Acanthosis Nigricans in an 8 year old girl

Abstract:  Eight year-old girl with acanthosis nigricans

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.

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

Dermatology Online Journal Volume 14  September 2008.  Free Open Access,  This is a good overview.

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Unusual Pigmentary Process

Presented by Dr. Ana Brasileiro

Central Lisbon Central Hospital

Lisbon, Portugal

Abstract:  51 yo man with 6 year history of pigmentary disorder.

HPI: The patient is a 51 year old male, born in Brazil and living in Portugal for 5 years. He reports that for the last 6 years he has experienced a slowly progressive dermatosis, first affecting the posterior cervical region and the superior part of the trunk, with progressive involvement of all the trunk, arms and legs. The patient denies pruritus or any other symptom. He has no contributory medical past story, and his only medication is occasional omeprazole 20mg. He has no relevant familiar history (neither the parents, brothers or children have any dermatological condition).

O/E:  This shows reticular pattern with hyper and hypopigmentation on the trunk extending to the arms and legs.

Clinical Photos:

Lab: Macrocytic anemia (Hb 11.5 g/L, VGM 106 fL) with normal levels of folic acid, B12 vitamin, as well as normal protein electrophoresis. Iron levels, ferritin and transferin are also normal (although these are not so relevant for a macrocytic anemia) The liver and kidney functions are within the normal range, and the tests for HIV, HCV and syphilis were negative. He has acquired immunity to HBV.

Histopathology: The biopsy showed poikiloderma with amyloid deposits in the superficial dermis, consistent with cutaneous discromic amyloidosis.

Diagnosis: Amyloidosis cutis dyschromica

Questions: What is your opinion about this case?
From your experience, do you know of any case of dyscromic cutaneous amyloidosis starting at the age of 45?
Should we consider other diagnosis?
Do you think that the macrocytic anemia is somehow related with this cutaneous condition?

References: 
1. Amyloidosis cutis dyschromica in two female siblings: cases report; Yang et al. BMC Dermatology 2011, 11:4 “This disorder is characterized by the following features: (i) dotted, reticular hyperpigmentation with hypopigmented spots without papulation almost all over the body; (ii) no or little itchy sensation; (iii) onset before puberty; and (iv) small foci of amyloid closely under the epidermis “

2.Familial amyloidosis cutis dyschromica; Karadag, A.; Simsek, G; Turk J Med Sci 2010; 40 (1): 151-154 “In the literature, there are a few reports of familial cases (…). Cases accompanying autoimmune connective tissue diseases and primary biliary cirrhosis were reported in the other primary cutaneous localized amyloid types, such as macular and lichenoid amyloidosis (7). However, no another systemic and dermatological disease accompany ACD. In the literature there is only one case accompanying generalized morphea (5).”

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Unusual Pigmentation of Legs

The patient is a healthy 51 yo woman with a 5 - 6 year history of asymptomatic progressive hyperpigmentation of the legs. She is in good general health and takes no medications by mouth. The process started on the calves and has spread proximally to the knees. She has rosacea in addition. She thinks her father has a similar problem.

O/E: Both legs from just above the ankles to the knees show punctate hyperpigmentation. The skin here has a slightly pebbly feel. Other than erythematous papules on both cheeks, the remainder of the cutaneous exam is normal. (There is no sclerodactyly, telangiectasas or sclerotic changes).

Photos:
Affected skin


Digital Zoom


Normal Skin (adjacent)


Dermoscopic Image


Lab: Biopsies of affected and normal skin were taken.

Diagnosis: Punctate Hyperpigmentation of the Legs. This does not look like the "salt and pepper" picture of scleroderma. Could this be an unusual genodermatosis?

Plan: Present to VGRD. Perhaps get serologies for scleroderma.

References: Nothing helpful found on PubMed.

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