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Panniculitides are a group of diseases characterized by an inflammatory process that originates primarily in the subcutaneous tissue. Histopathological classification of panniculitides in mostly septal or lobular types, according to the predominant localization of the infiltrate, is complemented with the eventual identification of vasculitis and the consideration of the vessel size. Additional steps involve the characterization of the inflammatory infiltrate and other findings, such as, the type of adipocyte necrosis or the presence of sclerosis, crystals and foreign bodies. Apart from this purely histopathological characteristics, clinico-pathological correlation is often required to encompass the clinical, histopathological and laboratory data in order obtain the correct diagnosis. We will review the classification of panniculitis, useful histopathological clues for their diagnosis and diagnostic algorithms that can be applied when sclerosis is present or the inflammatory infiltrate is mostly neutrophilic.

Erythema induratum (EI) is a rare chronic disease, which occurs with cutaneous tuberculosis (TB). Nodular vasculitis, much rarer than erythema induratum, describes the same condition but without cutaneous TB, with lesions usually in the lower legs and rarely on the breasts. We report the case of a 46-year-old female with a history of crusted skin and necrotic lesions two years before, which, once self-limited, multiplied one month before and transferred to uncommon sites of the body, such as the breast. There was no evidence of other clinical presentations, and a chest X-ray gave no pathological findings. A biopsy was taken from the lesions, and the patient was diagnosed with erythema induratum without cutaneous tuberculosis, that is, nodular vasculitis. After treatment with calcineurin-inhibiting tacrolimus ointment, topical corticosteroids, and immunosuppressive oral azathioprine, the lesions improved.

The skin is one of the organs most commonly affected by vasculitis and the only one that is open to direct inspection. Cutaneous vasculitis can be part of a systemic vasculitis, a variant restricted to the skin (e.g. systemic and cutaneous IgA1 vasculitis) or an independent cutaneous form (recurrent macular vasculitis in hypergammaglobulinemia, nodular vasculitis). For the nomenclature and classification of cutaneous vasculitides the scheme of the Chapel Hill Consensus Conference can be used and the vessels mainly affected determine the clinical picture of individual forms of vasculitis. Some cutaneous efflorescences and their distribution are so characteristic for certain forms of vasculitis that they provide diagnostic indications or even diagnostic criteria. A palpable purpura on the legs is typical for involvement of postcapillary venules in the context of immune complex vasculitis. If arterioles or venules in the dermis are additionally affected, the clinical presentation is that of plaques with marginal offshoots and central hemorrhagic blisters or necrosis (retiform purpura). In contrast to the purpura in occluding vasculopathies, which shows no or little surrounding erythema, the purpura in vasculitis is accompanied by an inflammatory erythema. In vasculitides of the arterioles and small-caliber arteries in the subcutaneous tissue, visible or only palpable nodules exist surrounded by an irregular livedo or also retiform purpura. Understanding how vasculitis-induced efflorescence arises and the histological manifestations helps their recognition during physical examinations in daily practice and is an important guide in the diagnosis and classification of vasculitides.

Background Erythema induratum of Bazin (EIB) – nodular vasculitis associated with Mycobacterium tuberculosis (TB) – and Tuberculosis-Associated Ocular Inflammation (TB-AOI) represent uncommon manifestations of TB. There is limited data and a lack of diagnostic and treatment standards for these conditions. Methods Eleven-year retrospective review of EIB and TB-AOI cases managed in a provincial TB program with prospective phone-based follow-up of anti-tubercular therapy (ATT) recipients. Presumptive TB-AOI and EIB diagnoses were determined by ophthalmologist or dermatologist assessments correlated with positive tuberculin skin test and/or QuantiFERON-TB Gold, along with pathologic criteria in EIB cases. Results Of 21 EIB and 20 TB-AOI cases that received ATT, 13 and 11, respectively, were reached for follow-up. The majority of EIB and TB-AOI cases were female and immigrated from TB high-burden countries. Median durations of pre-diagnosis symptoms were 2 and 0.8 years (IQR 2.5 & 1.1) for EIB and TB-AOI cases, respectively. Overall, 14 different ATT regimens were used for a median duration of 6 months (range 5–9). ATT related adverse events resulting in treatment discontinuation occurred in 14% of EIB and 10% of TB-AOI cases. On last follow-up, 76% of EIB and 42% of TB-AOI had improvement or resolution of disease. Conclusion EIB and TB-AOI were uncommon presentations receiving variable therapy. While treatment response was modest for EIB cases, TB-AOI cases had sub-optimal treatment outcomes. The unique diagnostic and management challenges presented by these conditions in TB low-incidence settings highlight a need for improved treatment candidate selection, therapy standardization, and cross-specialty medical collaboration. Electronic supplementary material The online version of this article (10.1186/s12879-019-3737-5) contains supplementary material, which is available to authorized users.

Purpose of review Cutaneous vasculitis reflects a spectrum ranging from skin limited to severe systemic forms. To date, there is still no generally acknowledged nomenclature for cutaneous vasculitis. This review aims to summarize the recent advances in the nomenclature of cutaneous vasculitis. Recent findings The most widely adopted vasculitis classification system is the one of 2012 Revised Chapel Hill Consensus Conference (CHCC) which represent not such a classification but a nomenclature system that name vasculitis on the basis of the size of the vessel affected. The CHCC 2012 did not deal with the special features of cutaneous vasculitis and did not explicitly discuss the presence of skin-limited or skin-dominant forms of vasculitis. Therefore, a consensus group was formed to propose an Addendum to CHCC 2012, focusing on cutaneous vasculitis. The Addendum better clarify the main aspects of some single-organ vasculitis, including IgM/IgG vasculitis, nodular vasculitis, erythema elevatum et diutinum and recurrent macular vasculitis in hypergammaglobulinemia. Moreover, it differentiated normocomplementemic from hypocomplementemic urticarial vasculitis. Finally, it recognized cutaneous polyarteritis nodosa as a distinct subtype of polyarteritis nodosa. Summary Classification criteria are useful tools to standardize names and definitions for cutaneous vasculitis; however, they do not represent diagnostic criteria. Collaborative efforts are still needed to get a shared classification and valid diagnostic criteria for cutaneous vasculitis.

Objectives: Potassium iodide (KI) is a medication that has been used for decades in dermatology and it is mentioned as a treatment option in all major dermatology textbooks. Yet, there is little recent information on its efficacy. In our study, we wanted to retrospectively evaluate the therapy response to KI in our patients. Methods: The hospital information system was searched for patients treated with KI at the Department of Dermatology (University Hospital Zurich) in the last 20 years (January 1, 1998 to December 31, 2017). A total of 52 patients were found and, subsequently, 35 patients were included in our study. Results: KI was prescribed for the following skin conditions: erythema nodosum, disseminated granuloma anulare, necrobiosis lipoidica, nodular vasculitis, cutaneous sarcoidosis, and granulomatous perioral dermatitis/ rosacea. The median duration of KI intake was 5 ± 7.7 weeks (range 1–26). The global assessment of efficacy by the treating physician showed an improvement of disease in about a third of all patients. No response was seen in 14 patients and 9 even had a progression of disease. An adverse event was documented in 16 cases. Conclusions: Our findings show that an improvement was reached in only about a third of all cases. High response rates with only mild side effects (in 16 out of 35 patients) were observed.

INTRODUCTION: Erythema Induratum (EI) is a relatively rare dermatologic disorder affecting subcutaneous fat tissue, which is often associated with Mycobacterium tuberculosis. This report details the presentation, diagnosis and management in a 70-year-old female who presented with a painful erythematous annular rash at the clinic. The rash was later diagnosed as EI associated with Mycobacterium leprae, one rarely seen in literature. DISCUSSION: EI is a rare form of panniculitis that typically presents as a recurrent grouping of tender nodules and plaques on the posterior aspect of the lower legs. Although EI is considered idiopathic in most cases, it can be associated with M. leprae. Given the atypical presentation of a rash, a biopsy was done. It showed epithelioid granulomatous dermatitis with lobar panniculitis. A DNA polymerase chain reaction (PCR) was also sent and revealed the presence of M. leprae. Treatment of EI without association with M. leprae includes potassium iodide, non-steroidal anti-inflammatory drugs (NSAIDs), rest, elevation, compression and, in severe cases, systemic immunosupressives. If tuberculoid leprosy is confirmed, the attending physician is encouraged to consult the infectious disease department as treatment varies with presentation. CONCLUSIONS: This case details the diagnosis and management involved in a case of tuberculoid leprosy masquerading as EI. Management of the EI involved NSAIDs and potassium iodide. The leprosy was treated with dapsone and rifampin in conjunction with an infectious disease consultation. Our case highlights the importance of relying on a strong clinical suspicion based on a patient’s social history in order to diagnose rare entities accurately.

A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Differentiating between interstitial granulomatous dermatitis and the interstitial form of granuloma annulare, early morphea and variants of borreliosis or scleromyxedema as well as interstitial granulomatous drug reaction can be very difficult. In long-standing cutaneous granulomatous infiltrates, T‑cell lymphoma should be excluded. Occasionally only a small number of atypical lymphocytes can be found. The detection of a monoclonal T‑cell expansion is then particularly helpful. Only recently, a CD8-positive granulomatous cutaneous T‑cell lymphoma was described which occurred in patients with immunodeficiencies. A granulomatous vasculitis in the skin is extremely rare. According to the Chapel Hill classification from 2012, systemic granulomatous vasculitis is equated with giant cell arteritis. Extracutaneous large arteries are involved. On rare occasions, involvement of the temporal artery can result in skin necrosis. More commonly granulomatous infiltrates in combination with vasculitis can be observed, whereby various infectious diseases, sarcoidosis and nodular vasculitis should be considered. Granulomatosis with polyangiitis (formerly Wegener's granulomatosis), presents in the skin as leukocytoclastic vasculitis. Here granulomas are extremely rare.

Nodular vasculitis (NV) refers to a chronic relapsing lobular panniculitis that is thought to be a hypersensitivity reaction to antigenic triggers. While it is commonly associated with tuberculosis, in many cases no underlying cause is found and the condition is difficult to manage. Here, we report three patients with refractory idiopathic NV effectively treated with colchicine, leading to a significant improvement or complete resolution of their symptoms. Colchicine was successfully used as a steroid-sparing agent, and in one patient its cessation was associated with a flare of disease.

We report the first case of recently characterized species M. monacense associated with chronic nodular vasculitis, infecting a young woman. This case represents the first isolation of M. monacense from Ecuador. The isolate was identified by conventional and molecular techniques.

A 30-year-old Japanese female noticed painful erythematous nodules with ulcers on both legs in 2000. Although the ulcers in the nodules disappeared spontaneously within several months, they recurred repeatedly in 2013 and 2014. She was clinically diagnosed with pyoderma gangrenosum (PG) and treated by a local doctor with oral prednisolone at 15 mg/day, which led to improvement. Leg ulcers recurred again in 2015, and she was therefore referred to our hospital. Physical findings demonstrated many [...]

Erythema induratum (EI), or nodular vasculitis (NV), is a type of panniculitis that is often associated with vasculitis affecting various-sized veins, venules, and arteries in reaction to various causative factors. Historically, EI was highly linked to tuberculosis, but in 1946, Montgomery first proposed the term NV to describe cases of EI not associated with tuberculosis. Only 2 reports of NV associated with inflammatory bowel disease have been reported in the literature. The authors report a 60-year-old woman with Crohn’s disease presenting with exacerbation of NV in the setting of vedolizumab therapy.

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.

We report a case of tuberculous granulomatous panniculitis without vasculitis in an 87-year-old female patient with B-cell chronic lymphocytic leukaemia. One month after starting chemotherapy with chlorambucil and prednisone she presented superficial erythematous plaques on the anterior side of the left leg. Three weeks later erythematous painless deep nodules appeared on the left popliteal fossa and on the left thigh. Cutaneous biopsy revealed granulomatous panniculitis without caseation necrosis or vasculitis. Polymerase chain reaction for Mycobacterium tuberculosis revealed positivity in the skin. The final diagnosis was reactivation of latent tuberculosis (TB) induced by deep immunosuppression associated with chemotherapy and haematological disease. Tuberculous granulomatous panniculitis without vasculitis is a rare presentation of cutaneous TB and may be part of the heterogeneous histopathologic spectrum of erythema induratum of Bazin (nodular vasculitis). Our case shows that the diagnosis of cutaneous TB requires the correlation of clinical findings with histopathology and microbiological tests.

We report a case of recalcitrant nodular vasculitis in a 23 years old male who was treated successfully with methotrexate. He presented to our Dermatology out Patient Department, Tribhuvan university teaching hospital with complaints of recurrent painful discharging ulcer over bilateral calf and lower legs of three years duration. The diagnosis of nodular vasculitis was made on the basis of the clinical presentation and the histopathological findings. The patient was treated with antituberculosis treatment with initial good response; however the lesions persisted later on, despite completing the course of ATT. The patient did not respond to treatment with systemic corticosteroids also. On starting methotrexate, the lesion started healing within a period of two weeks and therewas significant improvement in subsequent one month. This case report highlights the efficacy of methotrexate in the treatment of recalcitrant nodular vasculitis. Keywords: ATT; methotrexate; nodular vasculitis.

Nodular vasculitis was introduced by Montgomery for cases of erythema induratum-like lesions that were not associated with tuberculosis. Nodular vasculitis has been associated with both nontuberculous infections and noninfectious conditions. However, there has been no report on the development of nodular vasculitis during tumor necrosis factor-α inhibitor treatment. A 28-year-old man visited our clinic for the treatment of severe psoriasis with a 20-year history. Subcutaneous injection of etanercept (25 mg, twice weekly) was started. One year later, erythematous nodules developed on his lower leg. A skin biopsy showed lobular panniculitis with extensive necrosis and vasculitis. To exclude latent tuberculosis, an assay specific for Mycobacterium tuberculosis antigens was performed, with a negative result. After stopping etanercept under the diagnosis of nodular vasculitis associated with etanercept, the lesions gradually disappeared, leaving depressed scars in 3 months. There has been no recurrence after 6 months of follow-up.

Erythema Induratum of Bazin (EIB) is a tuberculid showing lobular panniculitis. EIB is classified under cutaneous tuberculosis. A diagnosis of EIB is based on cutaneous characteristics, a positive mantoux test, evidence of tuberculosis and histopathological findings. We present a case of a 43-year-old lady with recurrent crops of nodules on legs. Mantoux test was positive. Histopathology revealed lobular panniculitis, epithelioid cells, lymphocytes and giant cells surrounding foci of caseous necrosis which was suggestive of erythema induratum. Her lesions responded to antitubercular therapy (ATT).

The skin not only represents the organ which often reveals the first signs of systemic vasculitis, but also the organ which is most frequently involved in vasculitis. These diseases encompass systemic vasculitides and those which appear to involve the skin only. Among those vasculitides restricted to the skin, some are yet typically associated with other systemic diseases, such as nodular vasculitis, which often occurs during infections by M. tuberculosis, or erythema elevatum diutinum in patients with gammopathy. The type and localization of skin lesions give valuable indications as to the type of vasculitis. Subcutaneous nodules which ulcerate and are surrounded by livedo racemosa are suggestive of polyarteritis nodosa, a palpable purpura with predilection for the lower legs is almost pathognomonic for immune complex vasculitis (e.g. IgA vasculitis or cutaneous leukocytoclastic vasculitis), hemorrhagic papules and necrotic plaques which occur in acral areas after cooling indicate cryoglobulinemic vasculitis, hemorrhagic papules and macules which develop in patients who start to feel worse and develop fever should arouse suspicion of septic vasulitis, while the simultaneous presence of ulcerating nodules and hemorrhagic papules without predilection for the lower legs will suggest ANCA-associated vasculitis. The different morphology of the cutaneous signs of the various vasculitides depends to a large extent on the size of the vessels primarily involved. In this review the cutaneous signs of vasculitides will be presented with reference to the revised nomenclature of the Chapel Hill Consensus Conference from 2012.

We herein report a patient with erythema induratum/nodular vasculitis (EI/NV) associated with Crohn's disease (CD), which is considered to be a rare type of metastatic CD. A 54-year-old woman, who had a history of CD, presented with erythematous nodules on her legs. The histopathological features of the skin biopsy revealed a granulomatous, mixed septal and lobular panniculitis, which was characterized by many discrete epithelioid cell granulomas (necrobiotic/necrotizing-type and sarcoidal type), necrosis of the adipocytes, and granulomatous phlebitis in the muscular wall of a subcutaneous vein. A review of the pertinent literature and the presented case suggested the following: (1) panniculitis associated with CD may be either an erythema nodosum type or an EI/NV type; (2) so far, the reported cases of metastatic CD or granulomatous vasculitis in CD rarely presented with granulomatous panniculitis without dermal involvement, and most cases showed histopathological features that were similar to or indistinguishable from those of EI/NV; and (3) the finding of granulomatous vasculitis (especially the presence of discrete epithelioid cell granulomas involving the veins or venules) may be a characteristic feature of EI/NV associated with CD, in contrast to the finding of acute vasculitis, which is typically present in patients with EI/NV due to causative factors other than CD.

We report a chronic case of nodular vasculitis that responded to oral clofazimine 300 mg daily. The condition had previously responded to moderate dose oral prednisolone, 50 mg daily, but would recur with weaning. Multiple corticosteroid-sparing agents were trialled, however these were either ineffective or poorly tolerated. The introduction of clofazimine enabled prednisolone dose reduction, not achieved with other agents, to 22.5 mg daily, and was associated with complete suppression of disease activity. Unfortunately the patient developed a clofazimine-induced enteropathy and the treatment was ceased after almost 2 years of therapy. Cessation of clofazimine was associated with a flare of the condition. Clofazimine should be considered as a corticosteroid-sparing agent in resistant cases of nodular vasculitis. Clinicians should be aware of clofazimine-induced enteropathy as a potentially serious complication of the therapy.

We present the case history of a 48-year-old male patient with Chlamydophila (Chlamydia) pneumoniae who developed a nodular vasculitis. He developed a cutaneous vasculitis with the onset of respiratory symptoms. The diagnosis of Chlamydophila pneumoniae infection was based on serology. Since this infection is very common in our population, although often asymptomatic, it should be systematically considered as a causative agent of nodular vasculitis.