Pemphigus Foliaceus and Endemic Forms

Fig. 23.1

Clinical features of pemphigus foliaceus/fogo selvagem. (a) Localized (forme fruste), erosion(s) and/or crusted lesion(s) on photo-exposed/seborrheic areas. (b) Bullous invasive (generalized variant), disseminated superficial vesicles/blisters. No mucosal involvement. Circinate, erythematous plaques (resemble skin lesions seen in those infected with tinea imbricata). (c) Erythroderma (generalized variant), exfoliative erythroderma. (d) Hyperkeratotic, hyperkeratotic and hyperpigmented papules/plaques on the trunk. (e) Hyperpigmented, usually present at disease remission, may indicate a good prognosis. (f) Herpetiform, clustered vesicles accompanied, preceded, or succeeded by urticarial plaques

Pemphigus erythematosus (PE) is a distinct variant of PF that has clinical features similar to those of both PF and lupus erythematosus (LE). Flaccid vesicles and/or erosions are present on seborrheic areas of the skin. Like LE, lesions are photodistributed on areas of the upper chest and back and in a malar pattern on the face. Lesions, especially those on the face and scalp, are often hyperkeratotic with thick, greasy scales and yellow crusts [40].

PF may occasionally present with atypical skin findings. Several cases have described PF presenting as an acute eruption of many hyperpigmented and hyperkeratotic verrucous papules and plaques that closely resemble the appearance of seborrheic keratoses [41, 42]. Children with PF may present with flaccid vesicles and bullae and/or erosions in an arcuate, circinate, and/or polycyclic pattern [43]. PF presenting as an isolated erythematous plaque on the cheek with a “peau d’orange” appearance has also been reported [44].

23.4 Prognosis

Death occurs in 5–10 % of FS/PF patients and usually results from complications of prolonged systemic immunosuppressive therapy or secondary infections. Eczema herpeticum (herpes simplex), disseminated Strongyloides stercoralis, or bacterial sepsis may be fatal [38].

References

Robinson ND, Hashimoto T, Amagai M, et al. The new pemphigus variants. J Am Acad Dermatol. 1999;40:649–71.CrossRefPubMed

Santi CG, Maruta CW, Aoki V, et al. Pemphigus herpetiformis is a rare clinical expression of non-endemic pemphigus foliaceus, fogo selvagem and pemphigus vulgaris. J Am Acad Dermatol. 1996;34(1):40–6.CrossRefPubMed

Yamamoto S, Kanekura T, Gushi A, et al. A case of localized pemphigus foliaceus. J Dermatol. 1996;23:893–5.PubMed

Nousari HC, Moresi M, Klapper M, Anhalt GJ. Nonendemic pemphigus foliaceus presenting as fatal bullous exfoliative erythroderma. Cutis. 2001;67:251–2.PubMed

Diaz LA, Sampaio SA, Rivitti EA, et al. Endemic pemphigus foliaceus (fogo selvagem) I: clinical features and immunopathology. J Am Acad Dermatol. 1989;20(4):657–69.CrossRefPubMed

Diaz LA, Sampaio SA, Rivitti EA, et al. Endemic pemphigus foliaceus (Fogo Selvagem) II: current and historic epidemiologic studies. J Invest Dermatol. 1989;92(1):4–12.CrossRefPubMed

Sampaio SA, Rivitti EA, Aoki V, Diaz LA. Brazilian pemphigus foliaceus, endemic pemphigus foliaceus, or fogo selvagem (wild fire). Dermatol Clin. 1994;12(4):765–76.PubMed

Culton DA, Qian Y, Li N, et al. Advances in pemphigus and its endemic pemphigus foliaceus (fogo selvagem) phenotype: a paradigm of human autoimmunity. J Autoimmun. 2008;31(4):311–24.CrossRefPubMedCentralPubMed

Hans-Filho G, dos Santos V, Katayama JH, et al. An active focus of high prevalence of fogo selvagem on an Amerindian reservation in Brazil. Cooperative Group on Fogo Selvagem Research. J Invest Dermatol. 1996;107(1):68–75.CrossRefPubMed

10.

Auad A, Castro RM, Fraga S, et al. The treatment of Brazilian pemphigus foliaceus (fogo selvagem). Int J Dermatol. 1970;9(2):130–6.CrossRefPubMed

11.

Warren SJ, Lin MS, Giudice GJ, et al. The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research. N Engl J Med. 2000;343:23–30.CrossRefPubMed

12.

Robledo MA, Prada S, Jaramillo D, et al. South American pemphigus foliaceus: study of an epidemic in El Bagre and Nechi, Colombia 1982 to 1986. Br J Dermatol. 1988;118:737–44.CrossRefPubMed

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