++
++
There are a number of bullous diseases other than pemphigus and bullous pemphigoid that are important to recognize. Porphyria cutanea tarda is a disease that has no extracutaneous manifestations (Figures 194-1, 194-2, 194-3). Dystrophic epidermolysis bullosa belongs to a family of inherited diseases where blister formation can be caused by even minor skin trauma due to mechanical fragility. PLEVA (pityriasis lichenoides et varioliformis acuta) is a minor cutaneous lymphoid dyscrasia that can appear suddenly and persist for weeks to months. Dermatitis herpetiformis is a recurrent eruption that is usually associated with gluten-induced enteropathy.
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PORPHYRIA CUTANEA TARDA
++
A middle-aged woman presented with tense blisters on the dorsum of her hand (Figure 194-1). One bulla was intact, and the others had ruptured, showing erosions. Work-up showed elevated porphyrins in the urine (which fluoresced orange-red under a Wood lamp) and the patient was diagnosed with porphyria cutanea tarda.
++
Porphyria cutanea tarda (PCT) is the most common type of porphyria disorder.1
PCT occurs mostly in middle-aged adults (typically 30 to 50 years of age) and is rare in children.
It may occur in women on oral contraceptives and in men on estrogen therapy for prostate cancer.2,3
Alcohol, pesticides, and chloroquine have been implicated as chemicals that induce PCT.1,3
PCT is equally common in both genders.
There is an increased incidence of PCT in persons with hepatitis C4-6 and HIV7 (see Figure 194-2).
+++
ETIOLOGY AND PATHOPHYSIOLOGY
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The porphyrias are a family of illnesses caused by various metabolic derangements in the metabolism of porphyrin, the chemical backbone of hemoglobin. Whereas the other porphyrias (acute intermittent porphyria and variegate porphyria) are associated with well-known systemic manifestations (abdominal pain, peripheral neuropathy, and pulmonary complications), PCT has no extracutaneous manifestations. Photosensitivity is seen (as with variegate porphyria). PCT is associated with a reduction in hepatic uroporphyrinogen decarboxylase (fifth enzyme in hemoglobin synthesis).
++
Hepatitis C.
Alcohol-induced liver injury.
Hemochromatosis3 (iron overload)—50% of cases of PCT are associated with the same gene mutation for hemochromatosis.3
Use of estrogens.
Cigarette smoking.
Patients on dialysis.
Exposure to pesticides (halogenated hydrocarbons).
HIV infection.7
++
The classic presentation is that of blistering (vesicles and tense bullae) on photosensitive "fragile skin" (similar to epidermolysis bullosa). Scleroderma-like heliotrope suffusion of the eyelids and face may be seen. As the blisters heal, the skin takes on an atrophic, scarlike appearance. Excoriations, erosions, and milia can be seen in sun exposure areas, mainly the dorsa of the hands. Hypertrichosis (especially on the cheeks and temples) (see Figure 194-3) may occur and even be the presenting feature.
++
Classically, the dorsa of the hands are affected (see Figures 194-1 and 194-2).
++
The diagnosis can be confirmed by the orange-red fluorescence of the urine when examined under a Wood lamp. Urine also can turn red to brown after exposure to sunlight for several hours. Increased plasma iron may be seen (associated with increased hepatic iron in the Kupffer cells). Diabetes is said to occur in 25% of individuals.
++
Twenty-four-hour urine collection for porphyrins—These will be elevated in PCT.
Skin biopsy may help confirm PCT if the other information is not clear.
Once the diagnosis is made, secondary causes of PCT should be investigated:
Serum for ferritin, iron, and iron-binding capacity to look for hemochromatosis.
Order liver function tests, and if abnormal order tests for hepatitis B and C.
Consider α-fetoprotein and liver ultrasound if considering cirrhosis and/or hepatocellular carcinoma.
Order an HIV test.
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DIFFERENTIAL DIAGNOSIS
++
The acral vesiculobullous lesions may suggest nummular or dyshidrotic eczema. In younger individuals, the acral blistering may suggest epidermolysis bullosa. The lesions may also suggest erythema multiforme bullosum. The heliotrope suffusion may suggest dermatomyositis, and the atrophic changes may suggest systemic sclerosis.
Polymorphous light eruption; you also can see papules, plaques, vesicles in sun-exposed areas.
Bullous drug eruptions; check for drug exposure.
++
If the onset is associated with alcohol ingestion, estrogen therapy, or exposure to pesticides, reducing exposure is warranted.1,3
Phlebotomy of 450–500 mL of blood biweekly until the hemoglobin is decreased to 11 g or serum ferritin below 20 ng/mL is associated with biochemical and clinical remission within a year.8
Low-dose hydroxychloroquine or chloroquine can help maintain remissions. Standard dose of hydroxychloroquine is 100–200 mg and chloroquine is 125 mg two times per week for at least 6–12 months,9 whereas high-dose chloroquine can exacerbate the illness2 due to hepatotoxicity.10
Sun protection clothing, regular use of broad-spectrum sunscreens.
Treatment of hepatitis C has shown resolution of PCT.5,6
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++
Avoidance of potential precipitants (alcohol, estrogens, pesticides, cigarette smoking) and avoidance of excess sunlight exposure (to avoid hypersensitivity) are important. Avoidance of trauma and careful wound care is also necessary.
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EPIDERMOLYSIS BULLOSA
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A 34-year-old pregnant woman presents with active blistering in her axilla, and past history revealed that she lost her fingernails and toenails (Figure 194-4A) as a young child. She was diagnosed as a child with recessive dystrophic epidermolysis bullosa. None of her children had been affected because her husband was neither affected nor a carrier (Figure 194-4B). A topical steroid ointment helped relieve the pain and calm the blistering in her axilla.
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Dystrophic epidermolysis bullosa belongs to a family of inherited diseases characterized by skin fragility and blister formation caused by minor skin trauma11 due to mutations of the gene COL7A1, which encodes type VII collagen. The defect is located in the basal membrane zone (BMZ), altering the function, adhesion and integrity of the basal membrane of the skin and mucosa.12 There are autosomal recessive, autosomal dominant, and sporadic types. The severity of this disease may vary widely, from mild skin blisters to gastrointestinal, cardiac, or ocular involvement, as well as scarring, milia, and deformities. Onset is in childhood, and in later years severe dystrophic deformities of hands and feet are characteristic, affecting quality of life13 (Figure 194-5). Malignant degeneration, especially squamous cell carcinoma, is common in sun-exposed areas.14
++
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ETIOLOGY AND PATHOPHYSIOLOGY
++
Dystrophic epidermolysis bullosa has vesiculobullous skin separation occurring at the sub-basal lamina level, as opposed to junctional epidermolysis bullosa, which blisters at the intralamina lucida layer; epidermolysis bullosa simplex (Figure 194-6), which blisters at the intraepidermal layer; and Kindler syndrome, in which blisters are in multiple layers.15
++
++
Acral skin fragility and blistering are the hallmark in childhood. Minor trauma can induce severe blistering. As the disease progresses initially, painful and ultimately debilitating dystrophic deformities such as joint and tendon contractures, pseudosyndactyly, and dystrophic nails are typical. Repeated blistering of the hands can lead to fusion of the fingers and the "mitten" deformity (see Figure 194-5). Other clinical features include oral blisters, oral fibrosis, ankyloglossia (fusion of the tongue with mouth floor), esophageal erosions, and strictures.16-18
++
The typical distribution is acral (hands and feet), although blistering may extend proximally secondary to trauma. Other systems involved include gastrointestinal, commonly esophagus, small intestine; ocular with corneal abrasions; dilated cardiomyopathy; genitourinary stenosis; and osteoporosis.16-19
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LABORATORY STUDIES AND BIOPSY
++
There are no laboratory tests to confirm the diagnosis. A punch biopsy can provide adequate tissue for the dermatopathologist to differentiate among the different forms of epidermolysis bullosa—simplex, junctional, dystrophic, and Kindler syndrome—via transmission electron microscopy (TEM), immunofluorescence antigen mapping (IFM), or genetic testing.17
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DIFFERENTIAL DIAGNOSIS
++
Erythema multiforme bullosum may have a similar appearance, but the distribution is less apt to be limited to the distal extremities.
The appearance of an acral blistering on fragile skin is also characteristic of PCT, but the age of onset of PCT is typically in middle age and not in childhood.
The first appearance of the condition may be confused, with staphylococcal scalded skin syndrome (see Chapter 122, Impetigo).20
++
Management is primarily prevention of trauma, careful wound care, and treatment of complicating infections. Other supportive measures such as pain management, nutritional support, and electrolyte balance are often necessary with a multidisciplinary team.21
++
Gentamicin (topical or intralesional) is a promising therapy in the treatment of recessive dystrophic epidermolysis bullosa that induces production of type VII collagen, accelerating wound healing and reducing blistering formation.22
Occupational therapy may help with contractures. In some cases, surgical release of fingers/toes is necessary.17
Screening the skin for squamous cell carcinoma is important in the dystrophic form.17
Cyclosporine and mycophenolate mofetil have shown benefit as long-term therapy.23
Bone marrow transplantation is currently under clinical investigation, and preliminary results are promising.24
++
Periodic skin examinations should be done to help manage symptoms and screen for malignancy.
++
Avoid trauma, and come in early if there are any signs of infection or malignancy.
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PITYRIASIS LICHENOIDES ET VARIOLIFORMIS ACUTA (PLEVA)
++
A 22-year-old man presented with a varicelliform eruption that he has had for 6 weeks (Figure 194-7). Initially, he was diagnosed with varicella and given a course of acyclovir. Then he was misdiagnosed with scabies and treated with permethrin. A correct diagnosis of PLEVA was made by clinical appearance and confirmed with biopsy. His skin lesions cleared with oral tetracycline.
++
++
PLEVA or Mucha-Habermann disease and pityriasis lichenoides chronica are maculopapular erythematous eruptions that can occur in crops of vesicles that can become hemorrhagic over a course of weeks to months, leaving smallpox-like scars (Figures 194-7 and 194-8).25
It occurs predominately in a pediatric population, median 8 years.26
PLEVA occurs in preschool and preadolescent children as well.27
There is a predilection for males in the second and third decades.
+++
ETIOLOGY AND PATHOPHYSIOLOGY
++
PLEVA has traditionally been classified as a benign papulosquamous disease. However, there is increasing evidence that suggests that PLEVA should be considered a form of cutaneous lymphoid dysregulation of T cells.28 It may even evolve into an indolent form of mycosis fungoides (see Chapter 180, Cutaneous T-Cell Lymphoma).29
Cases of PLEVA associated with human herpesvirus 7 (HHV-7) and human herpesvirus 8 (HHV-8), Epstein-Barr virus (EBV), and parvovirus B19 have been reported. This suggests that it could be an inflammatory condition due to dysregulation of T cells rather than a lymphoproliferative disorder.30,31
++
PLEVA occurs with crops of maculopapular and papulosquamous lesions that can vesiculate and form hemorrhagic vesicles (see Figures 194-7 and 194-8). Although it resembles varicella, with multiple polymorphic lesions, new crops of lesions continue to appear over weeks and months. It can be thought of as "chickenpox that lasts for weeks to months."
++
++
Lesions typically occur over the anterior trunk and flexural aspects of the proximal extremities. The face is spared.
++
There are no specific laboratory tests for PLEVA except biopsy.
++
Dermoscopy findings have been described but do not replace biopsy for diagnosis.32
++
A punch biopsy is helpful in making the diagnosis. It may be necessary to differentiate PLEVA from lymphomatoid papulosis (see "Differential Diagnosis" below).
+++
DIFFERENTIAL DIAGNOSIS
++
Varicella—A varicella direct fluorescent antibody test can confirm acute varicella. If no viral testing was done and what appeared to be varicella persists, PLEVA should be considered (Chapter 129, Chickenpox).
Secondary syphilis—Direct fluoresce antibody, PCR and serology test help to establish the diagnosis (Chapter 225, Syphilis).
Pityriasis lichenoides chronica is the chronic form of PLEVA and can be distinguished from PLEVA by length of time and biopsy (Figure 194-9). It has a more low-grade clinical course than PLEVA, and the lesions appear over a longer course of time with relapse and remission periods.
Erythema multiforme (Chapter 185) is a hypersensitivity syndrome with abrupt onset in which symmetric target lesions are seen. The target lesions have epidermal disruption in the center with vesicles and/or erosions. Look for the target lesions to help differentiate this from PLEVA.
Lymphomatoid papulosis presents in a manner similar to PLEVA with recurrent crops of pruritic papules at different stages of development that appear on the trunk and extremities. Usually it is chronic and self-limited. Although it has histologic features that suggest lymphoma, lymphomatoid papulosis (LP) alone is not fatal. It is important to differentiate LP from PLEVA, because patients with LP need to be worked up for coexisting malignancy. Patients with LP tend to be older, and a punch biopsy can make the diagnosis.
Gianotti-Crosti syndrome (papular acrodermatitis of childhood) may resemble PLEVA, but the lesions are usually acral in distribution (Figure 194-10).5 The erythematous papules and vesicles are found on the extremities and sometimes on the face. It is a benign syndrome associated with many childhood viruses that may last 2 to 8 weeks.
Bullous arthropod reactions can be distinguished from PLEVA with a good clinical history and physical exam.
++
++
++
Oral macrolides are first-line treatment.33 Oral erythromycin 30–50 mg /kg/day is given in divided dosing 3–4 times per day for 1–4 months.34
Doxycycline 100 mg twice daily is an alternative. The efficacy of macrolides and tetracyclines is probably from their anti-inflammatory properties rather than their antibacterial effects.
Narrowband UVB may be used 2–3 times per week.33
Topical steroids such as triamcinolone 0.1% twice daily may decrease the itching.
++
UVA1 phototherapy has been used with some success.35
Recalcitrant cases or ulceronecrotic PLEVA can be treated with tumor necrosis factor (TNF)-α inhibitors, intravenous immunoglobulin, or methotrexate.36,37
++
Long term follow-up is recommended due to small risk of T-cell malignant transformation.33
++
PLEVA is usually a self-limited disease. There are treatments that can help the symptoms and may shorten the illness.
+++
DERMATITIS HERPETIFORMIS
++
A young man with a past history of diarrhea and malabsorption carries a past diagnosis of gluten-induced enteropathy. Despite a gluten-free diet, he continues to have a pruritic eruption on his elbows, knees, abdomen, and buttocks (Figures 194-11 and 194-12). Although the most likely diagnosis is dermatitis herpetiformis, a punch biopsy was performed to confirm this before starting the patient on oral dapsone.
++
++
++
Dermatitis herpetiformis is a chronic recurrent symmetric vesicular eruption that is usually associated with diet-related enteropathy, more specifically gluten hypersensitivity. It is a specific extraintestinal manifestation of gluten enteropathy.38 It most commonly occurs in the 20- to 40-year-old age group. Men are affected more often than women. Usually more common in Caucasians. In 80% to 90% of cases there is a strong association with genotype HLA DQ2, HLA DQ8, and HLA DR3.38,39
+++
ETIOLOGY AND PATHOPHYSIOLOGY
++
The disease is related to gluten and other diet-related antigens that cause the development of circulating immune complexes and their subsequent deposition in the skin. The term herpetiformis refers to the grouped vesicles that appear on extensor aspects of the extremities and trunk; the disease is not a viral infection or related to the herpes viruses. It is characterized by the deposition of granular immunoglobulin (Ig) A along the tips of the dermal papillae. The majority of patients will also have blunting and flattening of jejunal villi, which leads to diarrhea even to the point of steatorrhea and malabsorption.
++
The clinical eruption is characterized by severe itching, burning, or stinging in the characteristic extensor distribution (knees, elbows, back, buttocks, and the extensor aspect of the forearms). Herpetiform vesicles, papules, and urticarial plaques may be seen. Because of the intense pruritus, characteristic lesions may be excoriated beyond recognition (see Figures 194-11 and 194-12). The condition has relapses and remissions that are related to the gluten-restricted diet.
++
Classically, the lesions (or excoriations) are seen in the extensor aspects of the extremities, shoulders (see Figure 194-11), lower back, and buttocks (see Figure 194-12).
++
If the patient has gluten-induced enteropathy, antigliadin and antiendomysial antibodies may be present in 80% of the cases of dermatitis herpetiformis. A blood test for antigliadin antibody is a sensitive test for gluten-induced enteropathy.
++
Diagnosis is confirmed by a punch biopsy. It is best to biopsy new crops of lesions or intact vesicles. A standard histologic examination will show microabscesses of neutrophils in the dermal papillae, subepidermal vesicles, perivascular lymphocytic infiltrate, and few eosinophils. Direct immunofluorescence reveals granular deposits of IgA and complement within the dermal papillae, with almost 100% specificity and sensitivity.38
+++
DIFFERENTIAL DIAGNOSIS
++
Scabies may have a similar appearance with pruritus, papules, and vesicles. If the lesions and distribution suggest scabies, it should be ruled out with skin scraping looking for the mite, feces, and eggs. If the scraping is negative, but the clinical appearance suggests scabies, empiric treatment with permethrin should be considered as well. If the lesions persist, consider a punch biopsy to look for dermatitis herpetiformis (Chapter 149, Scabies).
Nummular and dyshidrotic eczema may also be diagnostic considerations, but response to steroids in eczema may be helpful in differentiation (Chapters 151, Atopic Dermatitis, and 153, Hand Eczema).
The classic differential for PCT is pseudoporphyria (caused by nonsteroidal anti-inflammatory drugs [NSAIDs] such as naproxen), epidermolysis bullosa acquisita, and variegate porphyria.
IgA bullous dermatosis: differs from dermatitis herpetiformis because in IgA bullous dermatosis the deposit of IgA is linear instead of the granular pattern seen in DH and often is caused by drugs.
Bullous arthropod bites; patient may recall insect exposure.
++
With a gluten-free diet, 80% of patients will show improvement in the skin lesions (see Figure 194-12). The degree of benefit is dependent on the strictness of the diet.38,39
A gluten-free diet may help the enteropathy and decrease the subsequent development of small bowel lymphoma.38
Dapsone at an initial dose of 100–200 mg daily or 0.5–1 mg/kg/day with gradual reduction to a 25- to 50-mg maintenance level may be necessary indefinitely.38 Monitor complete blood count (CBC), liver function test due to risk of hemolysis, methemoglobinemia. Some clinicians, recommend checking glucose-6-phosphate dehydrogenase (G6PD) activity prior to start of treatment.
Rituximab has been used in recalcitrant cases with good outcome, serologic and immunologic remission.40
++
Follow-up is needed to control the disease and monitor nutritional status.
++
Nutritional counseling is important for all patients with gluten-induced enteropathy. Persons with dermatitis herpetiformis and gluten-induced, enteropathy should not eat wheat and barley but can eat rice, oats, and corn. Patients can get information, dietary guidelines, and educational materials online via support groups such as https://www.gluten.org and https://www.celiac.org.
++
++
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