OVERVIEW
Benign lymphocytic angiitis and granulomatosis of the lungs (BLAG) was proposed in 1977 by Saldana et al. It is a disease similar to Wegener’s granulomatosis and lymphomatoid granulomatosis with lesions present in the skin and respiratory tracts, and cutaneous lesions presenting mainly as chronic recurrent lipomatous inflammation.
Etiology
The cause of the disease is unknown.
Symptoms
Commonly seen in adults between the ages of 20 and 40. In most typical cases, the damage and course resemble that of an acute pox-like mossy furuncle, differing in larger, fewer and more necrotic tendencies. The primary lesion is a red papule, up to about 1 cm in diameter, which may progress to papular blistering, papular pustular or hemorrhagic, with surface necrosis after several days and weeks. Typical damage may heal spontaneously within 8 weeks. Most tend to be chronic, and without treatment, new damage continues to appear, so that lesions from different periods can coexist. The damage heals with acne-like, hyperpigmented or hypopigmented scarring. Self-executive symptoms are mild. 10% to 20% of patients with lymphomatoid papulosis develop CD30 cutaneous T-cell lymphoma or Hodgkin’s disease.
Examination
Histopathologic examination: histology is diagnostic for this disease. The dermis shows wedge-shaped, patchy, or perivascular infiltrates. Larger damage infiltrates may occupy the entire dermis. The infiltrate may involve the epidermis with inflammatory cells pro-epidermal. Epidermal necrosis and erosion may occur as the damage progresses. Fibrin deposits are seen in the dermal vasculature, and lymphocytic vasculitis is occasionally seen. The dermal infiltrating cells consist of lymphocytes, eosinophilic leukocytes and neutrophils and larger monocytes. Atypical large or small lymphoid cells can be seen, accounting for more than 50% of the infiltrating cells. Histologically the damage can be divided into two types, type A and type B.
Diagnosis
Diagnosis can be made on the basis of clinical manifestations, lesion characteristics, histopathologic features, and immunohistochemical examination.
Differential diagnosis
This disease needs to be differentiated from lymphomatoid granuloma, Wegener’s granulomatosis, etc. The lack of necrotic changes is the main point of differentiation between this disease and Wegener’s granuloma.
Treatment
No treatment is usually required. There is no evidence that treatment prevents the development of secondary lymphoma. However, treatment may be appropriate if the patient is symptomatic and requires treatment and if the side effects of treatment are minimal. For example, high potency corticosteroids; PUVA systemic or localized treatment. Local application of carmustine (carzapine) for a total of 4 to 17 weeks of treatment suppresses damage without myelosuppression. Methotrexate (MTX), which results in significant symptomatic improvement in 90% of patients.
Prognosis.
Typical damage may heal spontaneously within 8 weeks. Pox-like, hyperpigmented, or hypopigmented scarring remains after healing of the damage.