Case for diagnosis

Abstracts

Descreve-se um caso de telangiectasia macularis eruptiva perstans em paciente do sexo masculino de 18 anos com história de máculas telangiectásicas disseminadas por tronco e membros. O sinal de Darier foi positivo. O exame histopatológico mostrou infiltrado inflamatório mononuclear, com acúmulo de mastócitos ao Giemsa. A telangiectasia macularis eruptiva perstans deve ser considerada em pacientes que apresentem lesões telangiectásicas de aparência e distribuição atípicas com início na infância.

Mastocitose cutânea; Telangiectasia; Terapia puva

WHAT IS YOUR DIAGNOSIS?

Case for diagnosis

Cristiane Rodrigues de Oliveira^I; Gabriella Corrêa de Albuquerque^II; Eliane Francine Simon^III; Sergio Soares Quinete^IV; Celia Regina S. C. de Carvalho^V

^IResident Physician (R3), Service of Dermatology, Hospital Central, Instituto de Assistência aos Servidores do Estado do Rio de Janeiro (Iaserj) – Rio de Janeiro (RJ), Brazil

^IIResident Physician (R3), Service of Dermatology, Instituto de Assistência aos Servidores do Estado do Rio de Janeiro (Iaserj) – Rio de Janeiro (RJ), Brazil

^IIIHead of the Service of Clinical Pathology, Instituto de Assistência aos Servidores do Estado do Rio de Janeiro (Iaserj) – Rio de Janeiro (RJ). Master in Clinical Pathology, Universidade Federal do Rio de Janeiro (UFRJ) – Rio de Janeiro (RJ), Brazil

^IVHead of the Service of Dermatology, Hospital Central, Instituto de Assistência aos Servidores do Estado do Rio de Janeiro (Iaserj) – Rio de Janeiro (RJ), Brazil

^VHead of the Technical Team, Service of Dermatology, Hospital Central, Instituto de Assistência aos Servidores do Estado do Rio de Janeiro (Iaserj) – Rio de Janeiro (RJ), Brazil

Mailing Address

ABSTRACT

We report a case of telangiectasia macularis eruptiva perstans in an 18-year-old male patient who developed multiple erythematous maculae on the trunk and limbs. Darier’s sign was positive. Histopathology revealed mononuclear cell infiltrate and increased number of mast cells identified with Giemsa staining. Telangiectasia macularis eruptiva perstans should be considered in patients with telangiectatic lesions of atypical appearance or distribution which start in childhood.

Keywords: Mastocytosis, Cutaneous; Telangiectasis; Puva therapy

HISTORY OF THE DISEASE

Male 18-year-old patient Born in Rio de Janeiro, he reported onset of small erythematous spots on the trunk, upper and lower limbs four years before. The lesions were pruriginous, especially when exposed to sun and physical exercise. The dermatological examination showed small erythematous-brownish and telangiectasic spots of irregular borders on the trunk and limbs. Positive Darier sign (Figure 1 and 2). The physical examination did not show hepatosplenomegalia or lymphonodomegalia. Histopathological analysis showed hyperpigmentation of the epidermal basal stratum and presence of perivascular mononuclear inflammatory infiltrate on the upper dermis (Figure 3 A). Giemsa staining showed oval cells with rounded nuclei and numerous metachromatic cytoplasmatic granules (Figure 3B). Complete blood count and biochemistry were normal. Serology for hepatitis and HIV was negative. Abdominal ultrasound and upper digestive endoscopy did not show any abnormality.

   

Therapeutic management comprised antihistaminic drugs and PUVA photochemotherapy. There was partial regression of skin lesions and relief of pruritus with treatment.

COMMENTS

Telangiectasia macularis eruptiva perstans (TMEP) represents a rare form of cutaneous mastocytosis, observed in 1% of the cases. The term refers to the aspect of the cutaneous lesions caused by permanent skin vasodilation, secondary to release of chemical mediators and angiogenic factors by mast cells ¹.

Many stimuli may trigger the activation of mast cells, such as physical agents (cold, heat, solar radiation, exercises); chemical agents (bacterial toxins, immunoglobulin E, bee sting and ethanol); stress and associated drugs, such as morphine, codeine, penicillin, acetyl salicylic acid, amphotericin B, nonsteroidal antiinflammatory drugs, and iodine-based contrast dyes.

TMEP, differently from other forms of cutaneous mastocytosis, affects in special young adults. The clinical presentation is characterized by erythematous-cutaneous telangiectasic spots, with irregular borders and diameter between 2 and 6mm. The regular location of lesions is the trunk and limbs. Pruritus, with exacerbation after heat and skin exposure is frequent, such as in the reported cases. Heat and pressure may produce localized urticaria: Darier sign characteristic of mastocytosis.

Systemic signs are rare and include flushing, palpitation, nausea, vomiting, diarrhea and abdominal pain, whose presence may indicate hematological diseases and systemic mastocytosis. Signs of systemic involvement: hepatosplenomegaly, lymph node enlargement and bone affections ².

In the described case, lesions were disseminated and had started at adolescence, without systemic signs, which indicated good prognosis.

Diagnosis was suggested by clinical presentation and was confirmed by histopathological analysis. Giemsa staining or toluidine blue is essential to reveal the presence of mast cells, through metachromic cytoplasmatic granules ³.

Differential diagnosis includes rosacea, carcinoid syndrome, hepatopathy or collagenosis, hereditary hemorrhagic telangiectasia and essential generalized telangiectasia.

The main aspect in treating mastocytosis is to identify and avoid factors that may stimulate mast cell degradation such as: sun exposure, temperature extremes, alcohol and drugs. H1 antagonists are used to pruritus and urticaria. H2 antagonists are used to control gastric hyperacidity.

PUVA leads to improvement in signs and regression of skin lesions, because it inhibits the release of histamine by mast cells. However, recurrence may occur after a variable interval from end of treatment 4. In the reported case, photochemotherapy was still in course and resulted in partial improvement of skin lesions and pruritus.

The 585nm pulsed laser seems to be effective, according to the report of two cases, in which there was recurrence 14 months after end of treatment. Preoperative care is essential to avoid complications of chemical mediator release. Their action may be secondary to reduction of blood vessels without acting over mast cells ⁵.

We emphasize that in the reported case, mild symptomatology made the patient remain undiagnosed for years, bringing him to the dermatologists only when the cosmetic aspect of the lesions became significant and started to bother him. Similarly to most other patients, the cosmetic affection of skin lesions is the most frequent reason for visiting the specialist, given that symptoms are scarce or absent.

REFERENCES

• 1.  Fernandes MC, Maya TC, Mosquera JAF. Telangiectasia macularis eruptiva perstans: a case report. An Bras Dermatol. 1998;73:51-3.
• 2.  Sarkany RP, Monk BE, Handfield-Jones S. Telangiectasia macularis eruptiva perstans: a case report and review of the literature. Clin Exp Dermatol. 1998;23:38-9.
• 3.  Carvalho MI, Friedmam H, Medeiros BM. Telangiectasia macularis eruptiva perstans: relato de caso. An Bras Dermatol. 2001;76:611-4.
• 4.  Kaatz M, Barta U, Wollina U. Mastocitose cutânea difusa na infância: tratamento bem-sucedido com PUVA. An Bras Dermatol. 2000;75:737-43.
• 5.  Ellis DL. Treatment of telangiectasia macularis eruptiva perstans with the 585-nm flashlamp-pumped dye laser. Dermatol Surg. 1996;22:33-7.

*

Trabalho realizado no Serviço de Dermatologia do Hospital Central do Instituto de Assistência aos Servidores do Estado do Rio de Janeiro (Iaserj) - Rio de Janeiro (RJ), Brasil.

Publication Dates

• Publication in this collection
  23 June 2009
• Date of issue
  Feb 2009

History

• Accepted
  20 Dec 2007
• Received
  18 Nov 2005