Presented by:
Dr.
Soheila Sotoudeh
Children’s
Medical Center
Tehran,
Iran
History: This 8- year-old girl, born of nonrelative
parents, presented
with a one year history of darkening and thickening of body folds especially neck and axilla. It began with pruritic, hyperpigmented and corrugated
plaques on her neck, axilla, groin and perioral and periumbilical area.
Family history is negative for any skin disease. Drug history: levothyroxine
Her disease is gradually progressive.
O/E: She is otherwise healthy. Her weight and height is in 50 percentile. There are hyperpigmented and corrugated plaques on her neck, axilla, groin and perioral and periumbilical area.
with a one year history of darkening and thickening of body folds especially neck and axilla. It began with pruritic, hyperpigmented and corrugated
plaques on her neck, axilla, groin and perioral and periumbilical area.
Family history is negative for any skin disease. Drug history: levothyroxine
Her disease is gradually progressive.
O/E: She is otherwise healthy. Her weight and height is in 50 percentile. There are hyperpigmented and corrugated plaques on her neck, axilla, groin and perioral and periumbilical area.
Photos:
Lab: Routine hematologic and biochemical parameters (including
blood glucose and insulin level) were normal. Chest x-ray and abdominopelvic sonography were normal. Clinical screening for evidence of internal malignancy was negative.
Histopathology: Skin biopsy showed hyperkeratosis, papillomatosis and
acanthosis, mild pigment incontinence and a sparse perivascular
inflammatory dermal infiltrate.
Diagnosis: Acanthosis nigricans
Comments and Questions: My diagnosis was Acanthosis Nigricans. I have prescribed Acitretin 0.5 mg/kg for more than 3 months but without remarkable response.
1-Do
you agree with the diagnosis of acanthosis nigricans?
2-What
type of acanthosis nigricans does she have?
3-Do
you recommend genetic testing?
4-What
other treatment do you recommend?
References:
1.
Remission of acanthosis nigricans,
hypertrichosis, and Hashimoto's thyroiditis with thyroxine replacement.
Dix
JH, Levy WJ, Fuenning C. Pediatr
Dermatol. 1986 Sep;3(4):323-6.
Abstract:
Hypothyroidism is not commonly associated with acanthosis nigricans (AN). We
examined a 13-year-old girl with AN, hypertrichosis, and Hashimoto's
thyroiditis. Overt biochemical hypothyroidism, thyroid enlargement, and
positive titers of antimicrosomal and antithyroglobulin antibodies confirmed
Hashimoto's thyroiditis. Both AN and hypertrichosis resolved with thyroid hormone
replacement. There was no evidence of insulin resistance, polycystic ovarian
disease, lipoatrophy, or other endocrine dysfunction, or of malignancy. In two
patients from the literature with AN and hypothyroidism, AN was attributed to
associated thyroid carcinoma or insulin resistance, rather than coexisting
hypothyroidism. Since the skin lesions improved with thyroid hormone therapy in
those two patients and in ours, hypothyroidism appears to be directly involved
in the pathogenesis of AN.
2.
A Case of Generalized Acanthosis
Nigricans with Positive Lupus Erythematosus-Related Autoantibodies and
Antimicrosomal Antibody: Autoimmune Acanthosis Nigricans? Y. Kondo,et. al. Case Rep Dermatol. 2012
Jan-Apr; 4(1): 85–91. Free Full Text Open Access.
3. Acanthosis nigricans: A practical approach to evaluation and
management
Steven P Higgins MD1, Michael Freemark MD2, Neil S Prose MD3
from Reuven Sobel, Kibbutz Revivum, Israel: "Thanks for asking for my thoughts but I am sorry to say I have no bright ideas--nothing from my (limited) experience with acanthosis nigricans ( mostly in PCO w severe insulin resistance) and nothing from a quick search on PubMed. The only wild thought I have after seeing the reference to the patient who responded to LT4 replacement therapy is to wonder if lT3, applied topically would be effective even though this child is, I assume, well controlled on systemic LT4. ALhough LT4 should be an adequate source of LT3, the active hormone, there are defects in the deiodinase system which converts LT4 to LT3 at the organ level."
ReplyDeleteComment from Dr. Henry Foong, Ipoh, Malaysia: "
ReplyDeleteI agree with the diagnosis of acanthosis nigricans.
Acanthosis nigricans can be associated with endocrinopathy especially with insulin resistance diabetes and thyroiditis.
I would check her fasting glucose level, thyroid function tests and thyroid antibodies. If any of this is abnormal, I will try to optimise the underlying endocrine abnormality.
Usually advise patient on weight loss and diet control. I am not sure if topical creams help but can try topical tretinoin.
I would probably not use acetretin in this young patient as long term side effects of acetretinoin eg musculo-skeletal problems such as hyperostosis and calcification of the ligaments."
Agree with the diagnosis. I'd recommend a complete endocrinologic investigation. In my limited experience, metformin works pretty fine for this condition. Omid
ReplyDelete