Recurrence of non-sexually acquired acute genital ulceration following COVID-19 vaccination.

There is a rare subset of non-sexually acquired acute genital ulcers, previously called Lipschutz ulcers, that are often preceded by a constitutional prodrome and have been associated with multiple viral and bacterial infections. These ulcers are categorized by some as a variant of complex aphthosis, with one hypothesized etiology involving a non-specific systemic inflammatory response to acute infection or vaccination. Although painful, these lesions resolve over the course of several weeks and recurrence is rare but possible. Recently, there have been reports of genital ulcer development due to either acute infection with COVID-19 or following vaccination against the same. We report a case of non-sexually acquired acute genital ulceration that initially presented in 2008 as Lipschutz labial ulcers associated with acute Epstein-Barr virus infection, with recurrence twelve years later following administration of the second dose of the Pfizer-BioNTech COVID-19 vaccine. This case report and exhaustive literature review challenges widely accepted views regarding the typical age range of patients affected by non-sexually acquired acute genital ulceration, the sexual history of affected populations, the pathophysiology of lesion occurrence, and possibility of lesion recurrence.

Follicular occlusion triad: an isotopic response or adverse effect of rituximab?

Follicular occlusion triad is a symptom complex of three conditions with a similar pathophysiology including hidradenitis suppurativa (HS), dissecting cellulitis of the scalp, and acne conglobata. Although the exact pathogenesis of the triad is unknown, it appears to be related to follicular occlusion in areas with apocrine glands. Wolf isotopic response refers to the occurrence of a new dermatosis at the site of another, unrelated, previously healed dermatosis. We present a 26-year-old man with a history of pemphigus foliaceus (PF) who developed large draining nodules with scarring and sinus tracts, compatible with follicular occlusion triad, preferentially at areas previously affected by PF thirteen months after treatment with rituximab. To the authors' knowledge there are no reported cases of follicular occlusion triad or HS manifesting as an isotopic response. However, one member of the triad, HS, has been reported to occur infrequently following the use of biologic agents such as adalimumab, infliximab, tocilizumab, and rituximab for chronic immune-mediated inflammatory diseases (psoriasis, Crohn disease, rheumatoid arthritis, and ankylosing spondylitis).

Successful treatment of two individual cases of generalized granuloma annulare with amoxicillin/clavulanic acid and a combination of doxycycline and pentoxifylline.

Granuloma annulare is a benign inflammatory skin disease potentially related to a delayed hypersensitivity reaction to the dermis. Generalized granuloma annulare (GGA) manifests as diffuse skin-colored to erythematous annular or nummular plaques affecting at least the trunk and either upper or lower extremities, or both. GGA is resistant to many therapeutic modalities, making it difficult to treat. Different therapeutic approaches to GGA have been attempted but definitive treatment for this disease remains elusive. This article focuses on the use of amoxicillin/clavulanic acid and a combination of doxycycline and pentoxifylline therapy as treatment options for GGA in two patients with histopathology-proven interstitial GGA. Both amoxicillin/clavulanic acid and doxycycline inhibit bacterial cell growth, raising the possibility that a bacterial pathogenesis may be of significance in GGA. This is the first reported case of successful treatment of GGA with these regimens.

Rituximab treatment of pemphigus in women of childbearing age: experience with two patients.

Pemphigus vulgaris is an autoimmune blistering disorder treated with systemic steroids and immunosuppressive agents. Treatment of this disorder in young women of childbearing age must take into consideration the patient's desire for pregnancy and effects of the treatment on both mother and child. We report two young women with pemphigus, initially treated with standard immunosuppressive medications, who expressed their wishes for pregnancy. The immunosuppressive agents were tapered and both patients were treated with Rituximab and IVIG, permitting discontinuation of other medications, conception and pregnancy without any oral steroids or immunosuppressive agents. Both patients maintained normal pregnancies and delivered healthy babies, supporting the use of this treatment early in the disease course of this population.

Polymorphic eruption of pregnancy.

Polymorphic eruption of pregnancy (PEP), a specific dermatosis of pregnancy also known as pruritic urticarial papules and plaques of pregnancy (PUPPP), is a benign, self-limited skin disorder. Key features include an increased prevalence in primigravidas, onset in the third trimester, remission near the time of delivery, and association with multiple gestation pregnancy. The clinical features are crucial to diagnosis. Histopathology is nonspecific, and immunofluorescence studies help differentiate PEP from pemphigoid gestationis. The pathogenesis of PEP remains elusive, and relevant theories are reviewed. There are no associated maternal or fetal risks, and treatment is largely symptomatic.

IgM ocular cicatricial pemphigoid: a unique insight into the immune system.

A 42-year-old man with ocular erythema and scarring had a conjunctival biopsy revealing depostion of IgM and C3 without IgG at the epithelial basement membrane zone.  Treatment with doxycyline, dapsone, and mycophenolate mofetil was unsuccessful and treatment with rituximab has led to partial remission of the conjunctival inflammation. He has undergone 4 cycles of rituximab treatment at intervals of 12 to 18 months.

Successful long-term thalidomide therapy for discoid lupus erythematosus-lichen planus overlap syndrome.

Although thalidomide is a U.S. Food and Drug Admistration (FDA) approved medication for erythema nodosum leprosum and multiple myeloma, it has many off-label uses, including for discoid lupus erythematosus (DLE), Behçet's disease, apththous ulcers in HIV patients, and prurigo nodularis. Herein, we present a patient with an overlap of discoid lupus erythematosus and lichen planus who was successfully treated with thalidomide for over 19 years without significant side effects. We propose that some of the most common side effects, including peripheral neuropathy, numbness, parasthesias, sedation, and constipation, can be avoided at lower doses, typically less than 100mg/day.

Adalimumab Induced Subcutaneous Nodular Sarcoidosis; A Rare Side Effect of Tumor Necrosis Factor-α Inhibitor.

Adalimumab and other tumor necrosis factor-α inhibitors have been shown in the recent years to successfully treat sarcoidosis refractory to systemic corticosteroids and other agents.  However, there have been an increasing number of cases of sarcoidosis paradoxically induced by these agents.  It is hypothesized that this is due to the disruption of the fine balance of cytokines involved in granuloma formation. We describe the first case of adalimumab-induced subcutaneous nodular sarcoidosis in a patient with pulmonary sarcoidosis.

A hyperpigmented perianal nodule.

Basal cell carcinoma (BCC) is the most common cutaneous malignancy. Ultraviolet light is an important risk factor for the pathogenesis of BCCs; the vast majority are found in sun-exposed areas. BCCs occurring in the perianal or genital regions are seldom seen. Less than 1% of all BCCs occur at these sites. Etiologic factors other than solar exposure must be taken into account for such cases. We report a rare case of BCC that was initially detected during a routine colonoscopy.

Genital ulcers associated with Epstein-Barr virus.

Genital ulcerations are a rare clinical finding of Epstein-Barr virus (EBV) infection. We present the case of a 16-year-old adolescent girl who reported the onset of diarrhea, headaches, dysuria, and vaginal discharge along with vulvar ulcerations with edema. Laboratory studies revealed a high-quantitative EBV IgG and early antigen as well as a positive IgM antibody for EBV. Although the association between EBV and genital ulcerations is rare, physicians should be aware of this clinical presentation to exclude other infectious entities, to be reassured to accept negative testing, and to quell patient distress or concerns of abuse.

Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: an overview.

Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis that is associated with systemic features including fevers, hepatosplenomegaly, lymphadenopathy, pancytopenia, hepatic abnormalities, hypertriglyceridemia, and coagulopathy without an elevated erythrocyte sedimentation rate. The panniculitis lesions show adipose tissue lymphocytic and histiocytic infiltration along with hemophagocytosis, which may also appear in bone marrow, spleen, lymph nodes, and liver. Patients may have a rapidly fatal disease course, a longer disease course with intermittent remissions and exacerbations for many years prior to death, or a nonfatal acute or intermittent course responsive to treatment. The cytophagocytic disorder in these patients is a hemophagocytic lymphohistiocytosis (HLH), similar to the infection-activated reaction associated with perforin mutations found in familial hemophagocytic lymphohistiocytosis. HLH is a group of autoinflammatory disorders, which include macrophage activation syndrome and infection-associated hemophagocytic syndrome, which if not treated rapidly, can be fatal. The relationship of CHP and HLH is discussed. CHP associated diseases include: subcutaneous panniculitis-like T cell lymphomas; infections, connective tissue diseases, other malignancies, and the molecular disorders that cause HLH. Treatment of CHP includes: glucocorticoids in combination with cyclosporine, combined chemotherapeutic medications and most recently, anakinra, an Interleukin-1 receptor antagonist; along with supportive care, search for underlying malignancies and treatment thereof, and control of associated infections.