Erythema Scarlatiniforme Desquamativum Recidivans: A Rare and Puzzling Condition

Recurrent erythema scarlatiniforme desquamativum recidivans (ESDR), also known as Féréol-Besnier disease, is a rare condition marked by a recurrent erythematous rash that is followed by extensive desquamation, primarily affecting the palms and soles. It is often preceded by prodromal symptoms such as malaise, headache, myalgias, digestive issues, and fever. The exact pathogenesis remains unknown, and diagnosis can be challenging due to its resemblance to various infectious, auto-inflammatory, or allergic conditions, leading to diagnostic variability. Given that most reported cases are over 50 years old, our objective is to highlight this rare and enigmatic pathology through a typical case of the generalized variant of ESDR in a 13-year-old girl. We aim to increase physician awareness of this condition and provide reassurance to both parents and their child regarding its benign nature.


Introduction
Erythema scarlatiniforme desquamativum recidivans (ESDR) is a rare condition marked by recurrent episodes of erythema followed by extensive lamellar scaling, particularly affecting the palms and soles [1,2].The rash is preceded by a prodromal phase that includes general malaise, headache, arthralgia, myalgia, digestive symptoms, and fever [3].ESDR is characterized by episodes of cutaneous flare-ups that resolve spontaneously, with intervals of variable duration ranging from a few weeks to several months or even years.The condition has two variants: generalized and localized [2].To illustrate this uncommon skin disorder, we present a typical case of the generalized variant of ESDR in a 13-year-old girl.

Case Presentation
Our patient was a healthy 13-year-old girl with a history of recurrent episodes of generalized pruritic macular erythema followed by lamellar skin peeling that regressed spontaneously.These episodes were preceded by fever, joint and muscle pain, asthenia, and headache.She had no history of atopy, drug hypersensitivity, familial cases, or other triggering factors, including medication or infections.The first episode occurred seven years ago.Initially, the symptoms recurred monthly for four years.Over the last three years, she experienced an average of three eruptions per year, each lasting about two weeks and resolving without treatment.She presented to our facility with a recent rash.Physical examination revealed diffuse macular lesions on the trunk, neck, and limbs (Figure 1), which extended to the hands and feet.The face and mucous membranes were spared.The patient was in good general condition and had no fever.The remainder of the clinical examination was normal.

FIGURE 1: (a, b) Diffuse macular skin rash on the trunk and limbs
One week after the onset of the skin eruption, significant lamellar desquamation was observed on the trunk and limbs (Figure 2), along with thickened, ragged peeling of the palmar and plantar skin (Figure 3).Laboratory tests showed a mildly elevated CRP level of 16 mg/L, a normal leukocyte count of 8,080/mm³ (with neutrophils at 2,070/mm³ and eosinophils at 102/mm³), an erythrocyte sedimentation rate (ESR) of 4 mm/1st hour, and a normal anti-streptolysin O level of 145 U/L (Table 1).Histological examination of the skin revealed epidermal hyperplasia with hyperorthokeratosis.The dermis showed congested vessels and perivascular lymphocytic infiltration.There were no apoptotic keratinocytes or eosinophilic infiltration; this aspecific histological appearance did not contribute to the diagnosis.Following topical therapy with emollients and keratolytics, the lesions resolved within two weeks.

Discussion
ESDR is a rare disorder characterized by recurrent erythematous rashes followed by extensive desquamation, primarily affecting the palmoplantar skin [4,5].First described by Féréol in 1876 [6], only a few cases have been documented since (Table 2) [1,3,[5][6][7][8][9].The rarity of ESDR is likely due to limited awareness [3,5], and the scarcity of literature results in a lack of reliable epidemiological data on its incidence and prevalence [2].ESDR presents in two forms: a generalized variant, which appears to be more common, and a localized variant [3,6].The generalized form, which our case exemplifies, features confluent macular erythema initially on the trunk, spreading to the limbs and face, followed by lamellar desquamation [1][2][3].Pruritus may also be observed, as in our patient, while mucous membranes are typically unaffected [2].The rash is preceded by a prodromal phase with general malaise, headaches, muscle aches, gastrointestinal symptoms, fever, joint pain, or bronchitis [1,3,5].Additional clinical features may include conjunctivitis, pharyngitis, epistaxis, alopecia, transient proteinuria, and microhematuria [1,3].The condition is marked by acute episodes lasting two to three weeks, resolving spontaneously, with asymptomatic intervals between episodes ranging from months to years [5,6].

Age
The etiology and pathogenesis of ESDR remain unknown [1].Triggering factors reported include drug hypersensitivity, viral and bacterial infections, particularly staphylococcal and streptococcal [2].In our patient, no specific etiology was identified.
Diagnosis is primarily clinical, as histological and laboratory findings are nonspecific [1].Blood tests typically reveal elevated inflammation markers, such as an increased erythrocyte sedimentation rate or CRP, and sometimes hyperleukocytosis, with occasional hypereosinophilia [3].In our patient, inflammatory parameters were normal except for a slightly elevated CRP.Histological examination may show hyperparakeratosis, orthokeratosis, acanthosis above the stratum granulosum, and inflammation [2,3].Differential diagnoses include scarlet fever, staphylococcal skin infections, toxic epidermal necrolysis, and recurrent fevers like familial Mediterranean fever or mevalonate kinase deficiency [3].
Currently, there are no specific guidelines for treating this rare benign condition.Symptomatic treatments, including topical and systemic steroids, systemic antibiotics, emollients, and topical keratolytics, may be useful but do not alter the natural course of the disease or prevent new episodes [1,3,5].

Conclusions
Although well-documented, ESDR remains a rare and often underdiagnosed skin condition.It should be considered in any patient presenting with recurrent erythema followed by lamellar desquamation, once other differential diagnoses have been excluded.Recognizing ESDR can help reassure parents regarding the prognosis.Symptomatic therapy is currently the primary approach for managing ESDR.Ongoing research and case studies are essential for refining diagnostic criteria and therapeutic strategies to better address this condition.