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Print version ISSN 0365-0596
An. Bras. Dermatol. vol.86 no.4 supl.1 Rio de Janeiro July/Aug. 2011
An unusual presentation of macular amyloidosis*
Bárbara Lima Araújo MeloI; Igor Santos CostaII; Clara de Assis Martins GoesIII; Celina Aguiar Frota TigreIII; Nara Frota AndréIV
IAssistant Dermatologist, Dona Libânia Dermatology Center, Fortaleza, CE, Brazil
IIPathologist and Dermatology Residency Preceptor, Dona Libânia Dermatology Center, Fortaleza, CE, Brazil
IIIResident Dermatologist, Dona Libânia Dermatology Center, Fortaleza, CE, Brazil
IVMedical Student, State University of Ceará (UECE), Fortaleza, CE, Brazil
Macular amyloidosis is a form of cutaneous amyloidosis characterized by dusky-brown lesions usually located on the upper back between the shoulder blades. This report describes the case of a 45year-old female presenting with hyperpigmented macules and lace-like, non-pruritic erythema in the sacral and cervical region as well as on both arms and legs. Histology revealed amyloid deposits in the papillary dermis which exhibited apple-green birefringence after Congo red staining. There were no systemic findings. This is a case of macular amyloidosis with an unusual presentation. The literature on the subject is also reviewed.
Keywords: Amyloidosis; Congo Red; Hyperpigmentation
Macular amyloidosis (MA) was first described in 1952 by Palitz and Peck.1 Although its pathogenesis is unknown, many risk factors have been described, including ultraviolet B light, Epstein Barr virus, race, genetic predisposition and atopy. The use of nylon towels or brushes on the skin has also been described as a risk factor for the development of MA.
Onset is typically during puberty, and the condition has an equal distribution between the sexes2. It may also be associated with progressive systemic sclerosis, systemic lupus erythematosus, pachyonychia and other systemic diseases. 2
Lesions are characterized by lace-like, reddishbrown stains usually located on the upper back and, more infrequently, on the arms, chest and legs. ³
Typical histological findings are an amorphous amyloid deposit in the papillary dermis between the interpapillary pegs, where foci of pigmentary incontinence can frequently be found. When stained with Congo red and viewed under polarized light, the amyloid substance exhibits clear birefringence. Direct immunofluorescence can reveal IgG, IgM and C3 in the amyloid deposits. 4
A 45-year-old, dark-skinned housewife was followed up after presenting with a one-year complaint of dark, non-itching lesions on her arms, neck, lower back and legs. Dermatological examination revealed irregular, reddish-brown, lace-like macules on the outer face of the arms, back of the neck, sacral region and legs. The patient did not present with any systemic complaints, and there were no abnormal findings on physical examination other than those already described.
Histopathological examination of the cutaneous lesion revealed significant quantities of an amyloid deposit in the papillary dermis, which could be more clearly observed after Congo red staining, as well as foci of pigmentary incontinence. Laboratory findings were as follows: Hb: 13.8; WBC count: 4380 (52%/ 1%/ 36%); platelet count 201,000; creatinine 0.6; alkaline phosphatase 200; urea nitrogen 20; glucose levels 105; aspartate transaminase 29; alanine transaminase 43; total protein 7.2; albumin/globulin ratio 1.43; and protein electrophoresis: albumin 4.25, a-1 globulin 0.22, a-2 globulin 0.56, b-1 globulin 0.40, b-2 globulin 0.40 and g-globulin 1.37.
After systemic involvement had been excluded, topical photoprotection was started and the patient was followed up.
Amyloidoses can be systemic or localized. The forms of greatest dermatological interest are localized primary cutaneous amyloidoses, which include macular amyloidosis, lichen amyloidosis and nodular amyloidosis. 5
MA is a primary cutaneous form and is characterized by hyperpigmented macules on the upper back, particularly between the shoulder blades. 3 The case described here is both unusual and extensive and presents symmetrically on the arms and legs, as well as in the sacral region and back of the neck (Figures 1, 2, 3 and 4). Histopathological findings of an amyloid deposit in the dermal papillae close to the basal layer of the epidermis confirmed the diagnosis (Figure 5). There was also mild pigmentary incontinence, resulting in the hyperpigmentation observed clinically. 6
Primary cutaneous amyloidosis (PCA) has an incidence that varies significantly with ethnic origin and is more common in South America and Asia than in North America and Europe. This suggests that genetic, environmental and even cultural factors may be implicated in PCA. 7 This type of amyloidosis is more frequent in females, particularly those in the 21to 50-year age range, suggesting that hormonal factors may be involved in the etiopathogenesis of the disease. 2 The patient described here is South American and lies in this age range.
There is a strong association between localized cutaneous amyloidosis and notalgia paresthetica. 8 Most cases of MA in the area between the shoulder blades are attributed to this syndrome. 9 This patient, however, denied itching of the lesions and had macules in unusual locations, as previously described. Furthermore, itching, which is common in PCAs, may not be present in the macular form, as in this case.
Occasionally, MA and lichen amyloidosis may coexist in the same patient, a condition known as biphasic amyloidosis. There are also reports of rare hypochromic and poikilodermatous forms4.
In cases of extensive or nodular cutaneous lesions it is important to carry out a systemic assessment. In the patient studied here this failed to reveal any abnormal results. This type of assessment is important as this condition has been associated with amyloidosis and multiple myeloma10.
Treatment of cutaneous amyloidoses usually yields disappointing results. Most cases can be treated with strong topical corticosteroids, normally for a short period. Calcipotriol or phototherapy are of limited use. 11, 12 Pigmentation has been reported to improve with topical use of dimethyl sulfoxide; however, when treatment was suspended the initial clinical picture reappeared, indicating that this is not an effective treatment for PCA. 13
Encouraging results were reported with the use of Q-switched Nd:YAG laser (1064-532 nm). This produced a significant reduction in pigmentation, which is considered by MA patients to be the most disturbing aspect of the condition. No side effects were observed, and patients tolerated the pain of the laser treatment well. However, as treatment follow-up lasted only two months, possible side effects such as residual hypopigmentation were not identified, particularly in patients with phototypes V or VI, for whom six months follow-up after the multiple laser sessions would be required. Additional studies with histological examinations would also be needed. 14 In the case reported here, treatment consisted of photoprotection and rehydration with clinical follow-up.
This case study describes an uncommon form of MA because of the location and extent of the lesions. It also highlights the need for further studies to elucidate the etiopathogenesis of PCA with a view to finding effective treatment for this type of cutaneous amyloidosis.
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11. Khoo BP, Tay YK, Goh CL. Calcipotriol ointment vs. betamethasone 17-valerate ointment in the treatment of lichen amyloidosis. Int J Dermatol. 1999;38:539-41. [ Links ]
12. Jin AG, Por A, Wee LK, Kai CK, Leok GC. Comparative study of phototherapy (UVB) vs photochemotherapy (PUVA) vs topical steroids in the treatment of primary cutaneous lichen amyloidosis. Photodermal Photoimmunol Photomed. 2001;17:42-3. [ Links ]
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14. Ostovari N, Mohtasham N, Oadras MS, Malekzad F. 532-nm and 1064-nm Q-switched Nd:YAG laser therapy for reduction of pigmentation in macular amyloidosis patches. J Eur Acad Dermatol Venereol. 2008;22:442-6. [ Links ]
Mailing address: Received on 18.11.2010. * Study carried out at the Dona Libânia Dermatology Center, Fortaleza, CE, Brazil.
Nara Frota André
Rua Pedro I, 1.033 Centro
Fortaleza (CE) - Brazil 60035 101
Approved by the Advisory Board and accepted for publication on 21.12.2010.
Conflict of interest: None
Financial funding: None
Received on 18.11.2010.
* Study carried out at the Dona Libânia Dermatology Center, Fortaleza, CE, Brazil.