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Telangiectasia macularis eruptiva perstans

  • Author(s): Chung-Leddon, Jeanie, MD PhD
  • et al.
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Telangiectasia macularis eruptiva perstans
Jeanie Chung-Leddon, M.D., Ph.D.
Dermatology Online Journal 6(1): 6

Department of Dermatology, New York University

PATIENT: 58-year-old man

DURATION: Ten months

DISTRIBUTION: Left upper extremity

HISTORY: The patient presented with a two-week history of erythematous patches on the left upper extremity. He complained of mild pruritus and a burning sensation but denied flushing, gastrointestinal complaints, palpitations, and syncope. He was treated with fluocinolone ointment and hydroxyzine and reported a slight improvement in the symptoms but no change in the appearance of the lesions. His past medical history includes hepatitis B virus infection and a cerebrovascular accident with mild residual weakness. He has been on gabapentin, nifedipine, and aspirin for many years and denies allergies to any medications.


Figure 1Figure 2

PHYSICAL EXAMINATION: On the left upper extremity involving the tricep and bicep regions, there were telangiectases over erythematous patches. No urtication was observed upon rubbing the telangiectatic patches.

LABORATORY DATA: A complete blood count, chemistry profile, liver function tests, rheumatoid factor, antinuclear antibody, erythrocyte sedimentation rate, and rapid plasma reagin test were normal or negative. Hepatitis B antigen and antibody were positive, but hepatitis B core and E antigens were negative.

HISTOPATHOLOGY: There is a sparse, superficial, perivascular and focally interstitial mononuclear-cell infiltrate associated with dilated small blood vessels. A Giemsa stain showed approximately 10 mast cells per high power field. A periodic acid-Schiff stain was negative for fungi.

DIAGNOSIS: Telangiectasia macularis eruptiva perstans

COMMENT: Telangiectasia macularis eruptiva perstans is a rare form of cutaneous mastocytosis and is observed in less than one percent of the patients with mastocytosis.[1,2] It is a disorder affecting adults and has been reported in one child.[3] Clinically, there are tan-to-brown macules and patchy erythema with telangiectases that most often involve the trunk. However, unilateral facial telangiectasia macularis eruptiva perstans has been reported.[4] Most patients only have cutaneous involvement with pruritus of variable degree.[5] Systemic involvement is rare but can include episodic headaches, flushing, gastrointestinal complaints, palpitations, syncope, splenomegaly, increased numbers of mast cells in the bone marrow, and abnormal skeletal radiographs. Telangiectasia macularis eruptiva perstans has not been associated with the development of malignant conditions. It is important to differentiate telangiectasia macularis eruptiva perstans from carcinoid syndrome.

The diagnosis of telangiectasia macularis eruptiva perstans can be confirmed by histologic examination. Mast cells surrounding capillary venules and the superficial venular plexus can be detected with Giemsa, toluidine blue, chloroacetate esterase, and aminocaproate esterase stains.

The primary objective of treatment in telangiectasia macularis eruptiva perstans involves symptomatic relief. H1 receptor antagonists can be helpful for reducing pruritus or flushing. There is a report of temporary treatment of telangiectasia macularis eruptiva perstans with the 585 nm flashlamp-pumped dye laser.[6] It is also important to pretreat with proper H1 and H2 receptor blockers to avoid potential complications from laser-induced mediator release.

References

1. Parkes Weber F, Mellenschmied R. Telangiectasia macularis eruptiva perstans. Br J Dermatol Syphilol 1930;42:374.

2. Cohn MS, Mahon MJ. Telangiectasia macularis eruptiva perstans. JJournal of the American Osteopathic Association, 1994 Mar, 94(3):246-8 PubMed.

3. Sarkany RP; Monk BE; Handfield-Jones SE. Telangiectasia macularis eruptiva perstans: a case report and review of the literature. Clinical and Experimental Dermatology, 1998 Jan, 23(1):38-9 PubMed.

4. Fried SZ, Lynfield YL. Unilateral facial telangiectasia macularis eruptiva perstans. Journal of the American Academy of Dermatology, 1987 Jan, 16(1 Pt 2):250-2 PubMed.

5. Soter NA. The skin in mastocytosis. Journal of Investigative Dermatology, 1991 Mar, 96(3):32S-38S; discussion 38S-39S PubMed.

6. Ellis DL. Treatment of telangiectasia macularis eruptiva perstans with the 585 nm flashlamp-pumped dye laser. Dermatologic Surgery, 1996 Jan, 22(1):33-7 PubMed.

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