Hidradenitis Suppurativa is a Diagnosis to be Discussed

One of the values of case reports is that they foster communication between the involved parties. The cases included in this mini-review all underline different aspects of communication: between peers but also between physicians and patients. Few things, if any, can equal the concrete occasion to discuss empirical data. Among physicians, communication can not only create awareness but just as importantly provide solutions or suggest perspective to problems, while communication with patients is at the very core of medicine. These aspects are demonstrated in four cases dealing with the disease hidradenitis suppurativa

Incomplete Schnitzler Syndrome

Schnitzler syndrome (SS) is a rare autoinflammatory disease that presents with chronic urticaria and monoclonal immunoglobulin (Ig) M or G, accompanied by fever, abnormal bone remodeling, skin biopsy with a neutrophilic dermal infiltrate, leukocytosis, or elevated C-reactive protein. It is usually refractory to antihistamines and immunosuppression. We present a case report of clinical SS without monoclonal Ig with robust response to interleukin-1 inhibitor anakinra. This suggests the possible existence of an incomplete form of SS and underlines the risk of false negative diagnosis in individuals with such “incomplete SS”

Incomplete Schnitzler Syndrome

Schnitzler syndrome (SS) is a rare autoinflammatory disease that presents with chronic urticaria and monoclonal immunoglobulin (Ig) M or G, accompanied by fever, abnormal bone remodeling, skin biopsy with a neutrophilic dermal infiltrate, leukocytosis, or elevated C-reactive protein. It is usually refractory to antihistamines and immunosuppression. We present a case report of clinical SS without monoclonal Ig with robust response to interleukin-1 inhibitor anakinra. This suggests the possible existence of an incomplete form of SS and underlines the risk of false negative diagnosis in individuals with such “incomplete SS”

Tumour necrosis factor-alpha and matrix metalloproteinase-2 are expressed strongly in hidradenitis suppurativa

Hidradenitis suppurativa is a chronic skin condition, charac terized clinically by painful, recurrent, deep-seated nodules and suppuration, and histologically by hyper trophic scarring of apocrine gland bearing skin and sinus tracts. The overall consequence of the disease is considerable tissue remodelling and the underlying alterations in innate immunity are poorly understood. The aim of this study was to evaluate the expression of human beta-defensin 2, tumour necrosis factor (TNF)-α and matrix metalloproteinase-2 in skin lesions of patients with hidradenitis suppurativa. A total of 14 skin samples from patients and 2 skin samples from healthy volunteers were evaluated by immunohistochemistry. Human beta-defensin 2 was negative in 12/14 specimens. Elevated expression of metalloproteinase-2 was observed in keratinocytes, fibroblasts and inflammatory cells in dermis, sweat glands, hair follicles and sinus tracts, suggesting a key role for hidradenitis suppurativa pathoge-nesis. Decreased human beta-defensin 2 in the presence of inflammatory (TNF-α-containing) cells suggests a decreased innate immunity in hidradenitis suppurativa-affected skin.publishersversionPeer reviewe

Mammary Hidradenitis Suppurativa Lesions – A Suggestion for Phenotyping

Background: Hidradenitis suppurativa (HS) is an under-diagnosed chronic inflammatory skin disease of the pilosebaceous unit of apocrine gland-rich parts of the body. The mammary area is the fourth most HS-affected area and, as typical lesions include non-fluctuating nodules, abscesses, and tunnels/sinus tracts, mammary HS is often mistaken for other mammary “boils”, such as sub-areolar and granulomatous non-lactating breast abscesses. Our objective was to present a spectrum of mammary HS lesions, explore a possible classification, and expose mammary HS as a possible differential diagnosis to non-lactational breast abscesses. Methods: A cross-sectional study on current and newly-referred patients treated for HS affecting the mammary area. Anamnestic information, subjective outcome measures, and lesion counts including anatomical location were collected. Patients with similar morphologies were grouped, and characteristics for the groups were investigated. Limitations: We were not aware of the number of morphologies we would find, and as a result the study did not have sufficient power to show significant differences after correction for multiple testing. Results: We found three morphologically different subtypes of mammary HS; the Sternal, the Frictional, and the Nodule types. These groups differed in anatomical lesion characteristics and other patient characteristics. Furthermore, we found a fourth Mixed type – a combination of the other three. Conclusion: Differential diagnosis between mammary HS and sub-areolar or granulomatous non-fluctuating non-lactating breast abscess is most easily performed by assessing the precise anatomical location of the lesion and determining if the mammary lesion is the only lesion present or if similar lesions exist in other HS-specific ar

Mammary Hidradenitis Suppurativa Lesions – A Suggestion for Phenotyping

Background: Hidradenitis suppurativa (HS) is an under-diagnosed chronic inflammatory skin disease of the pilosebaceous unit of apocrine gland-rich parts of the body. The mammary area is the fourth most HS-affected area and, as typical lesions include non-fluctuating nodules, abscesses, and tunnels/sinus tracts, mammary HS is often mistaken for other mammary “boils”, such as sub-areolar and granulomatous non-lactating breast abscesses. Our objective was to present a spectrum of mammary HS lesions, explore a possible classification, and expose mammary HS as a possible differential diagnosis to non-lactational breast abscesses. Methods: A cross-sectional study on current and newly-referred patients treated for HS affecting the mammary area. Anamnestic information, subjective outcome measures, and lesion counts including anatomical location were collected. Patients with similar morphologies were grouped, and characteristics for the groups were investigated. Limitations: We were not aware of the number of morphologies we would find, and as a result the study did not have sufficient power to show significant differences after correction for multiple testing. Results: We found three morphologically different subtypes of mammary HS; the Sternal, the Frictional, and the Nodule types. These groups differed in anatomical lesion characteristics and other patient characteristics. Furthermore, we found a fourth Mixed type – a combination of the other three. Conclusion: Differential diagnosis between mammary HS and sub-areolar or granulomatous non-fluctuating non-lactating breast abscess is most easily performed by assessing the precise anatomical location of the lesion and determining if the mammary lesion is the only lesion present or if similar lesions exist in other HS-specific ar