Acquired Progressive Kinking of the Hair in the Setting of Parry-Romberg Syndrome
May 2015
in “
Journal of The American Academy of Dermatology
”
Parry-Romberg syndrome unilateral facial atrophy semifacial atrophy dermal fibroplasia lipoatrophy perivascular lymphocytic infiltrate subcutaneous adipose tissue atrophy telogen hair cuticular weathering scarring alopecia dermal sclerosis linear scleroderma androgenetic alopecia sympathectomy sympathetic innervation PRS facial atrophy hair curling hair weathering hair loss alopecia sympathetic nerve removal
TLDR A woman with Parry-Romberg syndrome developed new curly hair on one side of her scalp, a condition not previously linked to the syndrome.
The document reports on a 67-year-old female with Parry-Romberg syndrome (PRS), a condition that causes unilateral facial atrophy, who presented with new-onset left semifacial atrophy and progressive curling of the hair on the ipsilateral frontoparietal scalp over one year. A punch biopsy of the face showed mild dermal fibroplasia, focal lipoatrophy, and mild perivascular lymphocytic infiltrate, while MRI confirmed subcutaneous adipose tissue atrophy. Scalp biopsy was noninflammatory with normal follicular units and telogen hair counts. Hair microscopy showed mild cuticular weathering. The case is unique as it describes an association between acquired progressive kinking of the hair and PRS, which has not been previously documented. The kinking was temporally and anatomically related to PRS development, with no changes in the contralateral scalp hair. The absence of scarring alopecia and dermal sclerosis ruled out linear scleroderma, and the involvement of non-androgen dependent sites made androgenetic alopecia unlikely. The patient's history of sympathectomy might be relevant, as sympathetic innervation affects hair maturation and sympathectomy has been linked to PRS development in case reports and animal models.
