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Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 226-228

Pemphigus and beyond

Department of Dermatology, Venereology and Leprology, K. J. Somaiya Medical College, Mumbai, Maharashtra, India

Date of Submission24-Apr-2020
Date of Decision22-Sep-2020
Date of Acceptance06-Oct-2020
Date of Web Publication26-Aug-2021

Correspondence Address:
Kavya Baddireddy
5, Ocean Drive Layout, Gitam Post, Visakhapatnam - 530 045, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/CDR.CDR_72_20

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A 23-year-old male diagnosed with pemphigus foliaceous presented with multiple asymptomatic hyperpigmented patches and plaques clinically resembling seborrheic keratoses over multiple sites on the body of 1-year duration. There were no fluid-filled lesions, active erosions, or mucosal involvement. Histopathological examination from the hyperpigmented plaque revealed marked acanthosis with intra-epidermal acantholysis. Enzyme linked immunosorbent assay (ELISA) for antidesmoglein-1 antibodies was positive with a titer of 157 units/ml. Treatment with oral azathioprine, dapsone, and topical halobetasol propionate produced a marked response with complete clearance of the lesions. A thorough workup helped us detect the persistence of disease activity and thus prevent a potential relapse. This case report highlights the unusual presentation of a common immunobullous disorder, Pemphigus acanthomata a rare entity with regard to its morphological, histopathological, and immunological features.

Keywords: Acantholysis, pemphigus acanthomata, seborrheic keratoses

How to cite this article:
Poojary S, Baddireddy K. Pemphigus and beyond. Clin Dermatol Rev 2021;5:226-8

How to cite this URL:
Poojary S, Baddireddy K. Pemphigus and beyond. Clin Dermatol Rev [serial online] 2021 [cited 2021 Dec 1];5:226-8. Available from: https://www.cdriadvlkn.org/text.asp?2021/5/2/226/324573

  Introduction Top

Pemphigus foliaceous is a chronic immunobullous disorder, which in its usual form presents as flaccid blisters and crusted erosions. Herein, we discuss an interesting presentation of this disorder where seborrheic keratoses like plaques developed in old healed lesions with no other signs of pemphigus foliaceous. These plaques served as a clinical marker and facilitated the detection of underlying disease activity.

  Case Report Top

A 23-year-old male, diagnosed with pemphigus foliaceous 11 years back presented with multiple asymptomatic dark-colored, flat, and elevated lesions affecting different regions of the body for the last 1 year. During the last several years, he had multiple episodes of clear fluid-filled lesions that ruptured easily leaving behind small painful erosions. He was earlier treated at other hospitals with oral and intravenous steroids, cyclophosphamide pulse, and dapsone. There was only partial response with each remission lasting for 3 to 6 months and the lesions healed with hyperpigmentation. However, since the last episode, 1 year back, he noticed the development of verrucous plaques over the previously healed hyperpigmented patches at few areas. There was no history of scarring, mucosal involvement, photoexacerbation, or koebnerization.

There were no systemic complaints.

The general physical examination was normal. Cutaneous examination revealed confluent hyperpigmented macules and patches, (1–5 cm) present over the chest, abdomen, back, both the upper and lower limbs. In addition, hyperpigmented seborrheic keratoses like verrucous papules/plaques were present over the hyperpigmented patches at few areas [Figure 1]a and [Figure 1]b. These plaques could not be scraped off. There were no active erosions. Nikolskiy's sign was negative. Laboratory investigations including complete blood counts, biochemistry panel, serum cortisol, and Vitamin B 12 levels were within normal limits. Histopathology of the verrucous plaque showed hyperkeratosis, acanthosis, papillomatosis, and focal subcorneal blister formation with acantholytic cells, suggestive of postpemphigus acanthoma [Figure 2]a and [Figure 2]b. Skin biopsy of the hyperpigmented macule revealed superficial dermal perivascular mononuclear cell infiltrate admixed with numerous melanophages, consistent with postinflammatory pigmentation. ELISA for antidesmoglein-1 (dsg-1) antibodies was positive with a titer of 157 units/ml (normal range: up to 20 units/ml) Intermediate result was obtained for anti-dsg-3 antibody with a titer of 10.8 U/ml (normal range <9 units/ml). Thus, the diagnosis of postpemphigus acanthoma was confirmed, following a collective interpretation of the clinical, histopathological, and immunological findings. The patient was treated with T. azathioprine 50 mg BD and T. dapsone 100 mg OD. Topical halobetasol propionate 0.05% ointment was advised for application over the verrucous lesions. There was complete clearance of lesions on the chest and a marked decrease in lesions on the back within 3 months of commencement of treatment at our hospital [Figure 3]. There was also no recurrence of fluid-filled lesions.
Figure 1: (a and b) Multiple verrucous plaques (seborrheic keratoses like) (1–2 cm in diameter) on a background of multiple hyperpigmented macules seen on the chest, abdomen, and back

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Figure 2: (a) Epidermis: Marked hyperkeratosis, acanthosis, papillomatosis, subcorneal cleft with acantholytic cells (arrow). Few acantholytic cells are present in the mid epidermis. Dermis shows superficial perivascular infiltrate and melanophages (H and E, ×10). (b) Acantholytic cells in higher magnification (×40)

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Figure 3: (a) Complete clearance of lesions on the chest. (b) Marked decrease in lesions on the back within 3 months after treatment

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  Discussion Top

Pemphigus foliaceous is a chronic, benign, autoimmune intraepidermal blistering disorder that is characterized clinically by fragile superficial vesicles, scaly crusted erosions in seborrheic distribution, histologically by subcorneal blisters, and immunopathologically by immunoglobulin G (IgG) antibodies to Dsg-1.[1] The idiopathic and endemic types are the two predominant clinical variants.[1] Idiopathic pemphigus foliaceous occurs sporadically in middle aged and elderly, while the endemic variant (fogo selvagem) found along the river banks of Brazil, Columbia, and Peru primarily affects children and young adults and is transmitted by the bite of a black fly, Simulium nigrimanum,[2] other rarer variants include drug-induced pemphigus foliaceous[3] and pemphigus erythematosus (Senear-Usher syndrome) which shows combined immunological features of pemphigus foliaceous and lupus erythematosus, characterized by tiny flaccid vesicles in photodistribution and antinuclear antibody positivity.[4] Chatterjee et al. have reported a case masquerading as IgA pemphigus where the diagnosis was clinched based on the typical immunological findings.[5] Epitope spreading as a result of the biochemical similarity between the Dsg 1 and Dsg 3 can very rarely result in the transition of confirmed cases of pemphigus vulgaris to foliaceous and vice versa, the former being more common.[6],[7] In its most severe form, as reported by Gopal et al., pemphigus foliaceous can present as erythroderma requiring prompt hospitalization to prevent serious and sometimes fatal complications from metabolic instability.[8] Inaoki et al. reported a case in which pemphigus foliaceus developed after cutaneous squamous cell carcinoma (SCC) metastasized to regional lymph nodes. Immunologic analysis in this patient revealed that SCC expressed dsg-1 suggesting a possible pathogenic mechanism.[9]

Hyperpigmented verrucous, seborrheic keratoses like lesions, i.e., pemphigus acanthomata are rare in pemphigus.[10],[11] Such acanthomatous lesions were first reported in 1956 by Williams in post eczematous conditions (Acanthoma of Murray Williams or Murray Williams warts).[12] Similar lesions were later described in pemphigus foliaceous and pemphigus vulgaris by Yesudian et al.; hence, they were termed as postpemphigus acanthomata. The term postpemphigus acanthoma describes the development of seborrheic keratoses like verrucous lesions at sites of previously healed blisters during remission.[13] The epidermal hyperproliferation is possibly induced by a unique subset of autoantibodies released by colonizing bacteria.[14] These lesions are commonly seen on the trunk and this distribution differentiates it from pemphigus vegetans wherein the large vegetating masses are seen in the flexural areas. Histopathologically, hyperkeratosis, acanthosis, and papillomatosis are seen in these lesions; features which are seen in seborrheic keratosis as well. The differentiating point, however, is the presence of intraepidermal clefting with acantholytic cells that are seen in every case of postpemphigus acanthoma.[13] The level of the cleft is midepidermal or in the granular layer in cases of postpemphigus foliaceous acanthomata and is suprabasal in postpemphigus vulgaris acanthomata. It also has to be histologically differentiated from Acantholytic acanthoma, which occurs as a solitary lesion and shows hyperkeratosis and acantholysis at multiple levels of the epidermis.[15] Localized and disseminated epidermolytic acanthoma can have a clinical resemblance to postpemphigus acanthoma.[16] The granular and vacuolar degeneration of the cells of the spinous and granular layers and absence of acantholysis distinguishes this entity from postpemphigus acanthoma.[17] Immunofluorescence has been reported to be positive in postpemphigus acanthoma. DIF positivity with the presence of acantholysis, despite apparent clinical remission, has led some authors to interpret these lesions as an early indicator of relapse.[18] In our case, the activity of the disease was proven by the high Dsg1 levels and intermediate Dsg3 levels. DIF was not done in our patient as he refused to undergo another biopsy for the same. Lesions of post pemphigus acanthoma tend to resolve spontaneously within 6 months. However, occasionally, they do require systemic steroids and/or immunosuppressants for resolution. Our patient also had persistent acanthomatous lesions for 1 year, which gradually resolved with azathioprine, dapsone, and topical steroids.

Thus, our case report indicates that pemphigus acanthomata, although occurring in the setting of the absence of bullous lesions, can be an indicator of the activity of pemphigus and warrant active management. Hence, we suggest the use of the term pemphigus acanthomata instead of postpemphigus acanthomata in view of the presence of active acantholysis and high Dsg 1 titers.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest

  References Top

Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol 1999;40:649-71.  Back to cited text no. 1
Robledo MA, Prada S, Jaramillo D, Leon W. South American pemphigus foliaceus: Study of an epidemic in El Bagre and Nechi, Colombia 1982 to 1986. Br J Dermatol 1988;118:737-44.  Back to cited text no. 2
Brenner S, Bialy-Golan A, Ruocco V. Drug-induced pemphigus. Clin Dermatol 1998;16:393-7.  Back to cited text no. 3
Chorzelski T, Jablońska S, Blaszczyk M. Immunopathological investigations in the Senear-Usher syndrome (coexistence of pemphigus and lupus erythematosus). Br J Dermatol 1968;80:211-7.  Back to cited text no. 4
Chatterjee M, Meru S, Vasudevan B, Deb P, Moorchung N. Pemphigus foliaceus masquerading as IgA pemphigus and responding to dapsone. Indian J Dermatol 2012;57:495-7.  Back to cited text no. 5
[PUBMED]  [Full text]  
Tsuji Y, Kawashima T, Yokota K, Tateish Y, Tomita Y, Matsumura T, et al. Clinical and serological transition from pemphigus vulgaris to pemphigus foliaceus demonstrated by desmoglein ELISA system. Arch Dermatol 2002;138:95-6.  Back to cited text no. 6
Li N, Aoki V, Hans-Filho G, Rivitti EA, Diaz LA. The role of intramolecular epitope spreading in the pathogenesis of endemic pemphigus foliaceus (fogo selvagem). J Exp Med 2003;197:1501-10.  Back to cited text no. 7
Gopal V, Pinto M, Mala MS. Pemphigus foliaceus: A rare case of exfoliative dermatitis. Clin Dermatol Rev 2017;1:19-21.  Back to cited text no. 8
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Inaoki M, Kaji K, Furuse S, Fujimoto A, Komatsu N, Takata M, et al. Pemphigus foliaceus developing after metastasis of cutaneous squamous cell carcinoma to regional lymph nodes. J Am Acad Dermatol 2001;45:767-70.  Back to cited text no. 9
Kahana M, Trau H, Schewach-Millet M, Sofer E. Pemphigus foliaceus presenting as multiple giant seborrheic keratoses. J Am Acad Dermatol 1984;11:299-300.  Back to cited text no. 10
Bruckner N, Katz RA, Hood AF. Pemphigus foliaceus resembling eruptive seborrheic keratoses. Arch Dermatol 1980;116:815-6.  Back to cited text no. 11
Williams MG. Acanthomata appearing after eczema. Br J Dermatol 1956;68:268-71.  Back to cited text no. 12
Yesudian PD, Krishnan SG, Jayaraman M, Janaki VR, Yesudian P. Postpemphigus acanthomata: A sign of clinical activity? Int J Dermatol 1997;36:194-6.  Back to cited text no. 13
Parsad D, Sharma PK, Gautam RK, Kar HK. Murray williams warts. Indian J Dermatol Venereol Leprol 1997;63:107-8.  Back to cited text no. 14
[PUBMED]  [Full text]  
Tokuda A. Isolated epidermolytic acanthoma. Jpn J Clin Dermatol 1973;27:431-7.  Back to cited text no. 15
Hirone T, Fukushiro R. Disseminated epidermolytic acanthoma. Acta Derm Venereol 1973;53:393-402.  Back to cited text no. 16
Knipper JE, Hud JA, Cockerell CJ. Disseminated epidermolytic acanthoma. IS J Dermato Pathol 1993;15:70-2.  Back to cited text no. 17
Vaishnani JB, Bosamiya SS. Pemphigus: Active or inactive? Indian J Dermatol 2009;54:186-8.  Back to cited text no. 18
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