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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.86 no.3 Rio de Janeiro May/June 2011
WHAT IS YOUR DIAGNOSIS?
Case for diagnosis*
Bárbara Nader VasconcelosI; João Carlos Macedo FonsecaII; Daniel Lago ObadiaIII
Professor of Dermatology, Pedro Ernesto Teaching Hospital, State University of
Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil
IIAdjunct Professor, School of Medical Sciences, State University of Rio de Janeiro (UERJ). Doctorate in Dermatology from the Federal University of Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil
IIISubstitute Professor of Dermatology / Dermatopathology, State University of Rio de Janeiro (UERJ) and Central Hospital of the Armed Forces, Rio de Janeiro, RJ, Brazil
Hidradenitis suppurativa is a chronic inflammation of the apocrine glands. It usually starts at or soon after puberty, and women are more often affected than men. The most affected sites are the axillae; however, other regions rich in apocrine glands may also be affected. Involvement restricted to the inframammary fold alone is rare and to the best of our knowledge there have been no reports of any similar cases. The etiology of this condition has yet to be fully clarified.
Keywords: Breast, Hidradenitis suppurativa, Sulfamethoxazole
A 13-year old female patient consulted at the Dermatology Department because of recurrent, very painful, fistulizing lesions that had first appeared three years previously in the inframammary region. At physical examination, she presented erythematous, painful papules and nodules, some of which were discharging a purulent secretion. There were also comedones and various types of scars, including erythematous, hypochromic-atrophic and hypertrophic scars, located in the inframammary region (Figures 1 and 2). Histopathology showed a dilated and ruptured follicular infundibulum (Figure 3) surrounded by inflammatory infiltrate consisting of lymphocytes, neutrophils and plasmocytes. In the reticular dermis, areas with fibrosis and vascular proliferation were found.
Hydradenitis is a chronic, suppurative inflammation of the apocrine glands. It is more common in women and begins at or following puberty. 1 The most commonly affected sites are the axillae; however, other regions rich in apocrine glands may be affected such as the perianal region, the breast areolae and external genitalia. 2 It is rare for the condition to affect the inframammary region alone and to the best of our knowledge, there have been no reports of other similar cases published in the past ten years. Its etiology is yet to be fully clarified. 3 Hydradenitis suppurativa (HS) is one of the diseases that compile the follicular occlusion tetrad together with acne conglobata, perifolliculitis capitis abscedens et suffodiens and pilonidal cyst, a group of clinically distinct diseases, albeit with similar histopathological findings. Onset of the disease occurs with suppurative infundibulitis and follicular rupture, followed by mixed superficial and deep inflammatory infiltration caused by neutrophils, lymphocytes and plasmocytes, and the formation of suppurative granulomas. Varying amounts of granulation tissue and shafts of hair are found in the dermis. Later, fibrosis and fistulae become prominent. 4 The inflammation of the apocrine glands, once considered to be the basic pathological process, is actually a secondary event resulting from the follicular involvement.
Clinically, the condition is characterized by the presence of painful, recurring, inflammatory papules and nodules with acute or chronic evolution that lead to the formation of fistulae and adherences.
In addition, hydradenitis suppurativa severely impairs patients' quality of life. Pain, pruritus and local sensitivity are the principal complaints and, statistically, this condition may exert a stronger negative effect on patients' quality of life than psoriasis, atopic dermatitis, urticaria or neurofibromatosis,5 making this a very significant disease in dermatology.
Various forms of treatment may be considered depending on the extent and severity of the condition. In acute cases, treatment should include local antiseptics and topical and systemic antibiotics, and in the case of a floating nodule, surgical drainage is indicated. In patients with the chronic form of the disease associated with fibrosis and formation of fistulae, treatment may require surgical intervention. In some cases in which control of the condition has proven difficult, oral isotretinoin may be used and, more recently, inhibitors of tumor necrosis factor-alpha production have been used in severe cases; however, results have not always been satisfactory. 6 Case reports and series of cases have suggested that infliximab may be an effective treatment for HS, although the drug has yet to be approved for this purpose. 6-10
In the case reported here, the patient is female and the condition began at puberty and was confined exclusively to the inframammary region. The treatment proposed was the systemic use of sulfamethoxazole-trimethoprim with a maintenance dose and gradual withdrawal, in addition to topical antibiotics. Up to the present time, the patient has responded well to therapy.
1. McMichael A, Guzman Sanchez D, Kelly P. Folliculitis and the follicular occlusion tetrad. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. 2nd ed. St. Louis: Mosby Elsevier; 2008. p.39,517-530. [ Links ]
2. Iglesias N. Hidroses. In: Sampaio SAP, Rivitti EA. Dermatologia. 3. ed. São Paulo: Artes Médicas; 2007. p. 30,403-417. [ Links ]
3. Hay RJ, Adriaans BM. Bacterial infections. In: Rook's textbook of dermatology. Oxford: Blackwell Publishing; 2004.p.27.82-84. [ Links ]
4. Ackerman AB, Böer A, Bennin B, Gottlieb GJ. Histologic Diagnosis of Inflammatory Skin Diseases. An Algorithmic Method Based on Pattern Analysis. 2nd ed. New York: Ardor Scribendi; 1997. p.174-5. (Cap. 7, Hidradenitis suppurativa). [ Links ]
5. Revus J. Hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2009;23:985-98. [ Links ]
6. Obadia DL, Daxbacher ELR, Jeunon T, Gripp AC. Hidradenite supurativa tratada com infliximabe. An Bras Dermatol. 2009;84:695-7. [ Links ]
7. Fardet L, Dupuy A, Kerob D, Levy A, Allez M, Begon E, et al. Infliximab for severe hidradenitis suppurativa: transient clinical efficacy in 7 consecutive patients. J Am Acad Dermatol. 2007;56:624-8. [ Links ]
8. Adams DR, Gordon KB, Devenyi AG, Ioffreda MD. Severe hidradenitis suppurativa treated with infliximab infusion. Arch Dermatol. 2003;139:1540-2. [ Links ]
9. Fernández-Vozmediano JM, Armario-Hita JC. Infliximab for the treatment of hidradenitis suppurativa. Dermatology. 2007;215:41-4. [ Links ]
10. Thielen AM, Barde C, Saurat JH. Long-term infliximab for severe hidradenitis suppurativa. Br J Dermatol. 2006;155:1105-7. [ Links ]
Mailing address: Approved
by the Editorial Board and accepted for publication on 06.06.2010. *
Study conducted at the Pedro Ernesto Teaching Hospital, State University of Rio
de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
Bárbara Nader Vasconcelos
Rua Marquês de Abrantes 178, apto 180 Flamengo
Rio de Janeiro - RJ. CEP 22.230-060
Conflict of interest: None
Financial funding: None
by the Editorial Board and accepted for publication on 06.06.2010.
* Study conducted at the Pedro Ernesto Teaching Hospital, State University of Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.