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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.78 no.6 Rio de Janeiro Nov./Dec. 2003
Sarcoidosis cicatricial alopecia*
Giane Pereira Giro TeixeiraI; Luciana Vieira de PaulaI; Maria Auxiliadora Jeunon de SousaII; Isabel Cristina Brasil SucciIII
IIHead of Dermapathology at the Hospital Universitário Pedro Ernesto - UERJ
IIICoordinator of Post-Graduate Studies in Dermatology; Head of the Dermatology Service at the Hospital Universitário Pedro Ernesto - UERJ
The authors describe a case of cicatricial alopecia associated with papular lesions on the face and trunk with four years of evolution. The diagnosis of sarcoidosis was confirmed by histopathological examination. During the follow-up the patient developed lung lesions. Cicatricial alopecia is a rare complication of sarcoidosis. It is often confused, in clinical findings, with other dermatoses, among them discoid lupus erythematosus and lichen planopilaris.
Keywords: alopecia; sarcoidosis.
Sarcoidosis is a multisystemic and typically granulomatous disease, whose etiology and pathogenesis are unknown. It may affect any organ, involving the skin in 25 percent of cases.1 The scalp is rarely affected.2 Initially lesions may be nodular or papulous, later coalescing and forming plaques that leave behind an area of cicatricial alopecia. The suspected diagnosis may be facilitated in the presence of other cutaneous and/or systemic lesions of sarcoidosis.
The case of a 42-year old black married female patient, a shopkeeper from Minas Gerais, is reported here. The patient referred to the emergence of an erythemato-squamous lesion on the vertex of the scalp four years earlier, which was pruriginous. It was associated with asymptomatic erythemato-papulous lesions on the face and trunk. A year and six months ago, the scalp lesion progressed into alopecia and new cutaneous lesions appeared. It was at this point that the patient sought assistance at the Dermatology Service of the Hospital Universitário Pedro Ernesto.
The dermatological exam showed patches with erythemato-squamous borders and an atrophied center, with scarce hairs. (Figure 1) Erythemato-papulous lesions were found on the face, the anterior region of the thorax and the back, some of which joined into a ring configuration. (Figure 2) The clinical examination was normal.
Laboratory tests yielded the following results: the hemogram showed no changes; the serum calcium dose (10.1 mg per dl) and urine (83.52 mg daily) were normal; FAN was negative and PPD did not react. Thorax and hand radiography, spirometry and ophthalmology tests showed no changes. The dose of the angiotensin-converting enzyme was normal (2.3 ng per dL).
During the histopathologic test of the cutaneous lesion and scalp, the authors observed numerous epithelioid granulomas on the skin with lymphocitic lack. (Figures 3, 4 and 5) With orcein staining (of the scalp), a noted absence was observed of elastic fibers in the areas in which granulomas was found. Direct immunofluorescence of the lesion was negative.
Treatment was introduced (40 mg daily prednisone for a month), followed by a schedule to withdraw the corticoid the month after and infiltration of triamcinolone on the scalp lesion. There was regression of the cutaneous lesions and improvement of the scaling on the edges of the alopecia plaque, though without repilation.
A year ago, the patient complained of dispenea and was directed to the Pneumology Service. A diffuse nodular infiltrate was observed on the thorax X-ray. Computerized tomography of the thorax showed predominantly peripheral reticulonodular infiltrate. The presence of lymphocitosis on the bronchoalveolar lavage, with BAAR negative in three samples of sputum; and accentuated restrictive ventilatory disturbance in the respiratory function test. Prednisone (80 mg daily) was instituted for three months. Prednisone (20 mg daily) has been used for five months with improvement of the dispenea. Computerized tomography of the thorax was normal.
Cutaneous sarcoidosis may occur in association with a systemic disease.3 In addition to the classical forms described above, a wide variety of uncommon presentations may be found. One such form is alopecic, which is often clinically confused with other dermatoses like discoid erythematous lupus, lichen planopilaris and foliculitis decalvans.4
Sarcoidosis alopecia represents a real cicatricial alopecia. The histopathologic examination showed an absence of elastic fibers.4 There exist only 28 published cases on the disease in the English-language literature, mainly on African-American women with evidence of intrathoracic involvement.3,5,6,9,10 Apart from alopecia plaques, cutaneous involvement is usually seen in other places. Clinically speaking, the areas of alopecia may appear as atrophic areas, whether in association or not with erythema and scaling, plaques, nodules or ulceration.11,12
Patients with lupus erythematosus may have discoid lesions on the scalp, resulting in cicatricial alopecia plaques, with variable atrophy and areas of hyperpigmentation and hypopigmentation. Follicular keratosis may be observed on the lupus alopecia plaques. It is often difficult to establish a diagnosis by histopathology.
Lichen planopilaris usually appears as a plaque of alopecia with individual follicular papules or hyperkeratosis. The condition tends to be progressive, with expansion and coalescence of individual plaques.4 Its clinical and histopathological diagnosis may be mistaken for that of lupus erythematosus.
In folliculitis decalvans, alopecia plaques are found with or without erosion, scaling or associated crusts. Around the lesion, outbreaks of follicular pustules occur. The late stage is characterized by reparative fibrosis.
Among these dermatoses, lupus is without a doubt the most frequent. Its cicatricial alopecia is indistinguishable from sarcoidosis as well as lichen planopilaris and folliculitis decalvans that, while rare, are also more common than sarcoidosis. Distinguishing among these affections becomes more difficult when there is no associated cutaneous lesion.
The histopathologic examination may help in the diagnosis. In the case of sacoidosis, it plays a determining role when granuloma that is not bound to the epithelioid cells is found.
As for treating alopecia, there is little response to (systemic or intralesional) corticosteroids or to chlorochine in related cases.5,7,8,13
The authors therefore emphasize recommending that sarcoidosis, in spite of being rare, be included in the differential diagnosis of cicatricial alopecias.
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Giane Pereira Giro Teixeira
Av. Américo Buaiz 501/205 - Torre Leste - Enseada da Praia do Suá
Vitória ES 29050-420
Tel/Fax: (27) 3345-6712 / 3345-6385
Received in July,
11th of 2000
Approved by the Consultive Council and accepted for publication in March, 10th of 2003
* Work performed at the Dermatology Service of the Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro