A skin lesion from Nigeria
A 51 year old woman from Nigeria presents with a skin lesion. What is it?
This patient had mycosis fungoides, a cutaneous T cell lymphoma. The average age of onset is between 45 and 55 and is more common in women. It was first described in 1806 byJean-Louis-Marc Alibert , a French dermatologist. The name means “mushroom – like fungal disease” but has nothing to do with a fungus but rather was named for nodules that can appear on the skin.
In the early stages the malignant cells are in the skin and can be treated with light therapy, electron beam radiationand photopheresis. As the disease progresses itcan be treated with systemic chemotherapy. The peripheral smear may show “buttock” cells.
Teaching point: There are other skin lesions which could look very similar and as with most of medicine, the history will be helpful. Pyoderma gangrenosum is more often seen in ulcerative colitis or crohns although it can be seen in RA, myelocytic leukemia, multiple myeloma and granulomatosis with polyangiitis.
pyoderma gangrenosum below:
Leprosy can present with ulcers in areas of nerve damage where sensation is impaired . These neuropathic ulcers are often on the feet and hands.
leprosy with neuropathic ulcers
Leprosy can present with ulcers in areas of nerve damage where sensation is impaired . These neuropathic ulcers are often on the feet and hands.
Leishmaniasis would have been a good guess because it also causes cutaneous ulcers. This is common in the Middle Eastand northern regions of South America. There is however, a new focus of leishmaniasis in Nigeria in the Jos East Local Government area Plateau State where 2.6% of the population have leishmaniasis. This would not have been associated with the bulky lymphadenopathy in the L neck of this patient.
Clinical Course: Our patient presented not only with skin lesions but large bulkly L cervical adenopathy. The disease is incurable, although it can be dormant for a time. In the end stages it invades solid organs.
Sezary syndrome occurs when the malignant cells are visible in the blood and the cells are filled with mucopolysaccharides.
References
Hwang ST, Janik JE, Jaffe ES, Wilson WH. “Mycosis fungoides and Sezary syndrome” Lancet 2008 371(9616): 945-57.
Alibert JLM (1806) descritions des maladies de la peau observes a l’Hopital Saint-Louis, et exposition des meilleures methods suivies pour leur traitement ( in France) Paris: Barrois l’aine p 286.
Spitalnik, S, Arinsburg, S, Jhang J. Sept. 2014 Clinical Pathology Board Review . Elsevier Health Sciences. P 310. “It is important to be able to recognize buttock cells and to know that they represent cleaved centrocytic cells of follicular lymphoma”.