Progressive Facial Hemiatrophy

A 30-year-old female patient had a 15-year history of progressive linear hyperpigmentation and atrophy of the face. Scleroderma en coup de sabre with trimipramine-induced hyperpigmentation had been diagnosed elsewhere. Headaches, light sensitivity, and visual disturbances since the age of 17 had been considered a complex pain syndrome (migraines, cluster headaches, tension headaches, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing [SUNCT]). Neurological examination and imaging of the brain were normal. Clinical findings included linear, sharply demarcated livid atrophy on the right paramedian side, accompanied by hyperpigmentation, asymmetry, and atrophy of the right half of the face (Figures 1 and 2). The rest of the integument was normal. Systemic sclerosis was ruled out. MRI of the skull, face, and brain revealed no bone erosion, muscle atrophy, or morphological changes. Neurological and ophthalmological findings were normal. Based on the skin findings and neurological symptoms, we diagnosed Parry–Romberg syndrome, a rare neurocutaneous disorder (prevalence of approximately 1/700 000, common in children and young adults). There is no known curative treatment. After slowly progressing over an average period of 7–9 years, the disease comes to a standstill.

Figure 1

Asymmetry, fat atrophy on the right

Figure 2

Asymmetry, fat atrophy on the right, hyperpigmentation from the right nasolabial fold to the neck

Siba Makhlouf, Klinik für Dermatologie und Venerologie, Helios Klinikum Krefeld, [e-mail]

Conflict of interest statement: The authors state that no conflict of interest exists.

Translated from the original German by Christine Rye.

Cite this as: Makhlouf S: Progressive facial hemiatrophy. Dtsch Arztebl Int 2025; 122: 522. DOI: 10.3238/arztebl.m2025.0123