Article Review and Case Report of Cutaneous Rosai-Dorfman Disease with Pinna Involvement

Abstract

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is considered a benign affection of unknown cause. However, it is thought that alterations in the immune system may cause the disease. It was described in 1969 by Rosai and Dorfman; ever since, about 600 cases of cutaneous affection have been reported around the world. In Mexico, there have been only eighteen cases. The extranodal affections occur in the 43%, including: skin, nasal cavity, paranasal sinus, the orbits, bones, and the central nervous system. A histological study is important for diagnosis, where a histiocyte and plasma cell infiltration shows histiocytes positive for S100 and CD68 protein. This is the case report of an eleven year old male allergic to penicillin, gentamicin, and clindamycin, with no other relevant clinical history.The patient was referred to the emergency department due to massive bilateral pinna growth, as well as non-painful erythematous nodular lesions. The patient added that he went for a swim a week before showing any symptom. The diagnosis: pinna perichondritis. Antibiotics and non-steroidal anti-inflammatories were prescribed, though the lesions did not improve. In a requested immunological profile, BHC, acute phase reactants were reported normal. A biopsy showed an inflammatory reaction throughout the dermis and the perichondrium, excluding the ear cartilage. The pathology outcome shows: moderate chronic perichondritis, histological and morphological changes that match the cutaneous Rosai-Dorfman disease. The immune-histochemical studies performed with S-100 positive CD-1a negative, confirm diagnosis. Apart from the eighteen cases reported in Mexico since 1969, only one has been reported with cutaneous bilateral pinna affection.