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Autoinflammatory diseases (AIDs) characterized by recurrent episodes of localized or systemic inflammation are disorders of the innate immune system. Skin lesions are commonly found in AIDs and cutaneous vasculitis can coexist with AIDs and even present as the most striking feature. This review aims to focus on the frequent cutaneous vasculitis association in three monogenic AIDs including familial Mediterranean fever (FMF), deficiency of adenosine deaminase type 2 (DADA2), and the recently identified adult‐onset VEXAS (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) syndrome. Cutaneous vasculitis in FMF is characterized by: (1) small‐vessel vasculitis similar to IgA vasculitis with palpable purpura but increased intussusception complication and less vascular IgA deposit, and (2) cutaneous arteritis–like vasculitis presenting as subcutaneous nodules most often with higher glomerular involvement. DADA2 has a wide spectrum of clinical presentations ranging from fatal systemic vasculitis with multiple strokes, especially in pediatric patients, to limited cutaneous disease in middle‐aged patients. DADA2 shares similar clinical and histopathological features with polyarteritis nodosa (PAN). As a result, DADA2 is commonly initially misdiagnosed as childhood PAN. Livedo racemosa reveals the most common cutaneous manifestation of cutaneous vasculitis in patients with DADA2. VEXAS syndrome is a life‐threatening disease. A diagnosis of VEXAS syndrome should be strongly considered or could be made in patients with skin lesions characterized by Sweet syndrome–like eruption, livedo racemosa, concomitant relapsing polychondritis, deep venous thrombosis, pulmonary involvement, and progressive hematologic abnormalities such as myelodysplastic syndrome with a unique finding of cytoplasmic vacuoles in myeloid and erythroid precursor cells from bone marrow aspirate smear. As skin involvement is common in AIDs and may present as the most frequent manifestation, especially in DADA2 (70% to 90%) and VEXAS syndrome (83% to 91%), dermatologists play a crucial role in contributing to the early diagnosis of these AIDs with early initiation of the appropriate therapy to avoid progressing fatal outcomes. [ABSTRACT FROM AUTHOR]

Cutaneous vasculitis can be classified into two types based on the affected vessel size: small vessel vasculitis predominantly affecting dermal venules, and muscular vessel vasculitis as found in cutaneous arteritis predominantly affecting arteries located at the dermal‐subcutaneous junction. We describe two cases with a novel small vessel vasculitis disorder, which exclusively affected arterioles in the mid‐dermis, and show clinical and pathological difference distinct from cutaneous polyarteritis nodosa and cutaneous venulitis. Both patients were male, and presented with painful infiltrative plaques, involving the palms, soles, and thighs without extracutaneous involvement except for fever and arthralgia. Histopathological examination revealed vasculitis in the mid‐dermis characterized by a predominant infiltration of neutrophils with vessel wall fibrinoid necrosis and leukocytoclasia identical to the features of leukocytoclastic vasculitis, except that the affected vessels were arterioles rather than venules. Serological examinations showed normal levels of serum complements, immune complexes, and antineutrophil cytoplasmic antibodies, and vasculitis disorders associated with systemic diseases were excluded in both patients. The patients showed a good response to short‐term treatment with prednisolone up to 30 mg. This novel cutaneous arteriolitis clinicopathologically different from both cutaneous venulitis and cutaneous arteritis appears to be a skin‐limited disorder. [ABSTRACT FROM AUTHOR]

I Dear editor, i Pityriasis lichenoides et varioliformis acuta (PLEVA) is a cutaneous inflammatory disease that presents with infiltrated erythema, papules with collarette scales and haemorrhagic or necrotising crusts on lower extremities [Correction added on 27 April 2020, after first online publication: "acuta" has been added to pityriasis lichenoides et varioliformis in the article title and first paragraph.]. Although it has been known to present features of lymphocytic vasculitis, the key finding of lymphocytic vasculitis characterised by vessel wall fibrinoid necrosis with a predominant infiltrate of lymphocytes has rarely been demonstrated. (HE, x20). c, d) Lymphocytic vasculitis with fibrinoid necrosis of the involved vessel wall and a predominant infiltrate of lymphocytes with the absence of nuclear debris. [Extracted from the article]

Abstract: The proposal by the 1994 International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC1994) and by the CHCC2012 markedly influenced the classification and way of considering cutaneous vasculitis. In the proposal by the CHCC1994, hypersensitivity angiitis was defined as an equivalent pathological condition to microscopic polyangiitis or cutaneous leukocytoclastic angiitis (CLA), and it was not adopted as a disease name. However, CLA which was positioned as a type of small‐vessel vasculitis is only a pathological name. In the proposal by the CHCC2012, a new category of single‐organ vasculitis included CLA and cutaneous arteritis. Vasculitis allergica cutis (Ruiter) corresponded to CLA and cutaneous polyarteritis nodosa corresponded to cutaneous arteritis. The Japanese Dermatological Association (JDA) prepared guidelines for the management of vasculitis and vascular disorders in 2008 based on the proposal by the CHCC1994 and their original viewpoint of dermatology. The JDA subsequently revised the 2008 edition guidelines in 2016 following publication of the proposal of the CHCC2012 in Japanese. We presented the outline of the 2016 edition guidelines and propose a treatment algorithm for primary vasculitides based on the evaluation of the cutaneous symptoms for cases suspected as primary cutaneous vasculitides, which integrates the 2008 JDA guideline and CHCC2012 classification. This is the secondary English version of the original Japanese manuscript for the guideline for management of vasculitis and vascular disorders published in the Japanese Journal of Dermatology 127(3); 299–415, 2017. [ABSTRACT FROM AUTHOR]

Objective: To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D‐CHCC). The goal was to standardize the names and definitions for cutaneous vasculitis. Methods: A nominal group technique with a facilitator was used to reach consensus on the D‐CHCC nomenclature, using multiple face‐to‐face meetings, e‐mail discussions, and teleconferences. Results: Standardized names, definitions, and descriptions were adopted for cutaneous components of systemic vasculitides (e.g., cutaneous IgA vasculitis as a component of systemic IgA vasculitis), skin‐limited variants of systemic vasculitides (e.g., skin‐limited IgA vasculitis, drug‐induced skin‐limited antineutrophil cytoplasmic antibody–associated vasculitis), and cutaneous single‐organ vasculitides that have no systemic counterparts (e.g., nodular vasculitis). Cutaneous vasculitides that were not included in the CHCC2012 nomenclature were introduced. Conclusion: Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for cutaneous vasculitis. Accurate identification of specifically defined variants of systemic and skin‐limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D‐CHCC, provides a standard framework both for clinicians and for investigators. [ABSTRACT FROM AUTHOR]

Background There is controversy on whether lymphocytic thrombophilic arteritis ( LTA) and macular arteritis ( MA) are a different entity from cutaneous polyarteritis nodosa (C- PAN). Objective To evaluate the controversy on LTA/ MA by examining the morphologic changes during the dynamic inflammatory process. Materials and methods A clinical and histopathological investigation of 46 biopsy specimens from 21 histopathologically proven C- PAN patients at our hospital was performed. Results Most of the biopsy findings revealed arteritis in the subacute and reparative stages. Coexistence of different stages was found in over half of the specimens. Two biopsy specimens consistent with LTA/ MA showed coexisting histologically proven (subacute stage) C- PAN in other lesions. Analyses of the longitudinal sections showed focal and segmental arteritis. Patients overall improved well with compression stockings, anti-inflammatory agents, a prostaglandin analog and antiplatelet agents. Currently, three patients are medication free and only require compression stockings. Conclusion LTA/ MA seems to be part of the spectrum of C- PAN. The vasculitic lesions of C- PAN are segmental and focal, and coexist in different stages. As the chronic stage of C- PAN can be mistaken as LTA/ MA, it is necessary to perform multiple biopsies, serial sections and analyses of longitudinal sections to confirm the main finding of vasculitis. [ABSTRACT FROM AUTHOR]

Key Clinical Message: We reported a patient with systemic lupus erythematosus complicated by livedoid vasculopathy (LV), who responded well to intravenous immunoglobulin and warfarin. Cutaneous lesions of LV resemble those of cutaneous vasculitis. LV should be included in the differential diagnosis of leg ulcerations even in the presence of autoimmune disorders. We reported a patient with systemic lupus erythematosus complicated by livedoid vasculopathy (LV), who responded well to intravenous immunoglobulin and warfarin. Cutaneous lesions of LV resemble those of cutaneous vasculitis. LV should be included in the differential diagnosis of leg ulcerations even in the presence of autoimmune disorders. [ABSTRACT FROM AUTHOR]

The main histopathological features in the cutaneous lesions of Churg-Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non-vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Ishibashi M, Kudo S, Yamamoto K, Shimai N, Chen K-R. Churg-Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid-like blisters. [ABSTRACT FROM AUTHOR]

Background: Although granulomatous arteritis is usually found in extracutaneous Churg–Strauss syndrome (CSS) lesions, the vasculitis in CSS cutaneous lesions typically shows small vessel vasculitis (leukocytoclastic vasculitis) without demonstrating the feature of granulomatous arteritis confirming the proper classification of CSS in the category of granulomatous vasculitis. Methods: Four deep excisional biopsies were obtained from three untreated CSS patients who presented with livedo reticularis and subcutaneous nodules. Tissue blocks were recut and submitted for hematoxylin and eosin and elastic tissue staining to evaluate the histological features of the affected vessels. Immunostaining for histiocytes, lymphocytes, and neutrophils were performed on serial sections to confirm the cellular infiltration. Results: In all specimens, subcutaneous granulomatous arteritis was observed. The unique histological feature distinct from other vasculitic disorders is characterized by marked infiltration of histiocytes and multinucleated giant cells in and around the disrupted subcutaneous arterial walls mixed with an eosinophilic infiltrate. In two specimens, granulomatous arteritis was found in the subsequent serial sections, not in the initial sections. The initial section may show extravascular granulomatous inflammation without evidence of vasculitis. Conclusions: Granulomatous arteritis as identified in the extracutaneous lesions can also be found in subcutaneous CSS lesions presenting with livedo reticularis and/or subcutaneous nodules. [ABSTRACT FROM AUTHOR]

The article presents information on the Ampiroxicam-induced photosensitivity. Ampiroxicam is a new prodrug of piroxicam, a non-steroidal anti-inflammatory drug, and has been available in Japan only since 1994. Because it is converted to piroxicam during absorption through the intestine, gastric side-effects are held to be minimized. Ampiroxicam is expected to induce photosensitive eruptions, as is well-known with piroxicam. It has been suggested that piroxicam-induced photosensitivity is caused by a phototoxic mechanism, because it usually occurs within a few days

A case of lupus erythematosus/lichen planus overlap syndrome (LE/LP overlap syndrome) was reported. A 53-year-old woman developed violaceous erythema around the nostrils and the upper lips and atrophic scaly erythema on the cheeks and neck. Histopathological studies revealed that the patient had distinct discoid lupus erythematosus (DLE), LP, and a lesion with combined features of DLE and LP. Direct immunofluorescent (DIF) studies of the mixed lesion revealed both prominent immunoglobulin (Ig)G deposits in a granular pattern at the basement membrane zone (BMZ) and IgM deposits in the clusters of cytoid bodies; the former are more typical of LE and the latter more of LP. DIF features in combination were unique for LE/LP overlap syndrome. The patient was satisfactorily treated with topical tacrolimus. While reports support the effectiveness of tacrolimus in either LE or LP, this is the first case of LE/LP overlap syndrome treated with topical tacrolimus.