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
0 comments:
Post a Comment