Friday, September 14, 2012

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.

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?


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).”


  1. Sunil Dogra, Professor of Dermatology, Chandigarh, India writes: " Interesting presentation - I will also like to keep Poikilodermatous Mycosis Fungoides as differential diagnosis. Review of histpathology including immunostain panel (for MF) can be done. Late age of onset, lack of familial history, no true dyschromia (depigmented macules missing) are odd for diagnosis under consideration. "

  2. Fran Storrs, Portland, Oregon: I thought tinea versicolor for sure until I saw the close up. Could a weird poikiloderma and occult dermatomyositis be in play here?

  3. I think the final dx may depend on the dermatopathology here. Clinically, I also thought of progressive macular hypopigmentation, but that does not really fit. Perhaps, sending the slides for second opinions may help. Hopefully, some of VGRD's pathologists will comment. (DJE)

  4. Ana BrasileiroSeptember 16, 2012

    Professor Sunil Dogra - we also thought about Mycosis Fungoides, but the histology is not suggestive.. although it cannot be completely excluded.

    Dr. Fran Storrs - actually the first time I saw the patient I asked for other colleague opinion and she suggested pityriasis versicolor, so the patient was treated accordingly (topic + systemic antifungals) without improvement. Only on the 2nd appointment I decided to perform biopsy. I apologize for the fact I didn't describe that first prescription.

    The patient will come again next Tuesday and I will ask for a blood smear, tyroid function as well as request a Haematologist opinion to study the macrocytosis.
    Other suggestions?

  5. Poikiloderma-like cutaneous amyloidosis syndrome (PAS), a variant of macular amyloidosis as pokilodermatous skin lesions, could be considered. Usually a patient starts as macular amylodoisis and manifest itself as poikilodermatous skin lesions. Does the patient have any exposure to dioxin in the past? Whether dioxin and amyoloidosis are actually associated or not there are a few reports about this relationship. Macrocytic anemia could well be related to amyloidosis. However, his macrocytic anemia could be from gastric ulcer although they likely present with microcytic anemia...knowing he is taking omeprazole 20mg occasionally. With his age being 51, he should probably have colonoscopy done if there is any family history of GI diseases.

    It is quite a rare condition. Thank you for posting the case of your patient and please keep us updated on how the patient is doing.

    You can access to an article onPoikiloderma-like cutaneous amyloidosis syndrome.

  6. Thank you for posting this interesting case.

    Clinically this patient has asymptomatic generalised hypopigmented macules over the upper back. Apparently it extend to the arms and legs.

    i think it is difficult to make a diagnosis of amyloidosis cutis dychromica (ACD) in this patient for the following reasons.

    (1) the age of onset is 45 yrs. this is very unusual as ACD usually has its onset at a much younger age usually before 15 yrs old.

    (2) It is a hereditary disease and yet there is no family history.

    (3) though the pathologist mentioned amyloid noted in the skin biopsy, was there amyloid deposits seen in the histology? If yes, where was it? papillary or reticulated dermis?

    (4) Was there special stain done? Congo red?

    I think the overall picture is more of post inflammatory hypopigmentation or macular progressive hypopigmentation.

    The macrocytosis is probably unrelated.

  7. I agree that even though hypopigmented mf is on the differential, the path is not consistent with this diagnosis. I also agree that the path is not very convincing for systemic or epidermal amyloidosis disorders. The presentation is rather diffuse for progressive macular hypomelanosis and the reticulated pigmentation would be unusual for this disorder. Nevertheless, examination with a Wood's lamp might be performed, since it should show red fluorescence in the follicles within the hypopigmented areas if the patient has progressive macular hypomelanosis. The lack of a lichenoid band helps to rule out a lichenoid drug eruption, which can give grayish poikilodermatous areas sometimes. I agree with post-inflammatory hypopigmentation as the likely diagnosis, for which narrow band UVB three times weekly for 24-36 sessions could be tried to even out pigmentation. If new lesions develop, especially if they are associated with erythema or mild scale, another biopsy should be done to rule out mycosis fungoides, since patients with patch/plaque mf often go 3-4 years and have multiple biopsies before a diagnosis is made. (AGP)

  8. Thank you all for your opinions, for me is a very interesting discussion.

    Dr Yoon Cohen - no history of dioxin exposure. Is it probable to have a macrocytic anemia due to gastric ulcer with normal cobalamin levels? No know history of familiar GI disease, namely gastric or colonic cancer. But still performing a colonoscopy could be a good ideia (age > 50 years)

    Dr Henry Foong - thank you for confirming that, the age of onset and the absence of familiar history made me curious about this case. Amyloid deposits were seen in the papilar dermis (although I know the pictures are not good enough to see them). Congo red stain was not done as the patologist said they don't usually get good results and he was quite sure about amyloid deposits. Maybe I can insist on that, perhaps with the new biopsy I've just done 2 days ago.

    Dr Amit Pandya - the patient denies consistently any previous dermatosis, so if we believe him the post inflamatory hypopigmentation his hard to explain. Actually last Tuesday he presented with erythematous papules of the fore arms, papular, mildly pruritic. Cutaneous biopsy was done, as well as another one from a reticulated area.

    I will keep you updated about this case.
    I really appreciate your help.


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