Acquired generalized anhidrosis: review of the literature and report of a case with lymphocytic hidradenitis and sialadenitis successfully treated with cyclosporine.

We report a case of acquired generalized anhidrosis successfully treated with cyclosporine. A skin biopsy showed T cell infiltration around the sweat glands and labial biopsy revealed lymphoplasmacytic infiltration around the minor salivary gland, suggesting an underlying autoimmune disease such as Sjögren's syndrome. Administration of cyclosporine markedly improved the patient's condition and sympathetic skin response; thus cyclosporine may be effective for treating anhidrosis in patients with autoimmune disorders.

Neutrophilic eccrine hidradenitis: a retrospective study.

BACKGROUND: Neutrophilic eccrine hidradenitis (NEH) is a benign neutrophilic dermatosis not well recognized beyond an association with malignancy. Although the disease is commonly reported in adults with malignancy, this association is uncommon in children. The diagnosis of NEH is predominantly based on histologic findings to exclude alternative diagnoses for adults, but biopsy is not usually required for children. METHODS: A retrospective study was performed of adult and pediatric patients diagnosed with NEH at three Mayo Clinic sites from January 1, 1992, to January 1, 2022. The aim of this study was to elucidate risk factors for NEH and its clinical characteristics, treatment options, and natural course. Clinical information and pathologic results were collected from health records. Available pathologic slides were reviewed with a dermatopathologist. RESULTS: Of 47 patients identified, 33 had either histologic or clinical confirmation of the diagnosis; 21 were adults (64%), and 12 were children (36%). Most adults (16/21; 76%) had underlying malignancy and received chemotherapy. Five adults (24%) were classified as having idiopathic NEH, and they were younger and had higher NEH recurrence rates than the other adults. Only one pediatric patient (8%) had underlying malignancy. For 10 children (83%), NEH was preceded by strenuous activity. Initial findings of idiopathic NEH were palmoplantar eruptions for both adult and pediatric patients, whereas malignancy-associated NEH commonly involved the face and axillae. CONCLUSIONS: Among adults, NEH is commonly associated with malignancy and chemotherapy. Among children, idiopathic NEH occurs primarily after overexertion, and malignancy is highly unlikely to be the cause of NEH.

Infectious eccrine hidradenitis: sweat glands as the portal of entry for cellulitis.

Neutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis involving the eccrine glands. It is commonly associated with haematological malignancy and administration of chemotherapy. An infective aetiology for NEH is termed infectious eccrine hidradenitis (IEH). Pathogens that have been associated with IEH include Nocardia, Serratia, Enterobacter sp., Staphylococcus aureus and Mycobacterium chelonae We describe a case of IEH in a patient with prolonged use of a compression sleeve for their upper limb lymphoedema. The histopathological findings of NEH and IEH are almost identical. Skin tissue culture and rapid clinical improvement with antibiotic therapy are keys in delineating the two subtypes.

Neutrophilic dermatoses: Kids are not just little people.

Neutrophilic dermatoses are a group of inflammatory skin disorders characterized by an overactive innate immune system with dysregulation of neutrophils without underlying infectious etiology. The major representative conditions discussed are Sweet syndrome; pyoderma gangrenosum; neutrophilic eccrine hidradenitis; palmoplantar eccrine hidradenitis; subcorneal pustular dermatoses; bowel-associated dermatosis arthritis syndrome; and synovitis, acne, pustulosis, hyperostosis, and osteitis. We will also discuss other neutrophilic conditions present almost exclusively in the pediatric population, including congenital erosive and vesicular dermatosis with reticulated supple scarring and the recently described group of autoinflammatory diseases. The clinical characteristics, diagnostic approach, and treatment management in the pediatric and adult population are discussed.

A case of infectious eccrine hidradenitis.

We report a case of "infectious" neutrophilic eccrine hidradenitis who developed papules on the upper arm and trunk. Histological findings revealed vacuolar degeneration and necrosis of epithelial cells in the eccrine sweat ducts and neutrophils that had migrated through ductal epithelium to the lumen. A microabscess was also seen in the eccrine sweat gland coil. Our patient had received no chemotherapy such as cytarabine. Gram-positive cocci were present in the lesional eccrine duct indicating an infective origin of the disease. Human beta defensin-2, one of epithelial antimicrobial peptides, was present in the lesional epidermis and eccrine duct.

[Adult idiopathic neutrophilic eccrine hidradenitis treated with colchicine]. / Hidradénite eccrine neutrophilique idiopathique de l'adulte d'évolution prolongée traitée par colchicine.

INTRODUCTION: Neutrophilic eccrine hidradenitis (NEH) is a rare disease belonging to the group of neutrophilic dermatoses. It has a characteristic histopathologic pattern, with necrosis of the eccrine glands and a local neutrophilic infiltrate. It occurs mostly in patients receiving chemotherapy for acute myeloblastic leukemia or, less frequently, another malignancy. Its occurrence in healthy patients is rare. CASE REPORT: We report the case of a 56-year-old woman with no remarkable medical history who developed an erythematous papular facial eruption. Skin biopsy showed typical features of neutrophilic eccrine hidradenitis including neutrophilic infiltrate. No cause was found. The patient was successfully treated with colchicine. DISCUSSION: This case is particular because NEH was not associated with malignant hematologic disease, solid cancer, chemotherapy, fever or any other disease, after a follow-up of 22 months. Such idiopathic NEH has been reported only rarely in adults. The second particularity is the length of the eruption, which required colchicine treatment. Clinical improvement occurred within 1 month. Because hematologic malignancies can in some cases be preceded by neutrophilic dermatitis, clinical follow-up is recommended in adults.

Hydradenitis suppurativa and inflammatory bowel disease: An unusual, but existing association.

Inflammatory bowel disease (IBD) could be associated with several extra-intestinal manifestations (EIMs) involving musculoskeletal, hepatopancreatobiliary, ocular, renal, and pulmonary systems, as well as the skin. In the last years, hidradenitis suppurativa (HS) is acquiring an increasing interest. IBD, especially Crohn's disease (CD), is among the most reported associated diseases in HS patients. The aim of this paper is to give a brief overview of data showing a possible epidemiologic and pathogenetic association between IBD and HS. We performed a pooled-data analysis of four studies and pooled prevalence of HS in IBD patients was 12.8%, with a 95%CI of 11.7%-13.9%. HS was present in 17.3% of subjects with CD (95%CI: 15.5%-19.1%) and in 8.5% of UC patients (95%CI: 7.0%-9.9%). Some items, especially altered immune imbalance, are generally involved in IBD pathogenesis as well as invoked by HS. Smoking is one of the most relevant risk factors for both disorders, representing a predictor of their severity, despite, actually, there being a lack of studies analyzing a possible shared pathway. A role for inheritance in HS and CD pathogenesis has been supposed. Despite a genetic susceptibility having been demonstrated for both diseases, further studies are needed to investigate a genetic mutual route. Although the pathogenesis of IBD and HS is generally linked to alterations of the immune response, recent findings suggest a role for intestinal and skin microbiota, respectively. In detail, the frequent finding of Staphylococcus aureus and coagulase-negative staphylococci on HS cutaneous lesions suggests a bacterial involvement in disease pathogenesis. Moreover, microflora varies in the different cutaneous regions of the body and, consequently, two different profiles of HS patients have been identified on these bases. On the other hand, it is well-known that intestinal microbiota may be considered as "the explosive mixture" at the origin of IBD despite the exact relationship having not been completely clarified yet. A better comprehension of the role that some bacterial species play in the IBD pathogenesis may be essential to develop appropriate management strategies in the near future. A final point is represented by some similarities in the therapeutic management of HS and IBD, since they may be controlled by immunomodulatory drugs. In conclusion, an unregulated inflammation may cause the lesions typical of both HS and IBD, particularly when they coexist. However, this is still a largely unexplored field.

[Axillary hidrosadenitis due to Leishmania: Case report and literature review]. / Hidrosadenitis axilar por Leishmania: presentación de un caso y revisión de la bibliografía.

BACKGROUND: Hidradenitis is a disorder where abscesses appear after the infection of the apocrine sweat glands. It is located normally in the axillae, groin, perineal region, and the scalp. CLINICAL CASE: A 37 year old male was referred by his GP to the General Surgery Department with axillary hidradenitis which had evolved over the years. The physical examination shows signs of hidradenitis in both axillae, with a noticeable suppurative hidradenitis in the right armpit. En bloc extirpation was performed to remove the whole affected area. The pathological examination revealed a cutaneous leishmaniasis. Subsequently, fucidin was administered topically, as well as local infiltrations of one millilitre of Glucantime™. DISCUSSION: Hidradenitis normally appears in intertriginous areas and its manifestation is accompanied by recurrent subcutaneous nodules. The incidence rate in females is three times higher than in males. The isolated Hidradenitis caused by Leishmania is a rare condition presented only in endemic areas or in immunocompromised patients, such as HIV-infected patients. Clinical manifestations can be different and the diagnosis can be confirmed through haematoxylin-eosin. The main pattern displays a disorganised granuloma without necrosis. Systemic or topical treatment can be applied. Immunotherapy treatment is the most common. CONCLUSIONS: Hidradenitis caused by Leishmania in HIV-negative patients is a rare condition. Therefore it is important to perform a good histological diagnosis and to administer the right treatment.

Idiopathic recurrent palmoplantar hidradenitis in children. Report of 22 cases.

BACKGROUND: Idiopathic recurrent palmoplantar hidradenitis (IRPH) is characterized by tender erythematous plaques and nodules on the soles and, less often, the palms of young patients. To date, 10 cases of IRPH have been documented in the literature. OBSERVATIONS: We describe 22 pediatric patients with characteristic cutaneous and histologic findings of IRPH. Their mean age was 6 years (age range, 1.5-15 years). The onset of the disease clustered in 2 peaks, in autumn and spring. All patients had complete resolution of their lesions within 3 weeks, in 16 cases without any treatment. Ten of the 22 patients experienced more than 1 episode of IRPH. CONCLUSIONS: Our study represents the largest number of cases of IRPH collected to date. Based on our data, the clinical picture is so characteristic that a histopathologic examination may not be necessary in every case. We suggest that there is a seasonality in the occurrence of this always benign and limited disease.

Neutrophil eccrine hidradenitis in a patient with AIDS.

Neutrophilic eccrine hidradenitis (NEH), first described as a rare, transient, and benign complication of various chemotherapy regimens for acute leukemia, has also been observed in other conditions, including three HIV-positive patients and even in otherwise healthy individuals (1-3). A similar histological pattern was described after intradermal bleomycin injections into normal human skin (4). We report the first case of NEH in a hemophilic HIV infected patient treated with stavudine, a new reverse transcriptase inhibitor.

[Eccrine neutrophilic hidradenitis: idiopathic plantar form in children]. / Hidradénite eccrine neutrophilique: forme plantaire idiopathique de l'enfant.

UNLABELLED: In this study, two cases have been reported of idiopathic plantar hidradenitis, an uncommon dermatological pathology with a spontaneous favorable outcome. OBSERVATIONS: Two children aged 12 and 14 years presented with a painful papulo-nodular plantar rash with major functional impairment. The diagnosis of idiopathic plantar hidradenitis was considered, and then confirmed in one case by plantar biopsy. Non-steroidal antiinflammatory drugs, associated with paracetamol in one case were administered. The symptoms disappeared spontaneously within a few days in both cases, without any recurrence. CONCLUSION: A knowledge of the symptoms connected with plantar hidradenitis in the child allows a rapid diagnosis to be made without hospitalization or further medical examination. Analgesic treatment and rest seem to be the only useful approaches. Biopsy to investigate eccrine gland infiltration by neutrophils can only be proposed in the case of an abnormally prolonged duration or an atypical presentation of this pathology.

[Two cases of minocycline-induced vasculitis].

We report two cases of drug-induced vasculitis subsequent to long-term minocycline therapy. Case 1; A 27-year-old woman, who was receiving minocycline hydrochloride for eosinophilic pustular folliculitis of 68-months'duration, showed polyarthralgia, intermittent low-grade fever, and light red reticulated erythemas with subcutaneous nodules on her extremities. Case 2; A 31-year-old man developed reddish-brown, reticulated erythemas with induration on bilateral lower legs after 24-months' duration of minocycline hydrochloride treatment for hidradenitis suppurativa. In both cases, skin biopsies demonstrated vasculitis affecting a small-sized artery at the border of the dermis and subcutis. Serum antineutrophil cytoplasmic antibodies (ANCA) showed borderline elevation. In both cases cessation of minocycline administration resulted in rapid improvement of cutaneous lesions and constitutional symptoms. Recently, attention has been focused on drug-induced ANCA-positive vasculitis. Accumulation of previous case reports of minocycline-induced vasculitis with cutaneous lesions disclosed that the duration of the treatment before clinical symptoms averaged 35 months. Patients may present with livedoid skin lesions accompanied by fever, polyarthritis, autoimmune hepatitis and drug-induced lupus. Early recognition of ANCA-positive vasculitis is essential during long-term minocycline treatment, because cessation of the drug can result in complete resolution.

Black pigmented aortic valve and sinus of Valsalva caused by life-long minocycline therapy.

Minocycline, a derivative of tetracycline, is a broad-spectrum antibiotic used in the treatment of various infections. Black discolouration of the skin, teeth, bones and the thyroid gland are sequelae of long-term minocycline therapy. We report an unusual case of minocycline-induced pigmentation of the aortic valve and sinuses of Valsalva.