Lichen sclerosus and atopy in boys: coincidence or correlation?

Background Lichen sclerosus (LS) is a sclerosing skin disease of presumably autoimmune origin affecting mainly the anogenital area. The aetiology is not completely understood. Comorbidity between genital LS and atopy in girls has been described but so far no controlled study has been performed. Objectives A prospective epidemiological case–control study was designed to clarify if there is comorbidity between genital LS and atopic skin diathesis (AD) in boys. Methods The study included a total of 92 boys aged between 1 and 17 years. The disease group consisted of 48 boys who underwent surgery for phimosis that was histologically confirmed as LS. The control group included 44 boys who were circumcised for phimosis for other medical reasons. Both groups were examined and the parents were interviewed following the criteria of the validated Diepgen atopy score. Patients with a score > 9 were assumed to have AD. Results Within the LS group (median age 8·7 years) 12 boys were diagnosed with AD (25%), while there were only three boys with AD (7%) in the control group (median age 5·3 years). The difference was significant using an age‐adjusted logistic regression (P < 0·05). Prior to our study nine boys of the LS group (19%) and four boys of the control group (9%) had already been diagnosed with AD. Conclusions We have demonstrated a significant comorbidity between LS and AD in boys. AD seems to be a priming precondition for the development of LS in boys.     

Spitz nevus and infliximab: association or coincidence?

Biological therapies, including anti-TNF agents, are important in the treatment of various chronic inflammatory diseases, including psoriasis, rheumatoid arthritis or inflammatory bowel disease. The increased use of these drugs translates into an increasing awareness of its adverse effects, which include malignancy. In this paper, we describe the case of a 28-year-old woman who developed a spitzoid melanocytic tumor after starting infliximab therapy for ulcerative colitis. The evidence for causality between anti-TNF and melanocytic proliferations is still sparse; nonetheless, treatment-associated immunosuppression seems to play a key role in this phenomenon. Therefore, a regular follow-up with a rigorous skin examination is essential in these patients. Noninvasive techniques such as dermoscopy or reflectance confocal microscopy are particularly useful diagnostic tools in these circumstances.     

The peripheral corneal melting syndrome and psoriasis: coincidence or association?

The peripheral corneal melting syndrome (PCMS) is a rare disease consisting of marginal corneal thinning that can progress to perforation. The PCMS carries a grave prognosis and it is of vital importance that dermatologists are aware that this may be responsible for a painful red eye in a patient with psoriasis. We highlight the features of the PCMS developing in an elderly woman with long-standing psoriasis to increase awareness of its significance, and hypothesize that an association may exist between the two conditions. Only one previous report has been published in which the authors speculate on the possible association of this syndrome with psoriasis. That few other cases have been described is either a consequence of under-reporting by both ophthalmologists and dermatologists unaware of a link or because the relationship between the syndrome and psoriasis is genuinely coincidental.     

Oral pemphigus vulgaris after anthrax vaccine administration: association or coincidence?

Pemphigus vulgaris is an autoimmune blistering disorder of the skin and mucous membranes. Numerous medications, ultraviolet light, and radiation have all been implicated in the etiology of the disease. We present a patient with pemphigus vulgaris whose disease developed after administration of anthrax vaccine. The histologic and immunofluorescence findings were characteristic of pemphigus vulgaris. Adverse systemic events associated with the anthrax vaccine consist primarily of flu-like symptoms. Previous cases of pemphigus vulgaris associated with anthrax vaccine administration have not been reported. Considering the recent deliberate outbreaks of anthrax and continued threats of bioterrorism, the potential exists for widespread administration of the anthrax vaccine. Accordingly, continued observation and documentation of true adverse events is needed.     

Dermatomyositis and pemphigus vulgaris: Association or coincidence?

A 76-year-old woman presented with a pruritic photodistributed rash and dysphagia. Serum anti-nuclear antibody was positive (titre 1/1280) and skin and muscle biopsies confirmed a diagnosis of dermatomyositis. She was treated with oral prednisolone (5-50 mg/day), mometasone furoate 0.1% ointment and lotion, and tacrolimus 0.03% ointment. Four years later she presented with multiple painful scaly erosions on the face, scalp and trunk. Histopathology and direct and indirect immunofluorescence confirmed a diagnosis of pemphigus vulgaris. Repeated malignancy screens were negative. She was treated with methotrexate (10 mg/week) and prednisolone (50 mg/day slowly tapered to 5 mg/day), with good control of both diseases.     

Concomitant psoriasis and bullous pemphigoid: coincidence or pathogenic relationship?

Psoriasis vulgaris and bullous pemphigoid represent two clinically well-characterized, chronic, inflammatory skin diseases. The concomitant occurrence of these two entities in a patient is rare, and the pathogenic implications of this phenomenon are unknown. We describe a 55-year-old woman with a 25-year history of plaque-type psoriasis who presented with disseminated tense bullae. Skin biopsies showed the typical histologic and immunohistochemical traits of bullous pemphigoid, and she had circulating immunoglobulin G (IgG) antibodies against the basement membrane zone, specifically the BP180 antigen. The bullous eruption was successfully treated with oral methylprednisolone and dapsone. Bullous pemphigoid is the autoimmune blistering disease that has most often been associated with psoriasis. Forty cases have been described in the literature. Classical psoriasis and psoriasis associated with bullous pemphigoid are identical. The pathogenic relationship between psoriasis and bullous pemphigoid is unclear. It has been postulated that the autoimmune process responsible for bullous pemphigoid lesions may be induced by ultraviolet light therapy, topical corticosteroids, and/or the inflammatory processes that occur in psoriasis. Immunomodulatory therapy may positively influence shared as well as distinct inflammatory mechanisms in patients who have psoriasis and bullous pemphigoid.     

Concurrence of systemic lupus erythematosus and pemphigus: coincidence or correlation?

BACKGROUND: Pemphigus and systemic lupus erythematosus (SLE) have previously been reported to coexist in the same patient. However, the relationship between the 2 diseases has not been elucidated. OBJECTIVE: This review was conducted to examine the relationship between pemphigus and SLE when they occur together in the same patient. METHODS: We conducted a retrospective review of the literature to identify previously reported cases of pemphigus and SLE coexisting in the same patient. The temporal relationship, clinical course, response to therapy and effects of 1 disease on the other were examined. RESULTS: Eight patients with a dual diagnosis of pemphigus and SLE have been previously reported. Most were female and non-Caucasian, with a mean age of 41 years. In the 8 patients reviewed here clinical outcomes, organ system involvement and demographic profiles are more typical of SLE. Seven of these 8 patients had pemphigus vulgaris, and 1 had pemphigus erythematosus. The limited follow-up did not permit studying issues of disease interaction. An additional 17 patients with pemphigus have been reported who have features suggestive of SLE. Organ system involvement in these patients was less typical of SLE. CONCLUSION: It appears that a true dual diagnosis of pemphigus and SLE is less common than suggested by the literature. Comparing patients with only pemphigus or only SLE to those with both may provide insights into genetic predisposition and pathogenesis, and provide an opportunity to study the effects of drugs that influence their course.     

Lichen planus or lichenoid tattoo reaction?

A 27-year-old white man with a tattoo of 3 months’ duration was referred for possible laser removal because a pruritic eruption had developed in the tattoo 1 month after placement. Physical examination revealed erythematous papules confined to the red areas of the tattoo ( Fig. 1 ). He also had flat-topped papules on the glans penis, which the patient stated had developed a few weeks after tattoo placement.

Figure 1 Open in figure viewer PowerPoint Tattoo before treatment, showing involvement of only the red areas     

Erythema elevatum diutinum and hypothyroidism: coincidence or causal relationship?

Erythema elevatum diutinum is a rare chronic leukocytoclastic vasculitis of unknown etiology. It is believed to be due to deposition of immune complexes in the vessels. Clinically it is manifested as erythematous violaceous papules and nodules, isolated or confluent with hardened consistency, symmetrical, usually located on the extensor surface of the extremities, particularly over the joints. Diagnosis is based on clinical and histological findings. We report the case of a woman, 71 years old, with erythematous violaceous nodules on the hands, elbows, back and legs, beginning two years ago, with pain and itching. Histopathological analysis revealed leukocytoclastic vasculitis, confirming the clinical suspicion. Laboratory tests revealed hypothyroidism. We report the case because of its rarity, with subsequent review of the literature.