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Unusual primary cutaneous amyloidosis with an incontinentia pigmenti‐like pattern

Primary cutaneous amyloidosis (PCA), defined as the deposition of amyloid in previously normal-appearing skin with no systemic involvement, is classified into three types: macular amyloidosis, lichen amyloidosis, and the rare nodular amyloidosis. Macular amyloidosis usually presents as moderately pruritic, brownish macules and patches with a reticulate or rippled pattern on the upper back, chest, and limbs. The rare clinical presentations of diffuse macular amyloidosis include nevoid-like hyperpigmentation, widespread diffuse pigmentation, poikiloderma-like presentation, and incontinentia pigmenti-like pattern. In this study, we report a case of unusually diffuse macular amyloidosis with an incontinentia pigmenti-like pattern. A 28-year-old woman presented with diffuse nonpruritic hyperpigmentation that had existed since early childhood. The skin lesions had first appeared on her back and then slowly extended to her trunk and both extremities, and no verrucous or blistery lesions had been previously noted. As the woman aged, the rashes had grown in proportion to her body size. She had no significant past medical history and no family history of any similar condition, and physical examination revealed her to have a normal development. The lesions on her skin consisted of multiple brownish patches and macules. Reticulated and linearly arranged patches and macules located on the trunk as well as the upper and lower extremities were found that reminded us of incontinentia pigmenti. The pigmentation was erratically patterned, with irregularly shaped splashes, lines, whorls, and fountain spray-like splatters that followed Blaschko’s lines in some places (Fig. 1). We detected no symptoms or signs suggesting systemic amyloidosis, such as macroglossia, muscle or joint dystrophy, or hepatosplenomegaly. A skin biopsy specimen from a pigmented patch on the patient’s back showed that dermal papillae were expanded by globules and amorphous deposits of eosinophilic material, and some necrotic keratinocytes were noted in the epidermis (Fig. 2). Laboratory investigations included a complete blood cell count, biochemical profiles, and serum immunoglobulin analysis, all of which were either negative or normal. A chest x-ray was taken, which also showed normal results. The patient was diagnosed with primary cutaneous amyloidosis and treated with oral administration of acitretin capsules and topical application of tretinoin cream. There are three recognized types of primary cutaneous amyloidosis. Macular and lichen amyloidosis are the most common. Diffuse macular amyloidosis with an