|Year : 2017 | Volume
| Issue : 1 | Page : 19-21
Pemphigus foliaceus: A rare case of exfoliative dermatitis
Vaishnavi Gopal, Malcolm Pinto, Manjunath Shenoy Mala
Department of Dermatology, Venereology and Leprosy, Yenepoya Medical College, Yenepoya University, Mangalore, Karnataka, India
|Date of Web Publication||28-Dec-2016|
Manjunath Shenoy Mala
Department of Dermatology, Venereology and Leprosy, Yenepoya Medical College, Yenepoya University, Deralakatte, Mangalore - 575 018, Karnataka
Source of Support: None, Conflict of Interest: None
Exfoliative dermatitis (ED) is a dermatological condition necessitating admission as it can sometimes be fatal because of its metabolic burden and complications. Papulosquamous disorders and drug reactions comprise over 75% of all the causes of ED. One must be vigilant for the rarer causes of ED as treatment protocols vary and prompt institution of treatment is lifesaving. We present a case of a 56-year-old man with ED who had a history of developing recurrent crusted lesions over the scalp and trunk over the last 3 years. He was treated with oral steroids which he stopped abruptly 6 months ago. Then, he applied topical herbal medications over the lesions before the skin disease progressed to the present state of ED. We uncovered his old records where the histopathology and direct immunofluorescence studies aided in the diagnosis. Based on the history, examination findings supported by histopathology and immunofluorescence studies we came to a final diagnosis of ED secondary to pemphigus foliaceus. This case has been reported for the rare presentation of a common immunobullous disorder and to highlight its diagnostic difficulties.
Keywords: Direct immunofluorescence, exfoliative dermatitis, immunofluorescence of hair follicle, pemphigus foliaceus
|How to cite this article:|
Gopal V, Pinto M, Mala MS. Pemphigus foliaceus: A rare case of exfoliative dermatitis. Clin Dermatol Rev 2017;1:19-21
| Introduction|| |
Erythroderma is a cutaneous disorder characterized by erythema and scaling involving more than 90% of the body surface area. There are multiple causes that can lead to its presentation necessitating an extensive evaluation and workup. Pemphigus is a group of immunobullous disorders characterized by recalcitrant vesiculation of skin. They are caused by auto antibodies directed against the desmosomal proteins - desmoglein 1 and desmoglein 3 resulting in loss of keratinocyte adhesion leading to a typical intra-epidermal acantholytic blister that is evident on histology. Pemphigus foliaceus (PF) rarely presents with oral ulcers and runs a comparatively benign course than pemphigus vulgaris. Erythroderma secondary to PF is rarely reported and challenges its diagnosis and management.
| Case Report|| |
A 56-year-old man presented with generalized erythema and scaling of 15 days duration. He gave history of recurrent crusted lesions over the scalp which progressed to involve the trunk, for the past 3 years. He was initiated on oral steroids by a dermatologist which he discontinued 2 months ago. This was followed by the appearance of fresh blisters after a period of 2 weeks. He later applied topical herbal medications over these areas as advised by a local practitioner, but the lesions exacerbated to the present state. There was no history of oral or genital lesions. Cutaneous examination revealed generalized erythema with overlying scales and moist crusts involving over 90% of the body surface area [Figure 1]. There were few areas of sparing over the legs. Nikolsky sign was positive. Histopathology revealed a subcorneal bulla [Figure 2] with few acantholytic cells. DIF revealed intercellular deposits of IgG in the epidermis and the inner aspect of outer root sheath (ORS) of the intact hair follicle [Figure 3] suggesting an autoimmune intra-epidermal blistering disease. Hence, a final diagnosis of exfoliative dermatitis (ED) secondary to PF was confirmed.
|Figure 2: Histopathology of lesional skin showing subcorneal bulla with few acantholytic cells (H and E, ×100)|
Click here to view
|Figure 3: Direct immunofluorescence of hair showing fluorescence pattern in the innermost layers of the outer root sheath|
Click here to view
| Discussion|| |
Managing a case of ED is directed towards the general management of metabolic derangements and also treating the inciting cause. ED is a common entity necessitating admissions for the workup of the underlying condition as it is usually not a straight forward one. ED can be fatal, even when properly managed, primarily because of its metabolic consequences and complications. Hence, it is imperative to establish its etiopathology to initiate precise management and to determine the disease prognosis. 
Eczemas, papulosquamous disorders such as psoriasis and drug reactions comprise over 75% of all the causes of ED. Among the rarer causes include Sezary syndrome and autoimmune bullous diseases such as PF.  The course of the disease is greatly influenced by etiology. It is progressive when the cause is attributed to drug allergy, lymphoma, leukemia, contact allergens, or staphylococcal scalded skin syndrome. ,,, A slower course is observed if caused by the primary skin diseases such as psoriasis or atopic dermatitis. ,,, The prevalence of PF as a cause of erythroderma is only 0.5%. It is usually preceded by moist, crusted lesions on the face, and upper trunk before the development of erythroderma. Scaling is conspicuous, moist, and adherent. Crops of thin-walled bullae may erupt, especially on the limbs. 
Generalized erythroderma has been reported as a result of exacerbation of this disease and it may occur due to triggering factors such as ultraviolet exposure, drugs, and various infections.  Our patient had abruptly stopped oral steroids by himself, and the underlying inflammation may have been aggravated by applying topical irritant herbal medications.
ED secondary to a suspected blistering disorder such as PF can be diagnosed based on histopathology. The earliest change is acantholysis in the upper epidermis, within or adjacent to the granular layer, leading to a subcorneal bulla as seen in [Figure 2].
The gold standard for diagnosis is DIF of perilesional skin. In PF, the IgG immunostaining on the epithelial cell surfaces can be granular and/or linear, resulting in a chicken wire pattern. The ORS of hair follicle is a functional stratified squamous epithelium structurally analogous to epidermis. Many studies have shown the pemphigus-specific immunofluorescence pattern seen in skin, is found in ORS of plucked hair follicle, even without scalp involvement. Desmoglein 1 is expressed in the inner root sheath and innermost layers of ORS, whereas desmoglein 3 is expressed throughout the ORS. [Figure 3] shows DIF of skin specimen with an intact hair follicle where we can see the above-mentioned fluorescence pattern in the innermost layers of the ORS signifying presence of desmoglein 1 as the antigen. Telogen hair can be collected for DIF as it comes off easily and preferred over anagen hair as it has abundance of desmoglein 3. 
The advantages of doing DIF of hair alone are that it can replace invasive, painful skin, and oral biopsies. DIF of the skin is better than indirect immunofluorescence to measure disease activity. 
Once diagnosed, treatment in majority of cases includes topical and systemic corticosteroids as well as other immunosuppressant drugs. He was managed with systemic corticosteroids initially and later with the addition of azathioprine as a steroid-sparing agent. He responded although very slowly and complete remission was attained in 8 months.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Okoduwa C, Lambert WC, Schwartz RA, Kubeyinje E, Eitokpah A, Sinha S, et al.
Erythroderma: Review of a potentially life-threatening dermatosis. Indian J Dermatol 2009;54:1-6.
Berth-Jones J. Eczema, lichenification, prurigo and erythroderma. In: Burns T, Breathnachs S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8 th
ed. Oxford: Wiley-Blackwell; 2010. p.23.46-50.
Sehgal VN, Srivastava G. Exfoliative dermatitis. A prospective study of 80 patients. Dermatologica 1986;173:278-84.
Rothe MJ, Bialy TL, Grant-Kels JM. Erythroderma. Dermatol Clin 2000;18:405-15.
Sigurdsson V, Toonstra J, Hezemans-Boer M, van Vloten WA. Erythroderma. A clinical and follow-up study of 102 patients, with special emphasis on survival. J Am Acad Dermatol 1996;35:53-7.
Breathnach SM. Management of drug eruptions: Part II. Diagnosis and treatment. Australas J Dermatol 1995;36:187-91.
Fariba G, Ayatollahi A, Hejazi S. Pemphigus foliaceus. Indian Pediatr 2012;49:240-1.
Kumaresan M, Rai R, Sandhya V. Immunofluorescence of the outer root sheath: An aid to diagnosis in pemphigus. Clin Exp Dermatol 2011;36:298-301.
Tanasilovic S, Medenica L, Popadic S. Direct immunofluorescence of the outer root sheath in anagen and telogen hair in pemphigus vulgaris and pemphigus foliaceus. Australas J Dermatol 2014;55:e74-6.
[Figure 1], [Figure 2], [Figure 3]