Cutaneous hyperneury: a new entity or an atypical cutaneous manifestation of MEN 2B?

A 33-year-old healthy woman with a family history of hyperlipidemia presented with asymptomatic skin-colored yellowish linear transverse plaques and papules on the trunk and forearms for a period of 6 months (Figure 1). She noticed that the lesions during this period have been gradually increasing in size and number. The initial clinical impression was eruptive xanthomas. A serum lipid profile showed a total serum cholesterol level of 406 mmol/L (high-density lipoprotein, 1.38 mmol/L; low-density lipoprotein, 2.73 mmol/L) and a triglyceride level of 1.1 mmol/L, which is within the normal range. A 4-mm punch biopsy was performed from the skin lesion on the patient's forearm, and findings showed the presence of bundles of mature nerve fibers in the papillary dermis (Figure 2) with a normal overlying epidermis. No abnormalities were seen in the subcutaneous tissue. There was no evidence of lipid deposition. A diagnosis of cutaneous hyperneury was made.

Congenital lupus erythematosus.

Neonatal lupus erythematosus (NLE) is an autoimmune disease affecting the fetus as a result of transplacental transfer of anti-Ro autoantibodies. Typically, it presents in the first few months of life with an annular form of subacute cutaneous lupus erythematosus. We report an unusual case of NLE presenting at birth with scaly erythematous telangiectatic patches and macules with skin atrophy involving the face, head, and upper trunk. Thrombocytopenia was discovered on laboratory investigations. Histopathology of skin biopsy was consistent with subacute cutaneous lupus. The mother was clinically free of disease and had no family history of autoimmune disease. Serology (extra-nuclear antigens) was positive in both the baby and the mother. This is a rare presentation of a rare disease.