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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Hypopigmentation in an African Child

Katie Ratzan, a third year Dartmouth Medical School student serving as a Schweitzer Fellow at the Schweitzer Hospital in Gabon, Africa, would like help and advice.
" I would like to ask your help with a six year old girl who presented to our clinic at the Hopital Schweitzer, with her father & aunt. The child has a recent onset of hypopigmentation of the left side of her face & neck. As of six to seven weeks ago, her skin was entirely normal. This change in skin color progressed over the past six weeks. It is asymptomatic. She has had no constitutional symptoms. She was not sick during the months/weeks prior to the color change, did not take any medications prior to the skin change, did not travel, did not have an accident with any sort of chemical, does not use anything on her face (i.e. cremes, etc.). No one else around her has anything like this. No one else around her is sick. She's never had this before. She now puts some sort of indigenous healing/darkening creme on the spots on the back of her neck, which is why that is darker than the areas of her face.

By history, this started on her cheek and moved toward her nose. It stops abruptly at midline. It has since spread to her neck and scalp. It's macular/patch-like depending on the confluence of abutting lesions. There is no involvement of mucous membrance (mouth & vagina are normal). She has no trouble with vision, taste, hearing, and her neuro exam (my brief version of it which essentially only tested sensation and gross motor) was normal.





Questions from Katie:
1. Does anyone think this is anything other than vitiligo?
2. Is this segmental vitiligo, and if so what special significance does this have?
3. What therapy would be appropriate for a child like this in this setting?
4. What is known of the psychological and social implications of such hypopigmentation in a girl in this setting?

Thank you,
Katie

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