Archive

Autumn 2017, Vol. 25 No. 3

Hong Kong J. Dermatol. Venereol. (2017) 25, 133-136

Case Report

Telangiectasia macularis multiplex acquisita

後天性多發性血管擴張與斑點

GH Na 羅佳慧, SH Noh 盧秀弦, K Park 朴建, EJ Kim 金垠貞

Abstract

Telangiectasia macularis multiplex acquisita (TMMA) is a rarely documented disease entity that usually occurs in the middle-aged men. A 68-year-old man presented with asymptomatic erythematous-brownish macules with telangiectasia on the upper arms, anterior chest, shoulders, and upper back. He had hypertension, diabetes, and hepatitis B infection for several years. Laboratory findings revealed elevation of liver enzymes, but autoimmune markers and sex hormone levels were normal. Biopsy specimen revealed perivascular lymphocytic infiltration. He was diagnosed as TMMA with hepatitis B infection.

後天性多發性血管擴張及斑點（Telangiectasia macularis multiplex acquisita）是比較罕見的疾病，主要發生在中年男性身上。68歲男性因為出現帶有紅色、褐色的斑點和多發性血管擴張而訪問了醫院。皮膚病變主要出現在兩側上臂、前胸、兩側肩膀以及上身等。患者有高血壓、糖尿以及B型肝炎。血液檢查結果肝指數上升，但自體免疫標記或性荷爾蒙指數屬於正常。活檢結果血管周圍有輕微的淋巴細胞（lymphocyte）浸潤。以上述內容為基礎，診斷為後天性多發性血管擴張及斑點（Telangiectasia macularis multiplex acquisita）。

Keywords: Telangiectasia macularis multiplex acquisita

關鍵詞: 後天性多發性血管擴張及斑點

Introduction

Telangiectasia macularis multiplex acquisita (TMMA) is a rarely documented disease entity with distinctive clinical manifestations.¹ The clinical criteria for diagnosis of TMMA include: (1) crops of telangiectasia superimposed on erythematous macules symmetrically on bilateral upper arms that may extend to the forearms, shoulders, V-area of the anterior chest, back or thighs; (2) no mucosal or systemic involvement; (3) not associated with autoimmune diseases, such as lupus erythematosus, dermatomyositis or systemic scleroderma that may induce cutaneous telangiectasia; and (4) no ataxia or unsteady gait.

Here, we report a case of TMMA occurring in a patient with chronic hepatitis B infection.

Case report

A 68-year-old man presented with a 5-year history of multiple erythematous-brownish macules with telangiectasia on the upper arms, anterior chest, shoulders, and upper back (Figure 1). He denied any history of excessive sun exposure. He had hypertension and diabetes mellitus for 10 years. The patient also had hepatitis B viral infection for 15 years, but had not received any treatment for this condition. On physical examination, palmar erythema was observed. Histopathological examination from the upper chest revealed solar elastosis and mild perivascular lymphocytic infiltration in the upper dermis (Figures 2a & 2b). Toluidine blue staining to identify mast cell infiltration did not demonstrate any specific findings (Figure 2c). Laboratory tests for autoimmune disease and sex hormone levels were within the normal range, but aspartate transaminase (95 IU/L, normal: 5-35 IU/L) and alanine transaminase (81 IU/L, normal: 5-40 IU/L) levels were elevated. Additionally, the patient was positive for HBsAg and HBeAg. These findings were not consistent with other conventional disease entities. As the patient fulfilled the clinical criteria of TMMA, he was diagnosed as TMMA accompanied by hepatitis B infection. We tried to monitor him regularly without specific treatment, but he subsequently defaulted further follow-up.

Figure 1 Clinical appearance at presentation. Multiple telangiectases superimposed on erythematous macules on the anterior chest, bilateral arms, shoulder, and back. (a) Front; (b) Back.

Figure 2 Histopathological findings. (a) Perivascular inflammatory cell infiltrate in the upper dermis (haematoxylin-eosin x40). (b) Lymphocytic infiltration in the upper dermis without significant telangiectasia (haematoxylin-eosin x100). (c) No mast cell infiltration in the lesion (toluidine blue stain x40).

Discussion

TMMA is rarely addressed in the English literature. It is mostly reported in Asians, primarily in Chinese, which may be due to the ethnic predilection to Asian populations.¹ The age of onset is variable, but mostly occurs in middle-aged men between the third to sixth decades.¹ TMMA typically presents with multiple telangiectasia superimposed on erythematous macules in a V-shape on the anterior chest, both arms, shoulders, and back.^1,2 The differential diagnosis of TMMA includes poikiloderma, telangiectasia macularis eruptive perstans (TMEP), acquired brachial cutaneous dyschromatosis, and acquired bilateral telangiectatic macules. Poikiloderma is a descriptive term referring to a combination of cutaneous atrophy, telangiectasia, and macular pigmentary changes that result in a mottled skin appearance.³ Solar radiation has been proposed as the main cause.³ However, TMMA presents with widespread skin lesions on both sun-exposed and non-exposed areas. Also, TMMA does not involve the face. Telangiectasia macularis eruptiva perstans is a variant of cutaneous mastocytosis, which shows mast cell infiltration in the papillary dermis.⁴However, TMMA lesions show no significant difference the mast cell count. Acquired brachial cutaneous dyschromatosis is a pigmentary disorder that shows gray-brown patches with geographic borders, occasionally interspersed with hypopigmented macule.⁵ It mostly involves the dorsal aspect of forearms and usually affects the post-menopausal women.⁵ Acquired bilateral telangiectatic macules is a newly described disorder in Korea.⁶ Unlike TMMA, skin lesions of this entity are primarily confined to the upper arms.⁶ Additionally, in contrast to these differential diagnoses, TMMA is thought to be associated with systemic diseases, including hepatitis, diabetes, or cardiovascular diseases.¹ Chang et al studied 25 Chinese patients with TMMA to determine disease associations.¹ They found that patients with TMMA had a much higher prevalence rate of dyslipidaemia (64.7%), viral hepatitis (56.5%), hypertension (50%), and diabetes (38.9%) than the general population.¹ Similarly, Wang et al reported two cases of TMMA with concomitant hepatitis B infection and inferred that TMMA might belong to the spectrum of vascular changes in liver disease. Chronic liver disease presents with various cutaneous manifestations such as palmar erythema, spider angioma, and arteriovenous hemangioma. Several mechanisms have been proposed to explain these vascular changes in liver disease, including dysfunction of arteriovenous anastomoses, portal hypertension, elevated plasma vascular endothelial growth factor, and an altered serum estrogen level.⁷ Based on these literature, we speculated that TMMA might be another cutaneous manifestation of liver dysfunction. The treatment of TMMA is still unknown. For cosmetic reason, dye laser or intense pulsed light can be chosen as treatment of TMMA in view of its effect on telangiectasia.^2,6

Herein, we report a case of telangiectasia macularis multiplex acquisita accompanied with hepatitis B infection. To our knowledge, this is the first case report in a Korean patient. In fact, this disorder is not rare. We thought that TMMA should be preferentially considered in patients with hepatic dysfunction or metabolic disease who present with telangiectasia superimposed on erythematous macules.

References

1. Chang CH, Lu PH, Kuo CJ, Yang CH. Telangiectasia macularis multiplex acquisita: a new entity in Chinese populations and an analysis of associated factors. Int J Dermatol 2013;52:426-31.

2. Wang G, Chen H, Yang Y, Wu K, Sun J. Telangiectasia macularis multiplex acquisita accompanied by hepatitis B infection. Australas J Dermatol 2017;58:e5-7.

3. Nofal A, Salah E. Acquired poikiloderma: proposed classification and diagnostic approach. J Am Acad Dermatol 2013;69:e129-40.

4. Tharp MD. Mastocytosis. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's dermatology in general medicine. 8th ed. New York:McGraw-Hill 2012, pp.1809-18.

5. Rongioletti F, Rebora A. Acquired brachial cutaneous dyschromatosis: A common pigmentary disorder of the arm in middle-aged women. J Am Acad Dermatol 2000;42:680-4.

6. Park JH, Lee DJ, Lee YJ, Jang YH, Kang HY, Kim YC. Acquired bilateral telangiectatic macules: a distinct clinical entity. JAMA Dermatol 2014;150:974-7.

7. Leonardo G, Arpaia MR, Del Guercia R, Coltorti M. Local deterioration of the cutaneous venoarterial reflex of the hand in cirrhosis. Scand J Gastroenterol 1992;27:326-32.