Benign familial pemphigus—premenstrual exacerbation suppressed by goserelin and oophorectomy

We report the case of premenstrually exacerbated benign familial pemphigus which showed dramatic improvement with subcutaneous goserelin, a gonadotrophin-releasing hormone analogue. Total abdominal hysterectomy and bilateral salpingo-oophorectomy and oestrogen replacement therapy sustained the improvement.     

Chronic urticarial eruption with arthritis, papillary edema, and meningoencephalitis of childhood onset

The authors report on a case of chronic urticarial eruption since infancy in a man who is now 27-year-old; the association of numerous chronic symptoms, principally meningo-encephalitis, polyarthritis, hepato-spleno-adenomegaly allows the authors to compare this case to those described by Prieur and Griscelli in 1981 and 16 others cases found in literature. It appears as a "systemic disease" without biological or immunological markers; it might belong to the group of chronic normocomplementemic vasculitis.     

Patient-reported outcomes in urticarial vasculitis treated with omalizumab: case report

Background

Despite the current knowledge of UV, there is a lack of consensus among diagnostic criteria and management. In general, antihistamine therapy is regularly used for the symptomatic management of pruritus but does not control inflammation or alter the course of the disease. Monoclonal antibodies such as omalizumab (anti-IgE) have been proposed as a potential treatment for urticarial vasculitis. A few studies have reported the benefits of omalizumab in patient-reported outcome measures (PROMs). Herein we describe a female patient with urticarial vasculitis who was treated with omalizumab. We discuss the response to treatment and possible implications of PROMs in guiding the management of the disease.

Case presentation

We describe the case of a 57-year-old woman with a diagnosis of urticarial vasculitis. Due to lack of response to first-line treatment and the severity of the disease, treatment with omalizumab was initiated. Omalizumab 150 mg was administered every four weeks for three months. Second-generation antihistamines were used as needed. Both CU-Q2oL and UAS 7 improved. After three-month therapy with omalizumab, disease severity improved from moderate severity (UAS7?=?19) to well controlled (UAS7?=?6). However, 5 months after the last administration of omalizumab, the patient complained of worsening symptoms and active disease with quality of life impairment. A single dose of omalizumab (150 mg) was prescribed with corticosteroids. Thereafter, the patient presented a disease activity and quality of life with a fluctuating pattern that was controlled with additional doses of omalizumab.

Conclusion

In chronic urticaria, patient-reported outcome measures (PROMs) are important for assessing disease status and the impact of symptoms on patients’ lives. However, to our knowledge, there is no validated tool to measure such outcomes in UV patients. Although UAS7 and CU-Q2oL were not designed for UV assessment, they might be useful in the clinical setting as objective measures to determine treatment efficacy. However, some domains in the CU-Q2oL questionnaires do not correlate well with UAS7, which might serve as a relative indication to continue treatment despite disease severity improvement. Based on our observations, we believe omalizumab 150 mg might be a feasible therapeutic alternative when first-line treatment is unsuccessful.

     

Pruritic urticarial papules and plaques of pregnancy: polymorphic eruption of pregnancy (PUPPP)

The most commonly diagnosed pruritic dermatosis of pregnancy has a confusing nomenclature. The most commonly term used in the United States is pruritic urticarial papules and plaques of pregnancy (PUPPP), and in Great Britain, since 1982, polymorphic eruption of pregnancy. The etiology of PUPPP is still unknown. Placental products, hormonal alterations, and damage to connective tissue with subsequent conversion of nonantigenic molecules to antigenic ones, have been proposed as possible causes. The maternal and fetal prognosis is excellent. Treatment consists of topical or oral corticosteroids. Delivery does not usually provide relief of symptoms.     

Pustular eruption in a malaria patient treated with chloroquine

A 42-year-old Thai woman with Plasmodium vivax malaria was treated with a total dose of 1800 mg of chloroquine phosphate over 6 h. Acetaminophen was administered to reduce fever. The patient awoke the next morning with diffuse macular erythema, studded with 1–2 mm vesicles and pustules, predominantly on the face and the extremities ( Fig. 1 ). Some pustules were follicular. The mucous membranes, palms, and soles were unaffected. A gram stain of the pustule contents showed only scattered neutrophils. A skin biopsy from a lesion on the upper leg showed the features of a drug-induced pustular eruption: cell-poor subepidermal blisters with scattered subcorneal pustules ( Fig. 2 ), mild spongiosis, and a sparse infiltrate of neutrophils and eosinophils in the papillary dermis. ¹ A urine screen (Lignin test) for sulfonamides was negative, and high pressure liquid chromatography analysis of the serum showed no sulfadoxine. Hematolgy and biochemistry values, erythrocyte sedimentation rate, and glucose 6-phosphate dehydrogenase levels were normal. Serologies for mycoplasma antibody, Herpes simplex immunoglobulin M (IgM) and IgG, cytomegalovirus IgM, anti-human immunodeficiency virus, venereal disease research laboratory test, antinuclear antibody, alpha-fetoprotein, and carcino-embryonic antigen were negative. A hepatitis B panel, serum pregnancy test, and three sets of blood cultures were negative. A chest X-ray was normal. The patient was in otherwise good health and had no history of skin diseases. Chloroquine was discontinued and the eruption quickly resolved with mild desquamation.

Figure 1 Open in figure viewer PowerPoint Erythematous macules studded with small pustules (arrows) on the upper legs     

A child with CARD14-associated papulosquamous eruption (CAPE) successfully treated with ustekinumab

CARD14-associated papulosquamous eruption (CAPE) is a rare inflammatory skin eruption that can have features of psoriasis, pityriasis rubra pilaris, and erythroderma. This skin condition is known for its resistance to topical or conventional systemic therapies. Successful treatment of CAPE with anti-IL-12/IL-23 and IL-17 inhibitors has been reported. We present a case of a 2-year-old girl with CAPE who was successfully treated with ustekinumab.     

Pruritic urticarial papules and plaques of pregnancy (polymorphic eruption of pregnancy): two unusual cases

Summary We report two unusually florid cases of polymorphic eruption of pregnancy (PEP) or pruritic urticarial papules and plaques of pregnancy, the first occurring in a triplet pregnancy, and the second with some clinical features more suggestive of pemphigoid gestationis (PG). The first case responded to treatment with moderately potent topical steroids while the second case required a short course of systemic steroids. Our first case is the only reported case of PEP occurring during a triplet pregnancy. We discuss the effect of multiple pregnancy on PEP and some distinguishing factors between PEP and PG.     

Pemphigus foliaceus complicated by Kaposi varicelliform eruption and bilateral herpes simplex keratitis

Pemphigus foliaceus (PF) is a chronic autoimmune blistering dermatosis (ABD) that often involves the face, scalp and trunk. Kaposi's varicelliform eruption (KVE) is a viral vesicular dermatosis that rarely complicates ABD. In addition, KVE infrequently causes ocular involvement including herpes simplex keratitis (HSK). Timely diagnosis of KVE may be difficult in patients with PF due to their overlapping clinical features. In this report, we described a 79-year-old female with PF complicated by KVE and bilateral HSK. This report reminds physicians that KVE should be considered in patients with refractory PF and other ABD.     

Intravenous immunoglobulin-induced severe vesicular eczematous eruption successfully treated with narrow band-ultraviolet B therapy

Intravenous immunoglobulins (IVIg) are increasingly being used to treat a wide spectrum of dermatological and neurological autoimmune diseases. Although the administration of IVIg does not usually result in severe adverse reactions, side effects of IVIg reportedly occur in 6-13% of patients. Most reported cases were not severe, and IVIg is considered a relatively safe drug. Some reports described a vesicular eczematous eruption caused by IVIg that was cured by applying topical steroid ointments or systemic steroids. Herein, we present, to the best of our knowledge, the first case of severe vesicular eczematous eruption all over the body induced by IVIg that was unresponsive to topical steroid ointment and was subsequently treated with narrow band-ultraviolet B (NB-UVB) therapy successfully. NB-UVB was started at a dose of 400 mJ/cm² once a week, and swift improvement was observed. The skin rash disappeared in the first 2 months, and the pathogenesis of IVIg-induced eczematous eruption remains unelucidated. No change in eosinophils and complement levels were observed in our case. Given the increase in the widespread use of IVIg, we have shown that NB-UVB therapy is a candidate choice for the treatment of IVIg-induced severe vesicular eczematous eruption.