Ofuji papuloerythroderma in an elderly woman with atopic erythroderma

To the Editor A 9-year-old Caucasian girl was referred to our clinic for hyperpigmented macules on her face and body. Four years previously subcutaneous nodules that had appeared on her knees and the occipital area were diagnosed as Schwannomas histologically. The ‘brownish’ skin lesions on her face, legs and sacral area, present since birth, were thought to be café-au-lait macules and the diagnosis was neurofibromatosis. The nodules that were not excised had curiously disappeared over time. Later, her mother became concerned when more macular lesions appeared on her arms and legs and the girl was referred to us. When questioned, the child complained of bone pain, occasional pruritus and urticaria. Physical examination revealed light pinkish-brown macules with tiny telangiectatic vessels close to the periphery of the lesions, the biggest ones on the sacral and right preauricular area, measuring 8 cm and 5 cm in diameter, respectively (figs 1 and 2). These ‘congenital’ lesions were warmer than the adjacent normal skin and neither the normal (dermographism) nor the lesional (Darier’s sign) skin urticated on stroking. The lesions did fade slightly with diascopy. The newly appearing lesions were smaller tan macules with few or no telangiectasia and were not warm. There were two new subcutaneous lesions on the left elbow. The liver was slightly enlarged on palpation and ultrasonographically. There was no splenomegaly or lymphadenopathy. Serum levels of liver transaminases and alkaline phosphatase were slightly above normal limits. Two per cent basophilia was noted on the peripheral blood smear. The results of the other laboratory tests and imaging studies, including biochemical analyses, protein electrophoresis, blood coagulation tests, hepatitis serology, cranial magnetic resonance imaging, chest X-rays, bone scan, bone marrow aspiration and biopsy were within the normal limits. A 24-h urine collection for methylimidazoleacetic acid was within normal limits. Because of the lack of permission from the parents, bone and liver biopsies were not performed. Histopathological examination of the macule on the sacral area showed hyperpigmentation in the basal layer, superficial perivascular inflammatory infiltrate with scattered spindle-shaped mast cells and telangiectatic dermal vessels (figs 3 and 4). A diagnosis of telangiectasia macularis eruptiva perstans (TMEP) was suspected. The most frequent site of organ involvement of mastocytosis is the skin. TMEP is generally agreed to be cutaneous mastocytosis, an uncommon variant of urticaria pigmentosa. 1 It appears most frequently in adults and only rarely affects young children or infants. Systemic involvement is more common in adult forms of mastocytosis. 2 Systemic signs and symptoms depend on the specific organ system affected. Bone pain in the legs of our patient was the only complaint that can be considered a systemic manifestation of mastocytosis, and it is