Abstract
Background
Phymatous rosacea is characterized by thickened skin and irregular surface contours as the result of variable combinations of fibrosis, sebaceous hyperplasia and lymphoedema. Otic phyma is rarely seen and has been rarely reported in the English literature.
Methods
We present another case of this uncommon condition, frequently misdiagnosed, maybe due to its underrecognition.
Results
Phymatous rosacea most commonly occurs on the nose, but may also develop on any sebaceous facial region, including the ears. Although it is a benign condition, there are significant morbidities associated with rosacea, and can even result in conductive deafness because of the obstruction of the external auditory canal.
Conclusions
The knowledge of this entity may be important for clinicians, especially dermatologists, ENT specialists and plastic surgeons, for an appropriate treatment and follow-up.
1
Introduction
Phymatous rosacea is characterized by patulous follicular orifices, thickened skin, nodularities and irregular surface contours as the result of variable combinations of fibrosis, sebaceous hyperplasia and lymphoedema. Otophyma, or otic phyma, has been rarely reported in the English literature. It is an uncommon and frequently misdiagnosed condition, maybe due to its underrecognition. While medical management may be beneficial for early to moderate phymatous changes, advanced phymatous rosacea needs surgical therapy to remove the hypertrophied tissue.
2
Case report
A 49-year-old healthy man was referred to our department for evaluation because of an 18-month history of slowly progressive ear swelling which had recently become very itchy. He had been previously diagnosed and treated as eczematous otitis externa without improvement despite topical antibiotics and steroid treatment. He denied pain, hearing loss or systemic symptoms and there was no history of trauma. On physical examination, his right ear showed mild erythema and diffuse enlargement characterized by skin thickening and edema, associated with accentuated adnexal pores and patulous follicular ostia (‘peau d’orange’ appearance) most prominently involving the conchal fossa ( Fig. 1 ). The skin was adherent to underlying tissues with no fluctuation. The swelling was not associated with any bleeding, pus or fluid discharge, and no scale was noted. There was neither similar swelling elsewhere nor palpable lymph nodes in the head and neck, and all routine blood test were within normal limits. A punch biopsy revealed diffuse edema, most marked in the superficial dermis, with a nodular granulomatous infiltrate composed of epithelioid cells, few histiocytic giant cells and admixed lymphocytes filling the upper interfollicular dermis ( Fig. 2 ). Also present were prominent sebaceous glands and dilated follicular infundibula containing keratinous debris and Demodex mites ( Fig. 3 ). Correlating the pathology and the clinical picture, otophyma was diagnosed. We initiated treatment with metronidazole 0.75% gel twice a day and doxycycline at doses of 100 mg/d. At 4 months of treatment, the patient showed improvement both in symptoms and cutaneous manifestations, with a reduction of the skin thickness, ear size, edema and erythema ( Fig. 4 ). Thus, doxycycline was tapered to 100 mg 3 days a week maintenance and treatment with metronidazole remained.
2
Case report
A 49-year-old healthy man was referred to our department for evaluation because of an 18-month history of slowly progressive ear swelling which had recently become very itchy. He had been previously diagnosed and treated as eczematous otitis externa without improvement despite topical antibiotics and steroid treatment. He denied pain, hearing loss or systemic symptoms and there was no history of trauma. On physical examination, his right ear showed mild erythema and diffuse enlargement characterized by skin thickening and edema, associated with accentuated adnexal pores and patulous follicular ostia (‘peau d’orange’ appearance) most prominently involving the conchal fossa ( Fig. 1 ). The skin was adherent to underlying tissues with no fluctuation. The swelling was not associated with any bleeding, pus or fluid discharge, and no scale was noted. There was neither similar swelling elsewhere nor palpable lymph nodes in the head and neck, and all routine blood test were within normal limits. A punch biopsy revealed diffuse edema, most marked in the superficial dermis, with a nodular granulomatous infiltrate composed of epithelioid cells, few histiocytic giant cells and admixed lymphocytes filling the upper interfollicular dermis ( Fig. 2 ). Also present were prominent sebaceous glands and dilated follicular infundibula containing keratinous debris and Demodex mites ( Fig. 3 ). Correlating the pathology and the clinical picture, otophyma was diagnosed. We initiated treatment with metronidazole 0.75% gel twice a day and doxycycline at doses of 100 mg/d. At 4 months of treatment, the patient showed improvement both in symptoms and cutaneous manifestations, with a reduction of the skin thickness, ear size, edema and erythema ( Fig. 4 ). Thus, doxycycline was tapered to 100 mg 3 days a week maintenance and treatment with metronidazole remained.
