Ann Dermatol Vener
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Factitious disorders constitute a complex pathology for the dermatologist. Although a diagnosis is often indicated, it is difficult to confirm and treatment is complicated. Dermatitis artefacta is the somatic expression of an often serious psychiatric disorder consciously created by patients on their own cutaneous-mucosal surfaces but the motivation is unconscious and no secondary benefits are sought (in contrast to simulation). Pathomimicry represent a specific entity: the provocation of outbreaks of a known disease, triggered by voluntary exposure to a causative agent. Herein we report on a case of pathomimicry in a context of hidradenitis suppurativa. ⋯ Several cases of dermatological pathomimicry (sustainment by the patient of an ulcer with a known cause, contact with an allergen found in eczema, or renewed use of a medication implicated in toxiderma) or systemic disease (insulin injection in a diabetic patient) have been reported. To the best of our knowledge, this is the first description of pathomimicry associated with hidradenitis suppurativa. Regarding therapy, aftercare should be multidisciplinary. Confessions should not be forced and confrontations, which risk serious psychiatric collapse, should be avoided. A reassuring attitude enables psychiatry to be applied once trust has been sustainably established, hence the crucial role of the dermatologist.
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Antisynthetase antibodies (ASA) are directed against aminoacyl-tRNA-synthetases, ubiquitous enzymes of which eight types have hitherto been described. They are seen primarily in antisynthetase syndrome (ASS), in which diffuse interstitial lung disease is associated with inflammatory myopathy, joint involvement and cutaneous signs, in particular mechanic's hands. The aim of this study was to determine the prevalence and semiological characteristics of cutaneous involvement in patients presenting ASA. ⋯ Mechanic's hands are a key indicator in cases of ASA and its outcome is intimately linked with underlying systemic involvement, particularly pulmonary. The characteristic semiology enables this disorder to be recognised and allows differentiation from psoriasis or irritant contact dermatitis of the hands, and it does not vary according to antibody. Whether or not the disease is life-threatening is unaffected by the presence of this sign.
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Isotretinoin is the most potent treatment for acne but paradoxical flares can occur. HS lesions of the face may be mistaken for acne. We report on 4 patients in whom an "acne" flare on isotretinoin led to the correct diagnosis of HS. ⋯ An acne flare on isotretinoin requires investigations with a view to potential diagnosis of HS. Patients presenting "acne" and atypical features such as involvement of the neck, large U scars and cord-like structures should be questioned about the presence of nodules in the axillae and groin since patients with mild HS may not spontaneously acknowledge such typical symptoms.
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Case Reports
[Acquired C1 esterase inhibitor deficiency via bradykinin-mediated angioedema: Four cases].
Acquired C1-esterase inhibitor (C1-INH) deficiency angioedema (C1-INH-AAE) is a form of bradykinin-mediated angioedema. This rare disorder is due to acquired consumption of C1-INH, hyperactivation of the classic pathway of human complement, and potentially fatal recurrent angioedema symptoms. Clinical symptoms of C1-INH-AAE are very similar to those of hereditary angioedema (HAE) but usually appear after the fourth decade of life and induce abdominal pain less frequently. Laboratory tests are essential in establishing the diagnosis with low levels or abnormal structure and function of C1-INH. Most patients present C1-INH autoantibodies. Furthermore, C1q is reduced in AAE, contrary to HAE. The long-term prognosis is determined by associated hematologic malignancies. ⋯ C1-INH-AAE induced by ACE inhibitors or ARBs may be associated with hematologic malignancies. This form of revelation does not necessarily indicate a diagnosis of ACE or ARBs angioedema, and screening should therefore be performed for C1 Inh and C1q. An underlying hematologic malignancy should be routinely sought and the long-term prognosis determined.