Folliculitis decalvans and orofacial granulomatosis

Folliculitis decalvans (FD) is a rare and chronic inflammatory dermatosis which pathogenesis remains unclear. It is classified as primary neutrophilic cicatricial alopecia presented clinically with follicular pustules and papules, hemorrhagic crusts, erosions and scarring within scalp. Orofacial granulomatosis (OFG) is a recurrent or persistent orofacial swelling histopathologically presented with non-caseating granulomas. The etiology of the disease is not fully understood. There are hypotheses concerning pathogenesis of the disease including genetic, allergic, infectious and immunological factors. We report a case of a patient with FD and concomitant orofacial granulomatosis successfully treated with dapsone.
A 32-year-old man was admitted to the Department of Dermatology to diagnose follicular pustules, erosions, yellow scales, crusts with scarred areas and focal hair loss within the scalp, especially within vertex and accompanying persistent lip swelling with normal appearance of the tongue and no oral lesions (Figure 1). Moreover, we observed erythrosquamous lesions and papules on the lateral surfaces of the trunk, within groins and armpits. The lesions on the scalp occurred in childhood and the lip swelling persisted for 3 years. Except skin eruptions the patient did not report any other complaints. He was initially treated in another clinic for seborrheic dermatitis and acne inversa topically with antibiotics, glucocorticosteroids, tacrolimus and orally with antibiotics (doxycycline), antihistamines with temporary improvement. The patient suffered from post-traumatic epilepsy and was treated with carbamazepine. He had a craniotomy due to head injury (concussions and subdural hematoma of the left temporo-parietal brain region, basilar skull fracture and squamous part of the right temporal bone fracture) during the traffic accident 20 years earlier. During the hospitalization routine laboratory tests showed an elevated C-reactive protein level and erythrocyturia which occurred to be irrelevant (the patient was consulted by the urologist). Swabs from erosions and pustules were collected and two types of bacteria were cultured – Staphylococcus aureus and Streptococcus agalactiae. To exclude concomitant systemic diseases, we performed additional examinations. There were no abnormalities in abdominal ultrasonography or chest X-ray. Antinuclear antibodies, lupus anticoagulant, cardiolipin antibodies (IgG and IgM), 2-glycoprotein 1 antibodies (IgG...


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