat faded at 3 months of age and had become yellowish before 5 months of age. Histologic examination of lesions at 3 months of age showed marked edema in the papillary dermis, mononuclear cell infiltration, a prominent fibroblastic population, and a large cluster of dermal lipocytes, suggesting an association between the inflammation and the development of dermal adipose tissue.
Insights
Focal dermal hypoplasia can present with an inflammatory phase, characterized by early skin lesions that evolve in color and texture. Histology reveals dermal edema and increased lipocytes, suggesting inflammation influences adipose tissue development.
Area of Science:
- Dermatology
- Pathology
Background:
- Focal dermal hypoplasia (FDH) is a rare congenital skin disorder.
- Its typical presentation involves atrophic and often linear skin defects.
Observation:
- A neonate presented with erythematous macules, papules, and linear atrophic lesions at birth.
- Lesions faded and became yellowish by 5 months of age.
Findings:
- Histological examination at 3 months revealed papillary dermal edema, mononuclear cell infiltration, and abundant fibroblasts.
- A significant cluster of dermal lipocytes was noted, suggesting active adipose tissue development.
Implications:
- This case highlights an unusual inflammatory phase in focal dermal hypoplasia.
- The findings suggest a potential link between inflammation and the development of dermal adipose tissue in FDH.