Iris K. Aronson

Pruritic urticarial papules and plaques of pregnancy: Clinical and immunopathologic observations in 57 patients

BACKGROUND Pruritic urticarial papules and plaques of pregnancy (PUPPP) has been described either as a homogeneous or a polymorphic clinical process. Its cause is unknown. OBJECTIVE We attempted to characterize the clinical and immunopathologic findings in PUPPP on the basis of long-term clinical and immunopathologic observations. METHODS The clinical and immunopathologic features of 57 patients with PUPPP were evaluated. RESULTS The clinical features in 57 patients with PUPPP were categorized into three types: mainly urticarial papules and plaques (type I), nonurticarial erythema, papules, or vesicles (type II), and combinations of the two forms (type III). Direct immunofluorescence studies in 48 of the 57 patients showed nonspecific immunoreactants in dermal blood vessels and/or moderate granular deposits at the dermoepidermal junction in 15 patients. CONCLUSION Type I PUPPP differed from types II and III in clinical appearance and distribution (absence of face, palm, and sole lesions), but trimester onset, parity, and direct immunofluorescence findings were not significantly different among the three groups.

Panniculitis associated with cutaneous T‐cell lymphoma and cytophagocytic histiocytosis

A 36‐year‐old woman had a 6‐year history of recurrent panniculitis with development of an angiocentric and angiodestructive cutaneous T‐cell lymphoma (CTCL) of the helper cell phenotype. She subsequently developed a rapidly progressive fatal syndrome characterized by cytophagocytic histiocytosis and hyperlipidaemia. Cytophagocytic histiocytosis has previously been reported in association with panniculitis, malignancy and infection, but not with CTCL and the precise relationship between panniculitis, CTCL, cytophagocytic histiocytosis and hyperlipidemia is unclear.

Evaluation of topical metronidazole gel in acne rosacea.

Topical metronidazole gel (0.75%) was compared to placebo gel in a randomized, double-blind, placebo-controlled, split-face clinical trial for the treatment of 59 patients with acne rosacea. Statistically significant differences in inflammatory lesions, erythema, and global assessments were seen at three, six, and nine weeks post-baseline in favor of the active treatment side. It did not, however, alter the telangiectatic component of the disease. No known drug-related side effects were detected, and the low topical dose along with low serum levels of metronidazole indicate a high safety profile for this therapeutic agent. This work suggests that metronidazole gel, as specifically formulated, is safe and effective in reducing the symptomatology of acne rosacea.

Panniculitis associated with histoplasmosis and alpha1-antitrypsin deficiency

A patient with mediastinal histoplasmosis, alpha 1-antitrypsin deficiency, and panniculitis is presented. The patient showed dramatic response to treatment with ketoconazole. The associations of panniculitis with histoplasmosis and alpha 1-antitrypsin deficiency are discussed.

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.

Topical Mupirocin in the Treatment of Bacterial Skin Infections

Mupirocin is an investigational topical antibiotic used for treatment and prophylaxis of bacterial skin infections. Mupirocin differs from other antibiotics in its synthesis, structure, and mechanism of action. In vitro, mupirocin possesses antimicrobial activity against staphylococci, streptococci, Hemophilus influenzae, and Neisseria gonorrhoeae. Few studies comparing mupirocin to other topical antibiotics are available. Initial studies comparing mupirocin to inactive vehicle in the treatment of impetigo indicate an overall 92 percent pathogen eradication rate with active drug and 58 percent eradication rate with vehicle. Overall response to treatment of secondary skin infections was favorable in 91 percent of patients treated with mupirocin and 77 percent of those treated with vehicle. Although incidence is not greater than placebo, adverse effects have included pruritus, burning, dry skin, and erythema. Additional trials and clinical use should further help determine the role of mupirocin in the treatment of minor, primary, and secondary skin infections.

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

Abstract 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.

Psoriasis and normouricemic gout.

A patient with moderately extensive psoriasis developed acute gouty arthritis while receiving topical corticosteroid and tar therapy. The serum uric acid levels were normal before, during, and after the acute gouty episode. Patients predisposed to gout by virtue of an appropriate associated condition should be evaluated for gout even in the presence of normal serum uric acid.

Lymphangioma-like Kaposi Sarcoma in Transplant-Associated Iatrogenic Immunosuppression: A Case Report

Lymphangioma-like Kaposi sarcoma (LLKS) is a rare histologic presentation of Kaposi sarcoma (KS), with only 28 cases reported in the literature. LLKS has been described in acquired immunodeficiency syndrome and in endemic African-type as well as classic indolent KS. We present the 1st reported case of LLKS in a transplant-associated iatrogenic immunosuppressed patient.